


1 




Class 

Book .- ?S 

Copyright^ 

COPYRIGHT DEPOSIT; 



DISEASES OF 
THE NERVOUS SYSTEM 



MODERN CLINICAL MEDICINE 

DISEASES 

OF THE 

NERVOUS SYSTEM 

EDITED BY 

ARCHIBALD CHURCH, M.D. 

PROFESSOR OF NERVOUS AND MENTAL DISEASES AND MEDICAL JURISPRUDENCE 

NORTHWESTERN UNIVERSITY MEDICAL DEPARTMENT 

CHICAGO, ILLINOIS 



AN AUTHORIZED TRANSLATION FROM "DIE DEUTSCHE KLINIK' 
UNDER THE GENERAL EDITORIAL SUPERVISION OF 

JULIUS L. SALINGER, M.D. 



WITH ONE HUNDRED AND NINETY-FIVE ILLUSTRATIONS 
IN THE TEXT AND FIVE COLORED PLATES 




NEW YORK AND LONDON 
D. APPLETON AND COMPANY 

' 1910 






Copyright, 1907, 1910, by 
D. APPLETON AND COMPANY 



PRINTED AT THE APPLETON PRESS 
NEW YORK, U. S. A. 



C CI. A 256775 



EDITOR'S PREFACE 



Evert practitioner must have been frequently impressed with the inade- 
quacy of the usual text book description of the various diseases with which 
he is brought into contact. Even books of reference do not always give the 
scope to special topics that are requisite to place the physician in a satisfac- 
tory position relative to any particular disease he may be called upon to treat. 
The articles on nervous diseases embraced within this volume meet this actual 
want in a manner so admirable that it is a satisfaction to have been associated 
with their production in English. To the beginning practitioner to some 
extent they supply the place of personal experience, and even to the specialist 
they furnish a most admirable resume and guide. 

The various subjects having been assigned to men of known experience, 
with large opportunities for their study, insured a presentation that is essen- 
tially practical and embraces the value of an authoritative personality. The 
manner in which the subject matter is presented also appeals to the medical 
reader, and while the statements are carefully made they are marshalled in 
an interesting and instructive fashion. 

One is struck throughout the book with the fact that while Germany is 
supposed to be the land of therapeutic nihilism these various authors have 
found therapeutics of real help and afford the reader the benefit of their 
experience and judgment in this important branch of practice. 

At first the editorial work was taken up with the expectation of making 
numerous addenda to the text, but when the proper scope of the work was 
realized it was deemed expedient to let each author stand responsible for the 
subject matter which he had personally presented and not to confuse the 
medical reader by discussions of disputed and unessential features. 

Archibald Church. 
Pullman Building, 
Chicago, 111. 



LIST OF CONTRIBUTIONS 



The Macroscopic Anatomy of the Central Nervous System, with Special Ref- 
erence to the Physiology of the Brain. By M. Rothmann, Berlin. 
Normal and Pathological Histology of the Central Nervous System, with 

Special Reference to the Neuron Theory. By H. Rosin, Berlin. 
General Neurological Diagnosis, Including the Examination of Patients with 

Diseases of the Nervous System. By P. Schuster, Berlin. 
Modern Aids in the .Diagnosis of Diseases of the Brain. By E. Redlich, 

Vienna. 
Lumbar Puncture. By H. Quincke, Kiel. 

Cerebral Hemorrhage and Embolism. By R. Geigel, Wiirzburg. 
The Symptom-Complex of Aphasia. By C. Wernicke, Breslau. 
The Nature and the Treatment of Disturbances of Speech. By H. Gutz- 

mann, Berlin. 
Neoplasms of the Spinal Cord and of its Membranes. By Fr. Schultze, 

Bonn. 
Myelitis. By E. v. Leyden and P. Lazarus, Berlin. 
Tabes Dorsalis (Gray Degeneration of the Posterior Columns of the Spinal 

Cord: Ataxic Locomotrice Progressive; Locomotor Ataxia). By Wil- 

helm Erb, Heidelberg. 
Multiple Sclerosis. By E. Redlich, Vienna. 
Syringomyelia. By Fr. Schultze, Bonn. 

Hereditary Ataxia (Friedreich' 's Disease). By H. Luthje, Erlangen. 
Spastic Spinal Paralysis, and Hereditary Spastic Spinal Paralysis. By 

E. Redlich, Vienna. 
Progressive Muscular Atrophy (Dystrophy), Progressive Spinal Muscular 

Atrophy and Bulbar Paralysis. By Fr. Schultze, Bonn. 
Paralyses of the Peripheral Nerves. By M. Bernhardt Berlin. 
Neuritis and Polyneuritis. By R. Cassirer, Berlin. 
Neuralgia. By H. Eichhorst, Zurich. 

Headache and Migraine. By L. Edinger, Frankfort-on-the-Main. 
Paralysis Agitans (Parkinson's Disease). By W. Erb, Heidelberg. 
Athetosis. By L. v. Frankl-Hochwart, Vienna. 
Tetany of Adults. By L. v. Frankl-Hochwart, Vienna. 

1* vii 



viii LIST OF CONTRIBUTIONS 

Thomsen's Disease {Myotonia Congenita). By L. v. Frankl-Hochwart, 

Vienna. 
Localized Spasm. By E. Remak, Berlin. 
The Present Status of Graves' Disease (Exophthalmic Goiter, Basedow's 

Disease). By A. Eulenburg, Berlin. 
Sexual Neurasthenia. By A. Eulenburg, Berlin. 
Epilepsy. By W. Vorkastner, Berlin. 
Hysteria. By Th. Ziehen, Berlin. 
Traumatic Neuroses. By P. Schuster, Berlin. 
Vasomotor Trophic Neuroses. By R. Cassirer, Berlin. 
Occupation Neuroses. By R. Cassirer, Berlin. 



CONTENTS 



PAGE 

The Macroscopic Anatomy of the Central Nervous System with Special 

Reference to the Physiology of the Braix . . . . . 1 

Normal and Pathological Histology of the Central Nervous System, with 

Special Reference to the Neuron Theory 34 

I. Preliminary Remarks: The Histologic Components of the Nervous System . 35 

A. The Nerve Tissue 35 

B. The Supporting Substance (The Gray or Vesicular Substance) ... 42 

II. The Grouping of the Nervous Substance and the Neuron Theory ... 44 

III. The Fibrillae Theory. (Neuro-Fibrils) 46 

IV. Neuron Systems and Neuron Diseases 47 

A. The Motor Tract 48 

1. The First Motor Neuron 48 

2. The Second Motor Neurons 60 

B. Diseases of the Motor Tract 69 

1. Diseases of the First Motor Neuron 70 

2. Diseases of Both Motor Neurons 72 

C. The Sensory Tract 73 

1. The First Sensory Neuron 73 

2. The Second Sensor}' Neuron 86 

3. The Third Sensory Neuron 97 

D. Diseases of the Sensory Neurons. Tabes Dorsalis 100 

E. Other Known Tracts in the Central Nervous System 103 

1. In the Cerebrum and Peduncles of the Brain 103 

2. In the Cerebellum 107 

F. Neuron Diseases of the Cerebellum. Friedreich's Ataxia . . . .112 

G. Combined System Diseases 113 

V. Other Diseases of the Central Nervous System 113 

A. Acute Myelitis, Encephalitis, and Acute Softening . . r . . .114 

B. Acute Poliomyelitis 117 

C. Acute Bulbar Paralysis 118 

D. Chronic Myelitis and Encephalitis 118 

E. Chronic Poliomyelitis 119 

F. Multiple Sclerosis 120 

G. Secondary Degenerations 120 

1. Secondary Descending Degeneration of the Spinal Cord .... 122 

2. Ascending Degeneration in the Spinal Cord 124 

3. Secondary .Degeneration in the Brain 125 

H. Syphilis of the Nervous System 125 

General Neurological Diagnosis including the Examination of Patients with 

Diseases of the Nervous System 129 

Introduction 129 

History (Anamnesis) 130 

ix 



X CONTENTS 

PAGE 

The Present Condition (Status Prsesens) * . . 135 

The Skull and the Vertebral Column 137 

Vasomotor and Trophic Disturbances 141 

Motility 149 

Examination of Coordination of Muscular Movements 168 

Involuntary Motor Symptoms 175 

Epileptic and Hysteric Spasmodic Attacks 179 

Examination of the Sensory Functions and of Sensation 183 

Examination of the Optic Nerve .184 

Examination of the Sense of Smell 187 

Examination of the Sense of Taste 188 

Examination of the Sense of Hearing 189 

Sensory Conditions of the Skin . 190 

Sensibility of the Deeper Tissues 194 

Examination of the Reflexes 196 

The Tendon Reflexes 196 

Cutaneous Reflexes 202 

The Internal Reflexes . 206 

Modern Aids in the Diagnosis of Diseases of the Brain .213 

Lumbar Puncture . • 223 

Cerebral Hemorrhage and Embolism 258 

The Symptom-Complex of Aphasia 265 

I. Disturbances of Phonetic Speech 265 

II. Disturbances of Written Language and General Pathology of Speech Dis- 

turbances 284 

Treatment 323 

The Nature and the Treatment of Disturbances of Speech 325 

Peripheral Impressive Disturbances 325 

Central Disturbances: Stammering 333 

Peripheral Expressive Disturbances 343 

Neoplasms of the Spinal Cord and of Its Membranes 347 

Varieties of Neoplasms 347 

Symptomatology 348 

The Seat, Extension and Composition 352 

Treatment 369 

Myelitis 371 

History 371 

Pathological Anatomy 372 

The Clinical Picture 382 

The Clinical Picture of Dorsal Myelitis 384 

1. Prodromal Stage 384 

2. The Stage of Paralysis 386 

3. Stage of Regeneration 403 

4. Termination of Myelitis 403 

The Segmentary Localization of the Function of Motion 404 

Spinal Localization of the Motor Functions 406 

Spinal Localization of the Reflexes • . . . . 409 

Transverse Diagnosis 410 

1. Cervical Myelitis 413 

2. Lumbar Myelitis 417 

3. Sacral Myelitis 418 

4. Diseases of the Cauda Equina 423 



CONTENTS XI 

PAGE 

Etiology of Acute Myelitis » 424 

Differential Diagnosis 428 

Prognosis of Myelitis 432 

Therapy of Myelitis 433 

1. Prophylaxis 433 

Treatment 434 

Tabes Dorsalis 451 

I. Introduction and History 451 

II. Etiology (The Causes of Tabes) 454 

III. Symptomatology 471 

IV. Pathological Anatomy 494 

V. Pathologic Physiology of Tabes. Pathogenesis of the Different Symptoms . 508 

VI. Course, Termination, and Definite Forms of Tabes 521 

VII. Diagnosis 527 

VIII. Prognosis 532 

IX. Treatment 534 

Multiple Sclerosis 557 

Etiology 558 

Symptomatology 563 

Course 570 

Pathology 570 

Differential Diagnosis 575 

Prognosis 580 

Treatment 580 

Syringomyelia 582 

Symptomatology 592 

Course and Prognosis 605 

Occurrence and Etiology 606 

Diagnosis and Differential Diagnosis 607 

Treatment 611 

Hereditary Ataxia (Friedreich's Disease) 612 

Clinical Course 613 

Pathological Anatomy and Histology 615 

Pathogenesis 620 

Etiology 623 

Differential Diagnosis 623 

Prognosis, Course and Treatment 625 

Spastic Spinal Paralysis, and Hereditary Spastic Spinal Paralysis . . . 627 

Symptoms 628 

Pathology 628 

Diagnosis . 629 

Treatment 632 

Progressive Muscular Atrophy (Dystrophy), Progressive Spinal Muscular 

Atrophy and Bulbar Paralysis 633 

1. Progressive Dystrophy (Myopathic Primitive, of the French) .... 633 

2. Progressive Neurotic Muscular Atrophy 646 

3. Progressive Spinal Muscular Atrophy with and without Bulbar Paralysis . 649 

(a) Infantile Hereditary Form (Werdnig-Hoffmann) 649 

(6) Progressive Spinal Amyotrophy, Aran-Duchenne Type .... 652 

(c) Chronic Anterior Poliomyelitis 654 

4. Amyotrophic Lateral Sclerosis and Amyotrophic Progressive Bulbar Paralysis 

(Charcot's Disease) 656 



Xll CONTENTS 

PAGE 

Paralysis of the Peripheral Nerves ........... 662 

1. Paralysis of the Facial Nerve 662 

Pathology 671 

2. Paralysis of the Trigeminal Nerve . . 676 

3. Glossopharyngeal Paralysis 681 

4. Paralysis of the Pneumogastric Nerve . 683 

5. Paralysis of the Spinal Accessory Nerve 687 

6. Paralysis of the Hypoglossal Nerve 690 

7. Multiple Cerebral Nerve Paralysis 693 

8. Paralysis of the Diaphragm 695 

9. Paralysis of the Suprascapular and Postscapular Nerves 698 

10. Paralysis of the Anterior Thoracic and Subscapular Nerves .... 700 

11. Paralysis of the Thoracicus Longus Nerve . . . . . . . 701 

12. Paralysis of the Axillary Nerve 705 

13. Paralysis of the Musculocutaneous Nerve 707 

14. Musculospiral Paralysis 708 

15. Paralysis of the Ulnar Nerve 712 

16. Paralysis of the Median Nerve 715 

17. Combined Paralysis of the Nerves of the Arm and Shoulder . . . .718 

18. Paralysis of the Muscles of the Back, Neck, and Abdomen .... ?24 

19. Paralysis of the Crural Nerve . . . 726 

20. Paralysis of the Obturator Nerve 728 

21. Paralysis of the External Cutaneous Femoral Nerve 729 

22. Paralysis of the Superior and Inferior Gluteal Nerves and of the Posterior 

Cutaneous Femoral Nerves . 730 

23. Paralysis of the Sciatic Nerve 731 

(a) Paralysis of the Peroneal Nerve 732 

(6) Paralysis of the Tibial Nerve 733 

24. Paralysis in the Course of the Pudendic and Coccygeal Plexuses . . . 736 
Neuritis and Polyneuritis 742 

Neuritis 742 

Symptoms 771 

Diagnosis 774 

Treatment 774 

Pathology 777 

Polyneuritis 782 

Symptoms 783 

Pathology 813 

Treatment • 826 

Neuralgia 828 

Symptoms 828 

Disturbances of Tactile Sense 833 

Diagnosis 835 

Etiology 836 

Treatment . 845 

Headache and Migraine 851 

Differentiation of True Headache 851 

Causes, Course, and Treatment 856 

Headache of Children 856 

Headache of Adolescents 858 

Headache of the Anemic 859 

Headache, Vasoparalytic . 861 



CONTENTS xiii 

PAGE 

Headache due to Organic Cause 863 

Headache, Indurative 863 

Headache, Syphilitic 869 

Migraine 870 

The Theory of Headache 875 

Paralysis Agitans 830 

Definition 881 

Etiology 881 

Symptoms 884 

Course 8S9 

Pathology . . .' . 889 

Diagnosis 893 

Prognosis 895 

Treatment 895 

Athetosis 899 

Pathology 902 

Tetany of Adults 907 

History 907 

Etiology 911 

Symptoms 914 

Diagnosis 918 

Prognosis 919 

Pathology 920 

Treatment 920 

Thomsen's Disease 922 

Etiology 925 

Symptoms 926 

Pathology 929 

Diagnosis 930 

Localized Spasm 933 

Differentiation of Forms 934 

Symptomatology 936 

Etiology 938 

Course 939 

Treatment , 939 

Clinical Varieties 940 

The Present Status of Graves' Disease 960 

Symptomatology 961 

Diagnosis 962 

Theories Concerning Graves' Disease 963 

Treatment 970 

Sexual Neurasthenia 976 

Definition and History 976 

General Symptomatology 980 

Special Symptomatology 982 

Pathologic Pollutions 982 

Spermatorrhea and Prostratorrhea 985 

Pathologic Erections. "Priapism" 986 

Neurasthenic Impotence 988 

Hypospermia (Oligospermia) and Aspermia . 993 

Etiology .994 



xiv CONTENTS 

PAGE 

Prophylaxis . > 999 

Treatment 1002 

Epilepsy 1012 

History -. . - . . . 1012 

General Symptoms 1013 

Individual Symptoms <. 1023 

Theories of Epilepsy . 1024 

Intervals between Attacks 1028 

Prognosis . 1030 

Etiology ■ . 1031 

Pathology 1037 

Diagnosis 1037 

Treatment 1040 

Hysteria 1045 

Etiology 1047 

Symptomatology 1050 

Permanent Somatic Symptoms 1051 

Disorders of Motility 1051 

Paralyses 1051 

Contractures 1055 

Clonic Muscular Spasms 1059 

Tremor 1061 

Disturbances of Coordination 1062 

Sensory Disturbances 1063 

Disturbances of Sight 1067 

Disturbances in Hearing . 1069 

Disturbances in Taste and Smell 1069 

Spontaneous Pains 1070 

Reflexes 1073 

Sympathetic Nervous System 1075 

Psychical Permanent Symptoms 1077 

Paroxysmal Symptoms 1078 

The Phase of Coordinated Movements . 1079 

Course and Prognosis 1084 

Differential Diagnosis 1085 

Treatment 1091 

Traumatic Neuroses 1098 

History 1098 

Etiology and Pathogenesis . 1101 

Symptomatology 1105 

Diagnosis; Simulation 1116 

Prognosis and Course 1122 

Prophylaxis and Treatment 1124 

Vasomotor Trophic Neuroses 1128 

Acroparesthesia 1130 

Raynaud's Disease 1132 

Erythromelalgia 1139 

Scleroderma 1141 

Acute Circumscribed Edema 1144 

Occupation Neuroses . . . .1149 

Writer's Cramp 1149 

Typist's Cramp 1154 



CONTENTS xv 

PAGE 

Cramps Caused by Playing Other Musical Instruments 1156 

Cramps of the Lower Extremities 1157 

Other Forms of Occupation Neuroses 1157 

Pathogenesis 1159 

Treatment 1159 

Index of Authors 1161 

Index of Subjects 1173 



LIST OP COLORED PLATES 



Plate I Facing page 450 

Fig. 1. — Influenza myelitis. 

Fig. 2. — Acute myelitis in the puerperium. 

Fig. 3. — Hematomyelia in the puerperium. 

Plate II Following page 826 

Fig. 1. — Sciatic nerve of a rabbit, peripheral portion; 10 days 

after severing the trunk. 
Fig. 2. — Nerve-cicatrix from the brachial plexus after an incised wound. 

Plate III . Following page 826 

Fig. 3. — Ulnar nerve in parenchymatous neuritis. 
Fig. 4. — The same as Fig. 3. 

Plate IV Following page 826 

Fig. 5. — Sciatic nerve in acute polyneuritis. 
Fig. 6. — Ulnar nerve in polyneuritis leprosa. 

Plate V . Following page 826 

Fig. 7. — Ulnar nerve in polyneuritis leprosa; different view than Fig. 6. 
Fig. 8. — Ulrar nerve in polyneuritis leprosa; the same field as Fig. 6. 



I 



LIST OF ILLUSTRATIONS 



FIG. PAGE 

1. — Median sagittal section through the brain of a human embryo at the end of the 

first month 2 

2. — The development of the ventricular system 3 

3. — Brain of a human fetus at the end of the fifth month 4 

4. — Brain of a human fetus at seven months 5 

5. — Lateral view of the brain 7 

6. — Longitudinal section through the middle of the human brain ... 10 

7. — Cortex of the cerebrum of apes 12 

8. — Left cerebral cortex of a female chimpanzee, showing the results of electric 

irritation 13 

9. — Horizontal section through the brain with the nucleus caudatus, thalamus 

opticus, and nucleus lentiformis 14 

10.— Portion of a median section through the cerebrum 15 

11. — Arrangement of the motor fibers in the anterior capsule .... 18 
12. — Horizontal section through the inter-brain, one-half centimeter below the 

surface of the thalamus and the nucleus caudatus 19 

13. — Diagram of the cerebral peduncles 20 

14. — The cerebellum from the dorsal side. 21 

15. — The cerebellum from the A'entral side 22 

16. — Combined sagittal section through the stem of the brain .... 23 

17. — The base of the brain, showing the origin of the roots of the cranial nerves . 25 

18. — The distribution of the arteries at the base of the brain and the circle of Willis 27 
19. — The lumbar cord, pars lumbalis medulla* spinalis, with the conus medullaris, 

the filum terminale, and the cauda equina, seen from behind . . 29 
20. — Transverse section through the cervical enlargement, intumescentia cervicalis, 
of the spinal cord at the point of exit of the roots of the sixth cervical 

nerve 30 

21. — Topographic relations between the surface of the brain and the skull . . 31 

22. — Nerve-cell from the anterior horn of the spinal cord of a rabbit .... 35 
23. — A, Sudan staining of the anterior horn of the spinal cord (man); B, Sudan 

staining of cerebral cortex (man) 37 

24. — Motor anterior horn cell with its processes .39 

25. — Ganglion cell of the anterior horn 39 

26. — Sensory nerve-cell 40 

27. — Nerve-cell of the sympathetic 40 

28. — Diagram of a motor neuron; first division 41 

29. — Diagram of a sensory neuron; first division 41 

30. — Medullated nerve fiber 42 

31. — Transverse section through the cervical enlargement 42 

32. — Transverse section through the upper lumbar cord 43 

33. — Transverse section through the normal gray substance of the anterior horn of 

the spinal cord 43 

xvii 



xvill LIST OF ILLUSTRATIONS 

FIG. PAGE 

34. — Transverse section through the normal white substance of the spinal cord . 44 

35.— Glia cells 44 

36,- — Diagram of the structure, the course, and the branching of motor neurons of 

the first and second divisions 45 

37. — Diagram of the conduction tracts of the white substance of the spinal cord . 47 

38. — Diagram of the various tracts and neuron systems in the spinal cord . . 48 

39. — The anterior roots and their relations to the membranes .... 49 
40. — Transverse section through the intervertebral disc, between the third and fourth 

cervical veretebrse 49 

41. — From the anterior horn of the gray substance of the spinal cord . . 49 

42. — Medulla oblongata, pons, peduncles, and adjacent parts seen from the base . 50 

43. — Base of the brain and cerebral nerves 51 

44. — Prolonged cord with the rhomboid fossa and corpora quadrigemina from 

above 52 

45. — Diagram of Fig. 44 .52 

46. — Section through the anterior corpora quadrigemina 52 

47. — The nuclear origin of the oculomotor and trochlear nerves in the middle brain 53 

48. — Nuclear origin of the cerebral nerves 54 

49. — Nuclear origin of the cerebral nerves 55 

50. — Facial origin and its surroundings 56 

51. — Section in the region of the origin of the abducens 57 

52. — Section through the medulla oblongata 58 

53. — Section through the medulla oblongata at the point of exit of the pneumo- 

gastric 59 

54. — Section through the medulla oblongata at the height of the posterior hypo- 
glossal roots 59 

55. — Convexity of the brain from above 61 

56. — Convexity of the brain seen from the side 62 

57. — Motor region of the cerebral cortex in man 62 

58. — Section through the cortex of a frontal convolution 63 

59. — A partly diagrammatic presentation of the cortex of the cerebrum . . 64 

60. — Section through the internal capsule 64 

61. — The pyramidal tract 65 

62. — The pyramidal tract beside the tract of the hypoglossal and facial nerves . 66 

63. — Frontal section through the hemispheres behind the optic chiasm ... 67 

64. — Horizontal section through the hemispheres 68 

65. — Course of the second motor neuron in the spinal cord 69 

66. — Transverse section through the spinal cord in atrophic spastic bulbospinal 

paralysis 73 

67. — Diagram showing the branching of the dorsal roots .74 

68. — Diagram of a transverse section of the spinal cord 75 

69. — Diagrammatic course of the first sensory neuron 76 

70. — Diagram showing the origin and termination of the nerve roots in the gray 
substance of the spinal cord and the distribution of the nerve cells in the 

latter 76 

71. — Diagram of the course of the sensory tracts from the posterior roots to the 

prolonged cord 77 

72. — The nuclear origin of the cranial nerves 78 

73. — The nuclear origin of the cranial nerves 79 

74. — Section through the medulla oblongata 80 

75. — Section through the medulla oblongata at the point of exit of the pneumo- 

gastric 80 

76. — Section through the medulla oblongata at the height of the posterior hypo- 
glossal roots ,♦,,..,,, 81 



LIST OF ILLUSTRATIONS xix 

FIG. PAGE 

77. — Base of the brain and cranial nerves 82 

78. — Medulla oblongata with the fourth ventricle and the corpora quadrigemina, 

seen from above 83 

79. — Diagram of the course of the cochlear nerve 84 

80. — Region of the corpora quadrigemina, thalamus, and medulla, from the right 

side 85 

81. — Corpora quadrigemina, thalamus, and medulla, from above .... 85 

82. — Tract of the optic nerve 87 

83. — The bulbus olfactorius of the mouse .88 

84. — Diagram of the origin and termination of the nerve roots in the gray substance 

of the spinal cord and the distribution of the nerve-cells of the latter . 88 
85. — Diagram of the course of the sensory tracts from the posterior roots to the 

medulla 89 

86. — Diagram of the motor and sensory conduction tracts 90 

87. — Diagram of the lemniscus tract 91 

88. — Origin of the facial nerve and its surroundings 92 

89. — Section in the region of the abducens . • 93 

90. — Transverse section through the pyramidal decussation 93 

91. — Transverse section through the medulla below the olives .... 94 
92. — Transverse section through the lower portion of the olives and the fourth 

ventricle 94 

93. — Transverse section through the middle of the olive and the lower portion of 

the fourth ventricle 94 

94. — Transverse section through the middle of the fourth ventricle ... 95 
95. — Transverse section through the lower border of the pons immediately above 

the olives and through the middle of the fourth ventricle ... 95 
96. — Transverse section through the middle of the pons and the anterior medullary 

velum 96 

96a. — Section through the pons 96 

97. — Transverse section through the upper margin of the pons and the posterior 

corpora quadrigemina 97 

98. — Transverse section through the upper corpora quadrigemina, the tegmentum 

and the crus cerebri 98 

99. — Transverse section through the upper corpora quadrigemina in its most anterior 

portion 99 

100. — Posterior roots and anterior roots in tabes 102 

101. — Tabetic degeneration 102 

102. — Tabetic degeneration in the lumbar cord 102 

103. — Tabetic disease of the cervical cord 102 

104. — Tabetic disease of the lumbar cord 102 

105. — Posterior columns in tabes 103 

106. — Association bundle projected upon the median surface of the hemisphere . 104 

107. — Median sagittal section through the brain 105 

108. — The most important commissure fibers 105 

109. — Diagram showing the arms of the cerebellar tracts 108 

110. — Cerebellar tracts 109 

111. — Cortex of the cerebellum 110 

112. — Friedreich's disease Ill 

113. — Friedreich's ataxia 112 

114. — Combined system diseases 113 

115. — Lumbar cord after an attack of acute poliomyelitis on the left side . . .117 

116. — Sclerotic foci in the spinal cord 120 

117.— Multiple sclerosis 120 

118. — Transverse myelitis with ascending and descending degeneration . . .121 



XX LIST OF ILLUSTRATIONS 

FIG. PAGE 

119. — Descending degeneration of a pyramidal tract of the spinal cord . . . 122 

120. — Bilateral descending degeneration of the pyramidal tract .... 122 
121. — Diagram of descending degeneration of a pyramidal tract . . . .123 

122. — Secondary degeneration after disease of the conus medullaris . . . 124 

122a. — Ascending degeneration (right-sided myelitic focus) 125 

123. — Secondary ascending degeneration ' . . < . . 125 

124. — Syphilitic endarteritis and gummatous infiltration of the surrounding area . 126 

125. — The normal and the syphilitically diseased anterior spinal artery . . . 126 

126. — Syphilitic spinal paralysis 127 

127.— Brain and skull 225 

128.— Cerebellum 233 

129a. — Site for lumbar puncture 236 

1296. — Site for lumbar puncture 236 

130. — Instrument for lumbar puncture 237 

131a. — Cauda equina of the new-born 238 

1316. — Cauda equina of the adult 238 

132. — Lumbar vertebral column of a child one year old 239 

133a. — Lumbar vertebral column 240 

1336. — Lumbar vertebral column 240 

134a. — Lumbar vertebral column . 240 

1346. — Lumbar vertebral column 240 

135. — Transverse section through the lumbar vertebral column, the puncture needle 

in situ 241 

136. — Instrument for lumbar puncture . . 253 

137. — Diagram of aphasia 281 

138. — Diagram of aphasia 282 

139. — Diagram of the brain .311 

140. — Diagram of the brain 312 

141. — Diagram of the brain 314 

142. — Diagram of the brain 316 

143. — Diagram of the brain 319 

144. — Ancestral tree 335 

145. — Diagram showing the normal process of speech in man 336 

146.— Stuttering in a girl 337 

147.— Stuttering in a girl 338 

148. — Stuttering during the enunciation of "1" and "p" in the word "lamp" . . 338 

149. — Diagram of stuttering 339 

150. — Diagram of stuttering 340 

151. — Segment innervation of the skin 355 

152. — Segment innervation of the skin 355 

153.— Diagram 356 

154. — Diagram 357 

155. — Diagram 358 

156.— Diagram 359 

157.— Diagram 360 

158.— Diagram . . . . . . .360 

159.— Diagram 362 

160. — Sarcoma of the cauda equina 366 

161. — Inflammation of the dorsal cord 374 

162. — Syphilitic myelitis of the lumbar enlargement . . . ... . . 381 

163.— Syphilitic myelitis 382 

164. — Arrangement in Brown-Sequard's paralysis 392 

165.— Posterior view of Fig. 164 393 

166. — Traumatic hematomyelia 400 



LIST OF ILLUSTRATIONS xxi 

PIG. PAGE 

167. — Destruction of the cord from the eighth dorsal segment down . . . 401 
168. — Schematic representation of the sensory and motor spinal cord tracts . .411 

169. — Distribution of tactile and thermic anesthesia and analgesia .... 420 

170. — Posterior view of Fig. 169 421 

171. — Apparatus 448 

172. — Apparatus 449 

173. — Diagram of the course of the spinal roots and of the nerf radiculaire . . 503 

174. — Syringomyelia 583 

175. — Syringomyelia 584 

176. — Syringomyelia of the cervical enlargement 588 

177. — Transverse section through the dorsal portion of the spinal cord . . . 589 

178. — Transverse section through the dorsal part of the spinal cord . . . 589 

179. — Transverse section of a dorsal part of the lumbar cord 589 

180. — Transverse section through the medulla oblongata 591 

181.— Syringobulbia 591 

182. — The various limits of the zones of sensory disturbance upon the head . . 597 

183. — Disturbance of the pain sense 598 

184. — Infantile muscular dystrophy 634 

185. — Neurotic muscular atrophy 647 

186. — Infantile hereditary spinal muscular atrophy 650 

187. — Diagram of the motor innervation tract for the facial nerve .... 670 

188. — Facial nerve from the base of the skull to its exit 671 

189. — Diagram showing the distribution of the sensory cutaneous nerves of the 

head 678 

190. — The facial nerve and its communications 679 

191. — Distribution of the sensory nerves in the head, the trunk, and the extremities 737 

192. — Distribution of sensory nerves 739 

193. — Diagram of spinal sensation 740 

194. — The points upon which indurations are mostly found 865 

195. — Diagram 990 



THE MACROSCOPIC ANATOMY OF THE CENTRAL 
NERVOUS SYSTEM 

WITH SPECIAL REFERENCE TO THE PHYSIOLOGY OF THE BRAIN 
By M. ROTHMANN, Berlin 

At the beginning of the twentieth century the study of the macroscopic 
anatomy of the central nervous system is most necessary. As in many other 
departments of science which are apparently devoid of clinical interest, here 
also in the victorious progress of clinical research the necessity has arisen to 
reveal to the clinician such exact knowledge of the anatomical relations of 
the central nervous system as in preceding decades only the specialist in this 
branch of medicine was expected to acquire. Not merely because the enor- 
mous advance in the study of the physiology and patholog} r of the brain and 
spinal cord now enable us almost daily to localize exactly the manifold affec- 
tions of the central nervous system, but surgery has very recently found its 
realm greatly enlarged in that it now searches for an abscess deep within 
the brain or locates a tumor of the brain or spinal cord by methods of diagno- 
sis which are constantly becoming more certain; it successfully removes these, 
or even a cicatrix upon the surface of the brain which has been the cause 
of severe epileptic convulsions. In all of these diagnoses and operations accu- 
rate knowledge of the individual parts of the central nervous system, their 
blood supply, their position in the skull and vertebral column, is absolutely 
necessary. In addition, one of the greatest contributions to our clinical 
armamentarium, Quincke's method of lumbar puncture, the importance of 
which is constantly proven, in its various modifications can be practically 
utilized only when we fully understand the position of the spinal cord and 
its membranes within the canal of the vertebral column. 

If the relations of the fully developed, human, central nervous system 
are to be correctly appreciated, its embryologic structure must be under- 
stood and the simpler forms in lower animals must be compared with these. 
The central nervous system is developed from the ectoderm. The medullary 
plates arising from the originally single-layered epithelium along the median 
line of the body grow toward each other, then close and form the embryonic 
medullary tube, which dorsally for some time reveals a space. Separated 
from this embryonic medullary tube is a ganglion crest, from which the 
ganglia of the head and spine arise, and a sympathetic layer, both of which 
subsequently develop spontaneously. In the lowest vertebrate animals known 
to us, the amphioxus, in which the brain is merely indicated by a slight 
thickening at the anterior end of the embryonic medullary tube, the central 
nervous system remains permanently in this stage. In all other vertebrates, 
2 1 



2 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

however, the medullary tube at its anterior portion has two ridges which form 
three spaces, the primary cerebral vesicles, the anterior middle, and the poste- 
rior cerebral vesicles, the latter of which passes immediately into the spinal 
cord. Soon afterward the embryonic medullary tube curves in such a way 
that the middle cerebral vesicle takes the uppermost position in the entire 
central nervous system. At about the fourth week of embryonic life the 
anterior brain shows a new development, the secondary anterior brain, which 
subsequently becomes the cerebrum, in contrast to the primary anterior brain 

Metf.thalamus 

s 

Epithalamus 



\ i 



Thalamus 



Pars mamillari: 
hypothalami \ 



Pallium s <\X e M 

>N T V \^"^&: Diencephalon 




Corpora quadrigemina 



Pedunculus 
cerebri 



o 

Corpus striatum ,X''r> 

Rhinencephalon -""' •/ » ,- 

Pars optica hypothalami ' 

Lobus anterior hypophyseos ' 

Pons (Varoli) ' 



Pars ventralis 



Brachia con - 
juntiva et 
Velum medul- 
lare anterius 

rhombericephali'^' ££ 
cr 

Metphcephalon tfrp 

« --^'S f "~ Cerebellum 

[Posterior brain) y^iO 



Myeler^ceph a 1 : oi ^.._ p ars dorsalis 

O* 
O 

a 

Medulla spinalis Ji) O^. p a rs dorsalis 

i- fi) 
Spinal cord J 



Pars ventralis ^ = 



Fig. 1, — Median Sagittal Section through the Brain of a Human Embryo at the End 
of the First Month. (After W. His.) 

or inter-brain; the posterior cerebral vesicle by curving forward, the varolian 
bend, is divided into the posterior brain and the after-brain (metencephalon), 
so that five cerebral vesicles are now present and communicate with one an- 
other, being separated from the spinal cord posteriorly by the cervical flexure. 

We, therefore, differentiate the telencephalon or anterior brain with the 
olfactory lobes at the base, the corpus striatum and the pallium which later 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 



c.a. 



extends to the hemispheres of the cerebrum, the diencephalon or inter-brain 
with the thalamus opticus, the mesencephalon or mid-brain with the corpora 
quadrigemina, the metencephalon or after-brain with the cerebellum and pons, 
and the myelencephalon or cerebrospinal axis with the medulla oblongata. 
The telencephalon — above all, the pallium — from which the cerebrum of the 
vertebrate animals is formed, is the most highly developed of these divisions, 
and attains its mightiest conformation in man. While in vertebrate fish the 
simple epithelial covering is permanent, in man this finally covers to a sur- 
prising extent the entire brain. In the telencephalon the process is the same 
as in all other parts of the brain, the basal section becomes very thick while 
the dorsal portions are thin but become very broad. Hence, in the telen- 
cephalon, the closure of the medial wall of the hemispheres does not develop 
nervous elements but fills out the lateral ventricles as the choroid plexus. 
The communications of the cerebral cavities with the surface of the brain, 
which are at first free, are arrested toward the end of the fourth month by 
the development of the corpus callosum. Through this, as well as the fornix 
which lies below it, the two hemispheres of the cerebrum expand, while the 
anterior cerebral ganglia develop from the basal portion the so-called ground 
plate of the telencephalon, from the corpus striatum which is divided by the 
projection fibers which pass from the cortex through the internal capsule into 
the nucleus caudatus, and from the nucleus 
lentiformis to which the nucleus amygdalae 
and the claustrum are added. Owing to 
the adhesion of the corpus striatum to the 
thalamus opticus in the perfectly devel- 
oped brain the space between the telen- 
cephalon and diencephalon is hardly no- 
ticeable. 

Simultaneously with the formation of 
the different portions of the brain and 
the more or less greatly increased thick- 
ness of their walls, the original spaces in 
the cerebral vesicles develop into a ven- 
tricular system varying in extent and 
form. In accordance with the develop- 
ment of the hemispheres two ventricles 
form in the cerebrum; the lateral ven- 
tricle, which at first communicate with the 
surface, are subsequently shut off from 
it but communicate with each other by 
means of the foramen Monro i, through 
which also we pass to the third ventricle 
in the cavity of the diencephalon. The 
latter is limited laterally by the optic 
thalamus, and above by epithelium from 
the choroid plexus, while it passes down- 
ward in funnel shape into the infundibu- 

lum. Through the comparatively wide third ventricle we pass dorsally to the 
narrow aqueduct of Sylvius, the canal of the mesencephalon, the upper bound- 




Fig. 2. — The Development of the Ven- 
tricular System. (After Welcker.) 

IV, Fourth ventricle; r.L, Recessus later- 
alis; a., Aqua?ductus Sylvii; 727, Third 
ventricle; F.M., Foramen Monroi; c.a., 
Cornu anterius; c.p., Cornu posterius; 
c.i., Cornu inferius of the lateral ven- 
tricles; h., Pes hippocampi major. 



4 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

ary of which is formed by the corpora quadrigemina, the lateral limits by the 
structures beneath, the red nucleus, the lemniscus tracts, etc., while the cere- 
bral peduncles are found upon the floor. In the region of the metencephalon 
these canals dilate and form the fourth ventricle, the so-called rhomboid 
fossa, the roof of which is formed by a thin membrane, the tela chorioidea 
posterior, above which we find the layers of the cerebrum developed from 
the dorsal wall of the occipital vesicles; this structure with its arms which 
extend to the other parts of the brain forms the lateral boundary of the fourth 
ventricle, while the mass of the pons and the medulla oblongata spreads out 
caudally toward the base. In the medulla the fourth ventricle gradually 
narrows and becomes the central canal of the spinal cord, and in various 
ways, in adult man. being partly obliterated, extends through the entire length 
of the cord, and terminates in the conus terminalis. 

After the development of the central nervous system of man in its exter- 
nal forms, in the adult human being the greatest interest centers in the forma- 
tion of the fossa? and convolutions from the pallium. A few so-called " transi- 
tory fossae " develop from the second to the third month but subsequently dis- 
appear, after which, in the fifth month, the first permanent fossa, the Sylvian 
fossa, is formed by that portion of the pallium which communicates with 
the corpus striatum, the island of Reil, which is slower than the remaining 
parts of the pallium in growth. At first there is a flat depression which, by 
the rapid extension of the remaining pallium, constantly becomes deeper so 
that at about the middle of the fifth month the anterior and posterior por- 
tion of the fossa Sylvii is well developed while the rest of the brain is still 

Frontal lobes Central fossa Parietal lobes 




Island \ ' yJT , ' -| Occipital lobes 



Sylvian fossa Temporal lobes 

Fig. 3. — Brain of a Human Fetus at the End op the Fifth Month. 

quite smooth. At the end of the fifth month the central fossa is formed, 
and in the sixth and seventh months the remaining f ossge, which, however, are 
still primitive but permit the principal convolutions of the perfect brain to 
be distinctly recognized. 

Even to-day the importance of the formation of convolutions and fossae 
has not been positively determined. Although it is generally maintained 
that the formation of fossae in the cerebrum occurs only in the higher animals 
and in these constantly undergoes greater development, and that, as we have 
just seen, this cerebral furrowing in the embryo of man takes place compara- 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 




Fig. 4.- 



Brain of a Human Fetus at Seven 
Months. 



lively late, there is no absolute parallel between the mental development of 
the animal and the formation of fossae in the cerebrum. AYe know that some 
animals have perfectly flat brains and that in others the brain is markedly 
convoluted, yet we are unable to determine a preponderance of cerebral activ- 
ity in the latter. Of course, in 
the highest group of mammals, 
monkeys, from the lowest forms 
up to the anthropomorphic apes, 
there is an increasingly rich for- 
mation of fossae and convolutions 
which finally reaches its acme La 
the human brain. 

The importance of the fossae 
unquestionably depends upon the 
greatest possible surface distribu- 
tion of the cortex of the cerebrum 
within the limited space of the 
cavity of the skull. Although 
the ganglion cells of the cortex 
constantly increase in numbers by 
the development of the brain, and 
space is formed by their over- 
lapping each oilier in layers, the 
thickness of the cortex thus being 
permanently increased, a juxtapo- 
sition of the nervous elements is 
of great importance, above all for 
the development of the projection 
fiber systems which unite the cor- 
tex of the brain with the deeper portions of this structure. Since, as Bail- 
larger pointed out, when the brain begins to -row its volume forms the cube 
of its diameter while the surface equals only the square of its diameter, many 
convolutions must develop in the surface in order to preserve this ratio between 
volume and diameter. 

At this point it may be interesting to discuss the question in how far 
in man the weight of the bruin and its richness in convolutions may presage 
his mental development. In beginning our investigations into the brain we 
incline to the general opinion that great weight of the brain is combined 
with a great intellect. Numerous measurements made for this purpose among 
various races and also measurements in the case of noted men have proven 
that there is for this no absolute rule, but that while the brains of some 
prominent men were very large, others did not exceed the average or were 
even below it. The fact that the brain in men is, upon the average, heavier 
than in women, and that in various races the weight of the brain does not 
markedly differ, may be attributed almost wholly to the difference in the 
general size and weight of the body, with which the weight of the brain 
appears to correspond. A little reflection will also show that the nervous 
elements in the cortex of the brain which mold our psychical life determine 
also the weight of the brain rather than the basic substance of the brain, 



Fiss. Svlvii; I\, gyr. central, ant.; Ii, gyr. front. 
sup. ; /;, gyr. front, mod.; It, gyv. front, inf.; II\, 
gyr. central, post. ; Hi, lob. parietal sup.; lis, lob. 
parietal, inf.; IIIi, ^yr. occ. sup.; Hh, gyr. occ. 
med. ; Ills, gyr. occ. inf.; IV\, gyr. temp, sup.; 
IVi, v.-.\, nyr. temp. med. u. inf.; V, insula; B, 
liss. parieto-occip., lateral portion; £", sulc. cen- 
tralis; P and P, anterior and posterior portions of 
the sulc. interparietalis;Pi, sulc. postcentralis; T, 
sulc. temporalis sup. 



6 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

the amount of blood it contains, or the size of its lower parts. As the weight 
of the brain cannot be a gauge for the intelligence of a person, neither is the 
large number of fossae and convolutions a general sign of high mental devel- 
opment. Whether in persons who have achieved distinction in special direc- 
tions — mathematicians, musicians, orators, poets, etc. — definite areas of the 
brain show a marked development and an unusual richness in convolutions 
cannot be certainly determined because of the comparatively small number 
of distinguished men whose brains have been examined for this purpose. Care- 
ful researches in this direction will, no doubt, give us valuable information. 

The study of the fossae and convolutions of the adult brain of man was 
for a long time very superficial, and no great practical interest was attached 
to it, while physiology, according to Flourens' teachings, demonstrated that 
the various sections of the cortex and cerebrum were of equal importance. 
But, after the important discovery of cerebral localization by Fritsch and 
Hitzig, a triumphal progress was begun which enabled us to localize correctly 
pathologic foci upon the surface of the cerebrum, and even to operate in 
suitable cases. Then scientific interest was concentrated more and more upon 
the exact determination of the fossae and convolutions. It was soon observed 
that these are positively and inseparably related to the localization of indi- 
vidual functions, and that their position in regard to each other and to the 
bony skull was of the greatest scientific and practical significance. Hence 
it appears expedient to mention here the most important points. I should 
like to state at once that, in the living brain, after opening the dura mater, 
the membrane which closely surrounds the arachnoid and pia mater presses 
the fossae so closely together that no clear view into these fossae, some of 
which enter deeply into the convolutions, can be obtained until after the re- 
moval of the membranes. This, however, is possible in a fresh normal brain, 
and still more easy after hardening it for a few days in a 10 per cent, formol 
solution. Then the deep furrows of the cerebrum may be seen very distinctly. 
While we were formally inclined to regard the convolutions as primary, and 
the fossae as secondary, we have lately recognized that the fossae undoubtedly 
develop first, and form convolutions only after entering deeply into the sur- 
face which was previously smooth. 

The fissures may be classified as main fissures which first develop and 
are characterized by great depth, as typical secondary fissures which are much 
more superficial although they may be found in every normal brain, and as 
atypical secondary fissures which show great variations. 

Among the main fissures of the brain the Sylvian fissure must first be 
mentioned, on account of its early formation and size, as well as because of 
the importance of the adjacent portions of the brain in which prominent 
centers and association tracts for the function of speech are located. These 
consist of the ascending truncus fissurae Sylvii, the ramus posterior horizon- 
talis which ascends from the base of the brain to the lateral surface of the 
hemispheres, the posterior portion passing upward as the ramus posterior 
ascendens, while at the anterior end two small, deep, lateral fissures appear, 
one, the ramus anterior horizontalis passing forward, and the other, the 
ramus anterior verticalis running upward. Next in importance to this is the 
sulcus centralis, the fissure of Rolando, around which the centers for the 
head and extremities are situated. This fissure runs upon the lateral sur- 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 7 

face obliquely forward and downward from the middle of the median border 
of the hemispheres, usually making two bends (the superior and inferior 
genu of the sulcus centralis) and then runs downward terminating a little 
above the horizontal limb of the Sylvian fissure. The two principal fissures 
are chiefly located upon the median surface of the occipital lobe, and are 
the fissura parieto-occipitalis which usually extends somewhat upon the lateral 
surface, and the fissura cal carina, these uniting at an acute angle and termi- 
nating immediately below the splenium of the corpus callosum, in the sur- 
roundings of which in the cortex, the sense of sight in man appears to be 
situated. The sulcus corporis callosi, sometimes also known as the fissura 
hippocampi, and the fissura cltorioidea, which is scarcely perceptible in the 
developed brain, are principal fissures in the embryo but do not proceed to 
complete development. 

To facilitate its description the division of the hemispheres of the cere- 
brum into separate lobes formed as far as possible by the main fissures is 




Fig. 5. — Lateral View of the Brain. (After Edinger.) 



important. Hence, we differentiate a frontal lobe situated in front of the 
central fissure and extending downward to the Sylvian fissure, also a parietal 
lobe which begins behind the central fissure and is also limited below by the 
Sylvian fissure. The boundaries of the temporal and occipital lobes are not so 
distinct, but by the aid of the fissura parieto-occipitalis and the Sylvian fissure, 
which must be extended, these may be determined. The island situated at the 
base of the Sylvian fossa is to be regarded as a special portion of the brain. 
The frontal lobe, which in no animal known to us, not even the anthropoid 
apes, attains the development that it does in man, has three surfaces, lateral, 
median, and basal. The last is adjacent to the osseous roof of the orbit, 
and is therefore called the orbital surface. On the lateral surface we invariably 
differentiate three fissures, the sulcus prcecentralis which lies parallel with the 
sulcus centralis and runs in front of it, often bifurcating into two parts, 



8 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

the- sulcus frontalis superior and sulcus frontalis inferior, both of which run 
anteriorly from the sulcus prsecentralis toward the median border of the hemi- 
sphere. The many smaller fissures, some of which are not invariably found, 
will not be enumerated. These fissures form four convolutions: (1) The 
gyrus centralis anterior, lying between the central fossae, the sulcus prsecen- 
tralis, and the Sylvian fissure; (2) The gyrus frontalis superior (first frontal 
convolution) between the sulcus praecentralis, sulcus frontalis superior, and 
median border of the hemisphere, and extending above the latter to the median 
surface of the hemisphere; (3) The gyrus frontalis medius between the upper 
and lower frontal fissures; (4) The gyrus frontalis inferior, known as the 
third or Broca's frontal convolution, and so designated by Broca, but pre- 
viously described by Gall; Broca, however, first definitely recognized it as the 
seat of the motor speech function. This is divided by the ramus anterior 
verticalis and the ramus anterior horizontalis of the Sylvian fossa into the 
pars opercularis, the pars triangularis, and the pars orbitalis; the latter ex- 
tends to the orbital surface of the frontal lobe, as also the two other frontal 
convolutions. Here the inconstant fissures, such as the sulcus cruciatus orbi- 
talis and, extending medially from it, the sulcus olfactorius, are included; the 
latter receives the tractus olfactorius. 

The parietal lobe with its lateral and medial surfaces has but one typical 
fissure, the sulcus inter parietalis, which rises immediately above and parallel 
with the Sylvian fossa, behind the sulcus centralis, and forms an arch behind 
the occipital lobes. A small branch of this sulcus, which extends upward to 
a greater extent than and parallel with the central fissure, is called the sulcus 
retro centralis. Here are formed the following convolutions: (1) The gyrus 
centralis posterior behind the central fissure ; above this and upon the median 
surface of the hemisphere it enters the paracentral lobe and unites with the 
gyrus centralis anterior; below it enters the central fossa and joins the oper- 
culum above the Sylvian fossa. This convolution is much narrower than the 
anterior central, from which, according to the investigations of Ramon y 
Cajal, it differs decidedly in its finer structure; (2) the gyrus parietalis 
superior (the upper parietal convolution) behind the gyrus centralis posterior 
and above the sulcus interparietalis, with the precuneus at the medial sur- 
face of the hemisphere; (3) the gyrus parietalis inferior (lower parietal lobe) 
is divided below the sulcus interparietalis into gyrii, the gyrus supramargi- 
nalis and the gyrus angularis, of which the former surrounds the posterior end 
of the Sylvian fossa, while the pli courbe, much discussed in scientific com- 
bats as the gyrus angularis and the cortical center of sight, surrounds the 
posterior portion of the sulcus temporalis superior, and is therefore not 
sharply separated from the occipital lobes. 

The occipital lobe has the form of a triangle with its base upon the parie- 
tal and temporal lobes while its apex points backward ; it is called the occipital 
pole. Corresponding to this is a frontal pole upon the most anterior portion 
of the frontal lobe. Fossae are not always found on the lateral surface of the 
occipital lobe. As a rule, the main furrows are the sulcus occipitalis trans- 
fer sus which is situated transversely behind the interparietal fissure and runs 
through that area in which we find in monkeys a deep space separating the 
posterior and parietal lobes, the sulcus occipitalis lateralis, situated in the 
lower part of the occipital lobe and running close to the posterior pole, and a 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 9 

sulcus occipitalis anterior in the anterior portion of the occipital lobe near 
the extension of the posterior ascending part of the sulcus temporalis medius 
The difference in these furrows accounts for the varied formation of three 
convolutions upon the lateral surface, which like the three frontal convolutions 
extend from before backward as an upper, middle and lower gurus occipitalis. 
Each of these convolutions passes forward uninterruptedly into another con- 
volution, the upper into the gyrus parietalis superior, the middle into the 
gyrus angulans and the lower into the middle temporal convolution 

Before describing the median division of the occipital lobe, we must first 
consider the temporal lobe which occupies the lowest part of the cerebral 
hemisphere, and is limited anteriorly and above by the Sylvian fissure while 
posteriorly it passes into the parietal and occipital lobes without a sharply de- 
fined Inn, at.on. Here, following each other from above downward and'run- 

n rfofTl ' th :A 1Viim f ° SSa ' are the SUICUS Um P° raUs ^'P^or, a 

h zontal ™ ! "I'" 1 the g '' rU ,. al ' gl,lariS - aml Which haS «*» a* anteror 

a Tc d,l i P T 1 f Cendmg P ° rti0n ' the Suhus ^oralis medius, 
Wr „ f M ,r, the .«''«'* tempo-raits inferior winch extends along the 
Wr surface of the hemisphere, and the sulcus occipito-temporalis inferior. 
Here are also three convolutions upon the lateral surface, the gyrus temporalis 
superior which onus the lower boundary of the Sylvian fossa and upon whose 
surface externally a few invisible gyrii temporalis transversi extend to the 
stand, * gyrus temporalis media, and inferior, the latter partly upon the 

—)/ M r 6 h6miSphe ? i "" , Uh "" V ' " the lattei »- "">■'- ^Pito- 

interior and the occipito-temporalis inferior which to a great extent forms 
posteriorly the basal surface of the occipital lobe; finally, the oy^occiZ- 
temporalis medialis or gyrus lingualis which is 'situated between he stflcus 

one P of tt mPOrall fi S lnfen ° r an *' fte flSSUra Ca,Carina P-ioush- described a 
one of the main fissures on the median and lower surface of the hemisphere 

nto 1 et ■" J 11 " 10 * 4 enti - 1 ;."" h - «* occipiU lobe. Anteriorly it passes 
into the gyrus hippocampi winch will shortly be described 

hJnJTtT that V °^ ° f th ° '""'""' lur f ace °f the hemisphere which 
as n„t yet been considered we sec the gyrus fornicatus passing from before 

Son the ar0W ' d & u arC " Hke C ° rpUS Call0Smn - The ll >.pcr portion, stated 

uZt e t corpus M r:: 1 ' is caIIed the n™ dn ^ iL ^ ***, *»***£ 

tenor port on T 1 ' ^ T hi '''"""'"'^ &<** are united upon the pos! 
tenor portion of the corpus eallosum by the narrow isthmus-gvri fornicati 

ScurtXeTh ° n ° f "Tf™ ,,ipP ° Campi Sh ° w8 a mark ^ thicken ng 
and curvature the «•«., which must be considered as being within the ante! 

n rC Tthe fTt *** "* "** * ^^ * a P-es7in tt 
interior of the ventricle, the cornu ammonis. Corresponding to these con- 

tte utTr ,i r ?% "^ Cal ' ° Sl,m - ^ "° te the «**•«*»* oXi 
ioin the PP „ „ ° , e fi:' US hl P? ocam ^ the fis™™ hippocampi, which 

or onic / 7 ',' a " d the "" rri Lmcisii in man - apparently being em- 

An ZZ :Z t ° nS " h ; i Ch P r int ° the Wer horn of th * lateral ventricles. 
An important fossa is the sulcus calloso-marginalis or sulcus cinguli which 

lotm aS a a nVn C a ab ° Ve "»****? Poorly from the genu o/tle clrpus 
eaiiosum, and passes upward over the splenium of the corpus callosnm L<\ 
^mediately behind the central fossa into the lateral surface of the hemi 



10 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

sphere. Anterior to this is a secondary fossa of this sulcus extending verti- 
cally; above it is the sulcus paracentral is. 

Among the anterior convolutions on the median surface of the hemisphere 
we find the gyrus frontalis superior and a few of its subdivisions, then the 
one mentioned above, the lobulus paracentralis, which corresponds to the cen- 
tral convolutions between the sulcus paracentralis and the ascending portion 
of the sulcus calloso-marginalis behind the precuneus, it is of a square form, 
and reaches posteriorly to the parieto-occipital fissure. This is joined dorsally 
by the triangular cuneus between the last mentioned furrow and the fissura 
calcarina, the anterior point of which runs as the pedicle of the cuneus to the 
isthmus gyri fornicati; finally we observe the gyrus descendens which passes 
downward behind the fissura calcarina to the gyrus lingualis, which was de- 
scribed when outlining the temporal lobe. 

A brief description of the island must be given. The island of Reil is 
covered by the surrounding portions of the brain, and externally immediately 




Fig. 6. — Longitudinal Section through the Middle of the Human Brain. 

(After Edinger.) 



adjoins the ganglia of the cerebrum. Around this structure runs the sulcus 
circularis Reilii; anteriorly and ventrally the island passes into the free 
structure at the base of the brain, and at the limen insula? directly into the 
lamina perforata anterior. A permanent sulcus, the sulcus centralis insula?, 
divides the island into two lobes; the larger anterior (pars frontalis) pos- 
sesses three to four gyri breves, the smaller posterior (pars parieto-occipitalis) 
has one to two gyri longi which pass to the temporal pole. The region of 
the island, which is especially developed in man as the association region of 
human speech, shows in animals a gradually ascending development. Waldeyer 
found in his investigations of the brain of anthropomorphic apes that the 
construction of the island in the monkey is fundamentally the same as in 
man; there is first an arching convolution with strongly linked frontal and 
weaker temporal arches; an increased development is apparent as we pass 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 11 

from the gibbon to the orang-utan, the chimpanzee and the gorilla, the struc- 
ture in the last named most closely resembling that of man. 

The only rational method for fixing in the mind the fossae and convolu- 
tions of the brain is to study the hardened brain, preferably that freed from 
its membranes. The great variety of fossae and convolutions will then be 
apparent; it is frequently difficult to recognize an individual fossa unless 
typical fossae, such as the Sylvian or the sulcus centralis, are chosen as the 
starting point for the investigation. The whole cortex of the brain with 
its fossae and convolutions becomes interesting to the practitioner with the 
development of cerebral localization, which shows that definite areas of the 
cortex of the brain are the origin of definite functions, and that after the de- 
struction of these parts of the brain the functions cease or show deterioration. 
This truth, which in part was taught by Gall at the beginning of the nine- 
teenth century, so that, notwithstanding many errors, this writer deserves an 
honorable position among the investigators of the brain, was subsequently 
promulgated by Broca who developed the localization of the function of 
speech, but it was generally accepted only when Fritsch and Hitzig in 1870 
for the first time demonstrated the faradic irritability of definite areas of 
the cortex, after which these authors began by experiments and extirpation 
to determine the symptoms of disease in these areas. It would lead us too 
far afield to discuss this subject fully, and I will merely state that through 
the untiring labors of men like Hermann Munk, Goltz, Ferrier, Horsley, 
Beevor, Schiifer, Sherrington, and others, a structure has been erected which 
reveals to us many of the functions of the cortex of the brain, and this has 
been a prerequisite for surgical successes in this difficult realm. To men- 
tion briefly the most important, the region of the central convolutions and 
their immediate surroundings is to be regarded as the seat of sensation of the 
body, and is the region in which sensations coming from the periphery of the 
body are expressed in movement, the uppermost portion of both central con- 
volutions and the paracentral lobe controlling the lower extremity, the middle 
portions of the central convolutions the upper extremity, the lower portion 
of the central convolution the facial nerve, and, still lower, the operculum, 
controlling the mouth, the tongue and the larynx. Contractions of definite 
muscle groups upon the opposite side of the bod} r originate from definite 
areas of these so-called " centers " ; these, however, can be produced only by 
the surface portions of the convolutions, and extirpation of entire centers may 
cause paresis in the affected extremities. It must be borne in mind that not 
isolated muscles but muscle synergists are located in the cortex of the cere- 
brum, and these are the better developed the more they are consciously called 
into action. In man, the surgery of the brain has exposed but little of the 
cortex of the brain to observation, and we must therefore depend chiefly upon 
investigations of the brain of anthropomorphic apes. Beevor and Horsley 
have made careful experiments in orang-utans, and these have been almost 
absolutely confirmed by the results in man; the recent experiments of Griin- 
baum and Sherrington in eleven ( !) anthropomorphic apes (gorilla, chim- 
panzee, orang-utan) have in the main confirmed these, but have shown that 
only the anterior central convolution is stimulated b}^ weak faradic currents. 
But it must be emphasized that the results of irritation only confirm the 
position of individual centers which, as is especially clear from the classical 



12 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

investigations of H. Munk, are sharply separated from one another in their 
entire circumference and occupy a much larger area. In front of the cen- 
ters for the extremities is the center for the muscles of the back of the 
neck, and the center for the muscles of the trunk is in the frontal brain 
which has not as yet been accurately studied. The sense of sight is in 





Fig. 7. — Cortex of the Cerebrum of Apes. (After H. Munk.) 
A, Center for sight; B, center for hearing; C—I, sensory sphere; C, hind-leg region, sensory- 
sphere; D, fore-leg region; E, head region; F, eye region; G, ear region; H, neck region; /, 
trunk region. 

the occipital lobe, that of hearing in the temporal lobe. Besides experi- 
mental investigations among which those of H. Munk are here also to 
be regarded as pioneer — we owe to him the recognition of mind blindness 
and mind deafness which have since become of the greatest importance in 
human pathology — valuable studies have been made of the pathological affec- 
tions of these areas in man. The center for most acute vision does not appear 
to be in the lateral surface of the occipital lobe but in the region of the 
fissura calcarina; it is impossible, however, at this point to discuss minutely 
the complicated conditions which produce hemianopsia. The relations of the 
temporal lobe to the sense of hearing are similar to those of the occipital 
lobe to sight, but they have not yet been accurately studied. It is usually 
the upper temporal convolution which is considered ; but the relations in man 
are much more complicated than in the most highly organized animals be- 
cause of the power of speech which, when these areas are diseased, shows im- 
pairment known as sensory aphasia; this corresponds with a much rarer patho- 
logic picture, optic aphasia, in destruction of the communications of the 
occipital lobe with the other " speech centers." The centers for movements 
of the eye and ear are not the same as the centers for sight and hearing; 
on the contrary, the centers for optical movements appear to be localized 
in two areas, in the gyrus annularis of the parietal lobes immediately in front 
of the center of sight and in the region of the second frontal convolution, while 
aural movements originate in the posterior division of the first temporal con- 
volution. In man the third, or Broca's, frontal convolution must be borne 
in mind; here closely joined are localized the centers for the muscle groups 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 13 

brought into play in motor speech function, the destruction of which there- 
fore, produces the well known picture of motor aphasia with its various modi- 
fications. Y\ e cannot here discuss the enormous extension of the localization 
of speech disturbances in the teachings of the last few years. In the physio 
logic localization of the centers for smell and taste, nothing decisive has been 
revealed. Comparative anatomical researches, it is true, point to the reo-ion 
of the gyrus hippocampi, the gyrus fornicatus, the fascia dentata, and, above 
all, to the cornu ammonis as the center for the sense of smell, since these 
portions of the brain are highly developed if the bulbus olfactorius is promi- 
nent, and in the dolphin in which the lobe of smell is absent they are cr rea tly 
decreased; therefore these areas of the brain together with the lobus olfactorius 
are known as the rhinencephalon. 

Nevertheless, the study of cerebral localization is far from being exhausted; 
on the contrary, it still promises rich results ; definite lobes of the brain and 



Anus and Vagina 



Sulcus centralis 



Shoulder 
Elbow 

Fingers and j$' \ ^xN^uV'.'- 



^. Abdomen 
-Ches£ 




Sulcus centralis 



Mastication 



Vocal cords 



-Left Cerebral Cortex of a Female Chimpanzee (Troglodytes niger), showing 
results of electric irritation. (After Grunbaum and Sherrington.) 



the 



definite convolutions included within them are the centers which control 
special functions, hence it follows that this division of the cerebral cortex 
into fossae and convolutions is physiologically important. While, however, 
m the human brain at least, some centers have been recognized, there remain 
large areas of the cortex of the brain which at present permit no recognition 
ot the localization of definite cerebral functions, and Flechsig has attempted, 



14 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

by the same method which he instituted so successfully in the investigation 
of the spinal cord and the development of the medullary sheath, to divide 




Fig. 9. — Horizontal Section through the Brain with the Nucleus Caudatus, Thala- 
mus Opticus, and Nucleus Lentiformis. (After Edinger.) 

the cerebral cortex into separate areas in which, besides the projection cen- 
ters just described which are connected by the corona radiata fibers with 
deeper sections of the central nervous system, he differentiated as higher 
psychical centers association centers said to be almost entirely free from fibers 
of the corona radiata and therefore parallel with Munk's centers of special 
sense from the fact that they unite the activity of the latter to higher units. 
After finding these three main association centers, a larger posterior area in 
the parietal lobe and the lateral portion of the occipital brain, a middle area 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 15 

in the island, and an anterior one in the anterior region of the frontal brain, 
and to facilitate the more comprehensive embryologic investigation of the 
brain, Flechsig differentiated many similar but smaller centers. Whether 
the teaching of Flechsig, whose anatomical and physiological theory has been 
often attacked, is correct is by no means yet certain, although its fructifying 
effect upon the study of the brain is undoubted. 

So far we have exclusively considered the cerebral cortex, which in man 
represents the dominating portion of the brain, and we now turn to the ganglia 
of the cerebrum, situated in the interior of the brain, and separated from the 
cortex by white medullary masses. To these primarily belongs the trunk 
ganglion, the cor/jus striatum, which in the early embryonic stages and in the 
lower animals is a uniform body, but in the fourth month and subsequently 
is divided into two parts by fiber masses which pass down from the pallium; 
also an external one, the nucleus leu I i for mis, and an internal one, the nucleus 
caudatus, while the fiber mass which separates them is called the internal 
capsule. The nucleus caudatus is a large gray mass which is directed forward 
into the lateral ventricle; its broadest part is the anterior extremity or 
" head " in front of the thalamus opticus, constantly becoming narrower pos- 
teriorly, and terminating laterally as a narrow, medullary fold, the stria ter- 
minalis, which runs posteriorly downward, at last anteriorly, and at the lower 
horn in the neighborhood of the apex of the temporal lobe it terminates as the 




Fig. 10. — Portion of a Median Section through the Cerebrum. (After Obersteiner.) 
CAm, Cornu ammonis; Cell, corpus callosum; cell, sulcus corporis callosi; clc, fissura calcarina ; 
Cng, gyrus cinguli; Cva, commissura anterior; Fcl, columna fornicis; Fcp, corpus foraicis ; 
Fcr, crus fornicis; Fd, fascia dentata; Fi, fimbria; Gee, genu corporis callosi; H, gyrus 
hippocampi; Rcc, rostrum corporis callosi; Spec, splenium corporis callosi; Splc, septum 
pellucidum; Stlm, stria longitudinalis med. 



"tail." Outward from the nucleus caudatus is the nucleus lentiformis, a 
wedge-shaped mass of gray matter almost wholly separated from the former, 
and entirely from the thalamus opticus by the fibers of the internal capsule; 
neither anteriorly nor posteriorly does it reach as far as the nucleus caudatus ; 
it is separated into three divisions by thin medullary sheaths of which the 
.medial portion is of lighter color and is known as the globus pallidus, while 



16 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

the outer, darker, and largest portion is called the putamen. The lateral 
surface of the putamen is parallel with the island of Reil from which it is 
separated by a narrow gray streak, the claustrum ; and between the latter and 
the putamen runs the narrow medullary lamina, the external capsule. Poste- 
riorly to the nucleus lentiformis and close to the tail of the nucleus caudatus, 
communicating with both, is the sharply defined gray mass, the amygdala, 
which is also united with the hippocampus major. The relation of these gray 
ganglia to each other and to the adjacent portions of the brain, and their 
physiologic importance in man and in the higher mammals, can be deter- 
mined only by microscopic and comparative anatomical examination, and in 
spite of many experiments and pathologic observations we are still completely 
in the dark concerning these. 

The cerebral hemispheres are united with one another by several com- 
missures: (1) The most important of these is the corpus callosum which is 
seen upon the floor of the great medial, longitudinal fissure. Besides this 
portion of the corpus callosum, called the truncus, we find also the posterior 
splenium corporis callosi which is thickened and lies above the corpora quad- 
rigemina, anteriorly bending somewhat downward the genu corporis callosi, 
which may be followed backward to the fornix as a narrow line, the rostrum. 
Between the corpus callosum and the rostrum the septum pellucidum spreads 
out in the form of two thin plates and contains the ventriculus septi pellu- 
cidi. (2) Below the corpus callosum the fornix unites both hemispheres in 
the form of a narrow band (the fimbria), passing out of the lower horn of 
the lateral ventricles at each side in the region of the cornu ammonis, uniting 
upon the two crura fornicis, passing along the lower surface of the corpus 
callosum, to divide in front at the lower end of the septum pellucidum into 
two columns, the columnar fornicis; the latter extend to the base of the brain, 
to the corpora mamillaria. (3) The anterior commissure as a white band 
joins the hemispheres in front of the columnar fornicis, which unite the basal 
portions of the temporal and frontal lobes. 

In their entire extent the cerebral hemispheres are permeated by cavities, 
the lateral ventricles mentioned above. These cavities pass arch-like from 
above anteriorly downward posteriorly and permit the differentiation of a 
middle portion, the cella media, and three horns, the anterior horn which 
passes into the frontal lobes, the posterior horn entering the occipital lobe, 
and the lower horn extending to the temporal lobes. The cella media shows 
upon its floor, besides other structures, the tail of the nucleus caudatus, while 
above is the middle portion of the corpus callosum. The anterior horn passes 
around the head of the nucleus caudatus into the posterior portion of the 
temporal lobe, and is bounded above by the corpus callosum, while medially 
it reaches the septum pellucidum. The posterior horn runs dorsally close 
to the occipital pole. The most important structure upon its base is the calcar 
avis or pes hippocampi minor, which is produced by the sinking in of the 
fissura calcarina on the medial surface of the occipital lobe. Here are also 
a number of fiber strands which play a role in the law of cerebral localization ; 
the optic radiation of G-ratiolet which passes from the thalamus opticus to 
the occipital lobe, the unilateral interruption of which produces hemianopsia, 
and the fasciculus longitudinalis inferior, which connects the temporal and 
occipital lobes. The posterior horn is covered from above by the fiber masses 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 17 

of the tapetum coming from the posterior portion of the corpus callosum. 
The lower horn passes downward and forward close to the tip of the temporal 
lobe and is covered on the upper side by the tapetum, also by the finer proc- 
esses of the tail of the nucleus caudatus which here passes into the nucleus 
amygdalae. The cerebral structures arranged upon the floor of the lower horn 
are very complicated. Here we find all of the structures with which we have 
become familiar in the description of the medial surface of the hemispheres, 
the gyrus hippocampi, the fascia dentata, the fimbria, the marked swelling of 
the cornu ammonis (pes hippocampi major), and the prominence of the emi- 
nentia collateralis Meckelii due to the deep entrance from without of the 
sulcus occipito-temporalis inferior which permits a passage to the posterior 
horn. 

The lateral ventricles do not directly communicate with each other but 
are indirectly united by the foramen Monroi, by the open space on each side 
between the thalamus opticus and fornix, and also between the lateral ven- 
tricles and the third ventricle. All of these ventricles are now filled by the 
plexus chorioidei, profuse vascular twigs covered by an epithelial layer; the 
latter corresponds to the inserted remains of the medial wall of the hemi- 
sphere and the roof of the inter-brain. Here we differentiate first a triangu- 
lar middle leaf, the tela chorioidea ventriculi tertii, which is spread out under 
the fissura transversa cerebri (Bichat's furrow) situated under the splenium 
corporis callosi and the columnae fornicis, thence the vascular villi reach the 
lateral ventricles by way of the foramen Monroi where they chiefly occupy the 
cella media and the lower horn as the plexus chorioidei lateralis, and then pass 
to the most anterior point <>f tin- latter. 

The white medullary masses situated below the cortex of the cerebrum of 
both hemispheres are called the centrum ovale. Among the various projec- 
tion fiber tracts here included, such as the corona radiata and the association 
tracts which have been thus far investigated, we shall minutely concern our- 
selves only with the internal capsule, i. e., that portion of the medulla of the 
hemispheres formed in thick bundles in which projection fibers are inserted 
by the lenticular nucleus from without, the caudate nucleus and the optic 
thalamus from within, a region especially important because here originate 
the hemorrhages which produce hemiplegia and its sequels. As the tracts 
are embraced within a narrow space formed by various parts of the cerebral 
cortex, and their fibers do not interlace, and as the tracts which pass to the 
cerebral cortex from the large ganglia, especially from the thalamus opticus, 
begin to separate and later pass to the corona radiata and the various cortical 
centers, a comparatively small focus may cause extensive symptoms as to 
motility, sensation, and the higher spheres of special sense, while these may 
be definitely localized according to whether such a focus is situated anteriorly 
or posteriorly. The internal capsule forms an obtuse angle in the genu of 
which the two crura meet; the anterior, smaller, and physiologically, so far 
as our knowledge extends, less important crus, the pars frontalis, is situated 
between the nucleus caudatus and the lenticular nucleus. The posterior, longer 
crus, which is most frequently affected and which is the usual seat of cere- 
bral hemiplegia, the pars occipitalis, runs between the thalamus opticus and 
the lenticular nucleus and extends beyond the latter more occipitally. Hence 
the entire internal capsule may be divided into four portions, the ventral, 



18 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

the lenticulo-striate, the knee portion, the lenticulo-optic, and the retro- 
lenticular part. Not to enter minutely into the exact division of the fibers, 
it need only be stated that the pyramidal tract, which is the most important 
and the only direct means of communication between the cerebral cortex and 
the spinal cord, runs in the anterior half of the lenticulo-optical portion, 
therefore in the posterior peduncle of the internal capsule. Starting at the 
knee we pass to the beginning of the posterior third of the lenticulo-optic divi- 
sion, therefore the irritative points in the various groups of muscles of the 
body (which we have chiefly learned to recognize by the studies of Beevor 
and Horsley in anthropomorphic apes) are situated in this area, from before 




Movement , 
. Eyes to the opposite side 
/ „, Eyes to the same side 
S /' ^ Mouth to the opposite side 

" .,-- Head to the same side 
•-"""'^^ Tongue 
— '_,— Retraction of the mouth 

Shoulder 

Elbow 

— Wrist 

-^~"-- Fingers 
rj"^- Thumb 
*-0"" Trunk 
\*^ Hip 

^x ^ Region of the root of foot 
^X v > Knee 
NX Hallux 
X> Toes 



Fig. 11. — Arrangement of the Motor Fibers in the Anterior Capsule. 

and Horsley.) 



(After Beevor 



backward, for the head, arm and leg. Uniting with the latter in the posterior 
third of the lenticulo-optic division is the sensory or lemniscus tract, and 
dorsally from this, in the retro-lenticular division of the internal capsule, the 
optic radiations of Gratiolet which pass to the occipital lobes. For this rea- 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 19 



son foci in the anterior portion of the posterior peduncles of the internal 
capsule produce uncomplicated hemiplegia, while the further the focus extends 
posteriorly the earlier hemianesthesia and 
even hemianopsia appear. 

Within the region of the internal cap- 
sule of the cerebrum we find the inter- 
brain whose most important structure is 
the optic thalamus, but which also in- 
cludes the pineal gland or epiphysis, the 
ganglion habenulce and, at the base of 
the brain, the corpora mamillaria, the 
chiasma nervorum opticorum, and other 
structures. The two optic thalami form 
the borders of the third ventricle, being 
directly connected with the gray sub- 
stance of its cavities. The mighty gray 
masses of the optic thalami are divided 
into several parts which may readily be 
recognized macroscopic-ally after trans- 
verse section. These nuclei of the optic 
thalami are the following: The tuber- 
culum anterius, the median nucleus, the 
ventral nuclei groups differentiated by 
v. Monakow in his experiments as vent, a, 
vent. b. vent, e, and vent ant. Dorsally 
from these are the lateral nucleus and 
the pulvinar; finally, at the point where 
the optic tract enters the brain on tin 1 
lateral side of the cerebral peduncles, 
the corpus geniculatum externum and 
the corpus geniculatum internus adjoin- 
ing medially the pedunculus cerebri from 
the external geniculate body. The cor- 
pus mamillare situated upon the base of 
the brain may be included with the nuclei 
belonging to the thalamus opticus; it 
is united with the hippocampus major. 
Nearly the entire structure of the thal- 
amus opticus has been investigated; its 
connection with the cortex of the cere- 
brum was discovered by v. Monakow during his pioneer investigations in new- 
born animals, by which he was able to prove that certain regions of the optic 
thalamus are intimately related to definite divisions of the cortex of the brain, 
and that wdien the latter, the so-called portions of the cerebrum, are removed 
they become atrophic. Certainly it might be proven that all of the nerves 
of special sense are connected with the cerebrum by means of the optic thal- 
amus; for instance, the cortical lemniscus, the tract of sensation for the body 
through the ventral nucleus of the thalamus, the acoustic tract through the 
corpus geniculatum internus, the optic tract through the corpus geniculatum 




Fig. 12. — Horizontal Section through 
the [nter-brain, Oxk-half Cextime- 
ter Below the Surface of the Thala- 
MUS AND IIH. XrcLEUS Caudatus. (Af- 
ter Obersteiner.) 

Com, Commissura mollis; Fcl, columna for- 
nicis ; Fcr, eras fornicis; Gcc, genu corporis 
callosi ; </h, ganglion habenulae; Olp, gland- 
ula pinealis; Xa, nucleus anterior thai- 
ami; AY, nucleus caudatus; XI, nucleus 
lateralis thalami; Nm, nucleus medialis 
thalami; Pdc, pedunculus gland, pinealis; 
Qa, anterior; Qp, posterior, corpora quad- 
rigemina; Spec, splenium corporus callosi ; 
Spl, septum pel lucidum; Sic, stria cornea ; 
Vli, lower horn of the lateral ventricle; 
Vlp, posterior horn; VS, third ventricle. 



20 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 



externus and the pulvinar. v. Monakow's investigations, which have recently 
been absolutely confirmed by Probst in another way, therefore show that the 
thalamus opticus is a receptacle for the fibers coming from deeper portions of 
the brain and from other trunk ganglia, also for the corticifugal fibers, while 
the structure sends comparatively few fibers downward. It is chiefly an organ 
of transmission between the periphery and the cerebrum, but our knowledge 
of its minute physiologic and pathologic function is extremely limited. 

The space between the two optic thalami, the third ventricle,, is bordered 
above by the plexus chorioideus, at its posterior end by the pineal gland or 
the epiphysis, close to the anterior division of the corpora quadrigemina. Im- 
mediately in front of it and equidistant at each side from the posterior region 
of the thalamus is the small ganglion habenulw. The epiphysis consists of 
solid, very vascular, epithelial tubules which contain peculiarly layered calcium 
concretions, the brain sand. We know nothing of the importance of the 
epiphysis in man; comparative anatomy, however, teaches us that this is 
a rudimentary organ which, in the lower vertebrate animals, extends forward 
as a long tube, its frontal end forming externally the frontal spot which is 
perceptible on the skull between the orbital spaces; a few saurians also, con- 
tain a second frontal "parietal organ," the terminal vesicles situated within 
one of the spaces of the parietal bone, its structure closely resembling an eye 

with cornea, lens and retina. In its lower 
portion the third ventricle shows the tuber 
cinereum which extends to the base of the 
skull, the cavity of which is called the in- 
fundibulum. The structures below the 
thalamus opticus compose the subthalamic 
region-, but this area has been but little in- 
vestigated microscopically and seldom gives 
opportunity for macroscopic examination. 
Immediately below the optic thalamus is 
the nucleus ruber tegmenti which a little 
lower passes into the corpora quadrigemina, 
and outside of this is the corpus sub- 
thalamicum, Luys's body. At the base of 
the brain the cerebral peduncles run to the 
pons; here is situated the pes pedunculi 
which receives the fibers from the internal 
capsule in the form of an accumulation 
of markedly pigmented ganglion cells, the 
substantia nigra Soemmeringi, which are 
distinct from the fibers of the lemniscus 
situated above. 

We now reach the region of the mid- 
brain, the principal structure of which 
is the corpora quadrigemina, so called be- 
cause here, immediately behind the third 
ventricle and the epiphysis, four small eminences are recognizable, two in 
front and two behind ; each of these structures possesses an arm, the anterior 
and posterior superior peduncle which passes to the inter-brain. The anie- 




Fig. 13. — Diagram of the Cerebral 
Peduncles. (After Obersteiner.) 

Qa, Anterior corpora quadrigemina; 
AS, aqueduct of Sylvius; Fls, pos- 
terior longitudinal bundle; Tg, teg- 
mentum; Nt, red nucleus; SnS, sub- 
stantia nigra Soemmeringi ; 1, frontal 
pontal tract; 2, tract of the motor 
cranial nerves; 8, pyramidal tract; 
4, tract of muscle sense; 5, sensory 
portion of the fibers of the cerebral 
peduncle; 6, bundle from the lem- 
niscus to the foot; 7, stratum inter- 
medium. 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 21 

rior eminence in man is exceedingly small compared with that of the lower 
animals, for its function as the center of the optic nerve is almost entirely 
merged in that of the occipital lobe and the thalamus opticus. Immediately 
below its center is the narrow posterior continuation of the third ventricle, the 
aqueduct of Sylvius; at either side extending downward is the region of the 




Fig. 14. — The Cerebellum from the Dorsal Side. (After Edinger.) 



tegmentum which, as we have just seen, is separated from the foot of the 
cerebral peduncles by the substantia nigra. The posterior eminences appear 
macroscopicallv to l>o more sharply defined than the anterior. In all trans- 
verse sections of the corpora quadrigemina the red nucleus may be recognized 
as a round red structure, lying dorsally from the aqueduct of Sylvius and 
near the median line; in this structure generally terminates the brachium 
conjunctivum which comes from the cerebellum and has just crossed it. Be- 
tween the two red nuclei are the nuclei and fiber bundles of the third cranial 
nerve, the oculomotor, the exit of which at the base of the brain will be later 
described, and behind it are the nuclei of the trochlear nerve. 

The aqueduct of Sylvius after dilating becomes posteriorly the fourth ven- 
tricle, the rhomboid fossa, the roof of which is formed by the cerebellum, the 
floor and sides by the continuation of the foot and tegmentum of the mid- 
brain which form the pons. The foot of the cerebral peduncle is here covered 
by large, transverse, running masses of fibers and is permeated by them, while 
the tegmental fibers receive their peculiar configuration from the disappear- 
ance of the red nucleus and by the passing to and fro of the fibers from the 
cerebellum. 

The cerebellum itself bears undoubtedly an alternate relation to the cere- 



22 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

brum, for in animals in which the latter is markedly developed the former 
is likewise, and when there is atrophy of one hemisphere of the brain, atrophy 
of the crossed half of the cerebellum is also observed. The cerebellum is com- 
posed of the vermis situated in the middle and of two hemispheres. It is united 
with the red nucleus and the thalamus opticus by the anterior brachium con- 
junctivum, sends the middle brachium conjunctivum or pontal arms to the 
pons, and by means of the posterior brachium conjunctivum, the corpora 
restiformia, is connected with the medulla and the spinal cord. The vermis 
of the cerebellum is divided into lobes by furrows; the most important which 
lie dorsally upon the upper vermis, and extend from before backward, are the 
lingula, the central lobe, and the monticulus, and upon the lower vermis the 
nodulus, uvula, pyramis and the tuber vermis. The hemispheres of the cere- 
bellum show above, dorsally, and anteriorly a quadrangular lobe at the side 
of the monticulus, and posteriorly the lobulus semilunaris superior; below, 
besides the nodulus, is the flocculus, upon the uvula the tonsilla, laterally from 
this the cuneiform lobe, to which the inferior posterior lobe is posteriorly 
joined. The vermis and hemispheres possess a medullary nucleus; in its 
interior, particularly in the vermis, gray nuclei are found, also the corpus 
dendatum at the boundary of the vermis and hemispheres, and medially from 
this the embolus, the nucleus globosus, and the tegmental nucleus. From the 
cerebellum over the fourth ventricle to the corpora quadrigemina passes ante- 




Fig. 15. — The Cerebellum from the Ventral Side. (After Edinger.) 



riorly the velum medullar e anticum, posteriorly the velum medullare posticum, 
and these extend to the posterior columns of the spinal cord. The plexus 
chorioidei, situated in the region of the ventricles, and which also passes into 
the sinuses of the fourth ventricle and into open spaces the largest of which 
is the foramen of Magendie, permits communication with the arachnoid space ; 
this is of importance in equalizing variations of pressure. The manifold 
communications of the cerebellum with most of the other structures of the 
central nervous system by means of the brachium conjunctivum cannot here 
be mentioned; they have become known only by minute comparative anatom- 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 23 



ical investigations in degeneration. It is certain that the cerebellum is indi- 
rectly connected with the cortex of the cerebrum by the pontal arms, to the 
brachium conjunctivum by the red nucleus and thalamus opticus, and thus 
indirectly with the cerebrum, while the corpus restiformia communicates with 
the cerebrum from the spinal cord and medulla. 

The cerebellum has recently acquired a special importance because, owing 
to tumors and suppurations in this region or in its immediate vicinity, it has 



\Vma 




Pos 

Fig. 16. — Combined Sagittal Section through the Stem of the Brain - , Twice Enlarged. 

(After Obersteiner.) 

Brcj, Brachium conjunctivum; Cm, corpus niammillare; Cop, posterior commissure; DLm, 
Lemniscus decussation; DPy, pyramidal decussation: F)ig, funiculus gracilis; Jf, infun- 
dibulum; Lm, lemniscus; Na, anterior nucleus of the thalamus; Xg, nucleus gracilis; No, 
olivary nucleus; Ntg, red nucleus; Oaa and Oac, ventral and dorsal secondary olive; Po, 
pons; Pp, pes pedunculi; Py, pyramid; Qa and Qp, anterior and posterior corpora quad- 
rigemina; SnS, substantia nigra; Tho, thalamus opticus; Yg, anterior column ground 
bundle; Vma, velum medullare anterins. 

been laid bare, and has been operated upon. Experimental and pathologic 
observations have conclusively shown that the cerebellum serves to maintain 
equilibrium, for which it is especially designed by its connection with the 
varied sensory and motor apparatus of the central nervous system. Disturb- 
ances arising after affections of the cerebellum are usually referred to the 
vermis, while but little is known of the hemispheres. At all events the fact 
is interesting that by electric irritation of the cortex of the cerebellum mus- 
cular twitchings similar to those originating in the cortex of the cerebrum 
may be produced, only they do not occur in the extremities of the same, but 
in the opposite, side. 

As the fourth ventricle in its path toward the cauda gradually becomes 
smaller, and the pyramids pass ventrally to the surface, the comparatively 
large pons enters the medulla oblongata, which also gradually becomes nar- 
rower. At the point where the pons passes into the medulla, the abducens, 
facial and auditory nerves originate, while the trigeminal nerve arises in the 
lateral portions. 

The lower olive (nucleus olivaris inferior) lies alongside the very promi- 
nent pyramids, which are characteristic of the medulla upon all transverse 



24 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

sections up to the lemniscus decussation, which then becomes visible medially 
as an open, peculiarly serrated, arc-like body behind the pyramids. In the 
upper portions of the medulla, dorsally and laterally, we note the large corpus 
restiforme which in its downward path rapidly decreases in size and finally 
disappears. On the other hand, the lower half of the medulla oblongata after 
the fourth ventricle has closed to the narrow central canal, dorsally of the 
latter, the nuclei of the spinal posterior columns, the nucleus gracilis and the 
nucleus cuneatus appear, while from the pons up to the upper spinal cord the 
so-called ascending root of the trigeminal nerve (which in reality degenerates 
downward in combination with the substantia gelatinosa which passes into 
the posterior horn of the spinal cord) becomes prominent. The nuclei of the 
cranial nerves from the ninth to the twelfth are situated in the dorsal portion 
of the medulla, some immediately upon the floor of the fourth ventricle; 
they are insusceptible to macroscopic investigation. Medially behind the 
pyramids is the lemniscus layer which, immediately above the well known 
pyramidal decussation, forms the lemniscus decussation. 

Before passing to the study of the spinal cord which begins at the height 
of the pyramidal decussation, we will briefly review the structures at the base 
of the brain, arranged in an antero-posterior direction. Their relations 
are of practical importance inasmuch as we know there are a number of 
basal processes, tumors, syphilitic exudates, etc., in which an exact, local 
diagnosis necessitates a knowledge of the sequence of these individual parts, 
particularly the order in which the cranial nerves are affected. Most ante- 
riorly at the base of the brain and below the frontal lobes are the olfactory 
bulbs, which narrow posteriorly toward the olfactory tract. Behind them is 
the substantia perforata anterior, above which thin white strands, the roots 
of the olfactory nerve, pass into the medulla of the hemispheres. 

Posteriorly to these is the optic chiasm from which the optic tracts run 
posteriorly and outwardly, surrounding the cerebral peduncles, and extend- 
ing to the thalamus opticus and the corpora quadrigemina. Behind the optic 
chiasm we find the floor of the third ventricle, the tuber cinereum with the 
infundibulum, at the extremity of which is the hypophysis. This latter struc- 
ture has in the last few years attracted great attention because it has fre- 
quently been found to be enlarged in acromegalia, and the pressure upon the 
chiasm has produced bitemporal hemianopsia. In how far these enlarge- 
ments of the hypophysis are the cause of acromegalia has not yet been deter- 
mined. Behind the hypophysis are the two corpora mammillaria, also a gray 
substance, the substantia perforata posterior, lying upon the floor of the 
subthalamic region between the two cerebral peduncles. At either side of this 
structure the cerebral peduncles pass posteriorly and medially to the pons. 
The oculomotor nerve appears in front close to the origin of the great trans- 
verse fibers of the pons and on each side of the internal border of the pedun- 
cles, while somewhat further on, and posteriorly between the lateral posterior 
border of the peduncles and the pons, the trochlear nerve arises. The fifth 
nerve with its narrow motor and broad sensory divisions emerges from the 
lateral posterior portion of the pons. While, up to this point, the cranial 
nerves are isolated at the base of the brain, when the pons enters the medulla 
and the cerebellum begins, the origins of the cerebral nerves are close together, 
so that, for instance, tumors which here implicate the bones of the posterior 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 25 



groove of the skull or the cerebellum, even without attaining great develop- 
ment, may cause multiple cranial nerve paralyses. The abducens emerges 
medially from the transverse furrow between the pons and the medulla, 
while the facial and acoustic (VII and VIII) nerves appear at the side 
of the medulla oblongata. The glossopharyngeal and vagus follow caudad, 



Bulbus olfactorius (I) 
Tractus olfactorius 
Chiasma opticum 
N. opticus (II) j0t<S;\ 



Polos frontalis 



Fissura longitudinalis cerebri 
Sulcus olfactorius 

Hypophysis' 



Trigonum olfactorium 
Tractus opticus 



N. oculomotorius 
(III) 

N. trochlearis 
(TV) 

N. trigeminus. _ 
(V) 

N. abducens 
(VI) 

N. facialis 
(VII) 

N. inter- 
medius 

N. acusticus— - 
(VIII) 

N. glos.so ^f 
pharyngeus (IX) 



Fissura cerebri lateralis 
(Sylvii) 

Polus temporalis 



Substantia per- 
'''forata anterior 



Infundibulum 

Tuber cine* 
reum 



N. vagus (X) 



N. accessor ius 




Fossa inter- 

peduncularis 

(Tarini) 



\ Plexus chorioideus 
ventriculi quartj 



Foramen caecum 
Hcmisphferium cerebelli 



N. hypoglossus 



N. spinalis I 



Medulla oblongata 

Decussatib pyramidum 
> 

Medulla spinalis, 
Polus occipitalis 

Fig. 17. — The Base of the Brain Showing the Origin of the Roots of the Cranial Nerves. 
The basal surface of the cerebrum in its posterior division is covered by the cerebellum. 

while the spinal accessory and hypoglossal nerves arise from the lateral parts 
of the lowest portion of the medulla oblongata, the former, indeed, from 
the uppermost part of the cervical cord. The individual parts of the cere- 
brum and the cerebellum at the base of the brain have been discussed in 
preceding sections. 



26 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

It is characteristic of the arterial blood of the brain that it is supplied 
by two channels; the anterior portion of the brain by the internal carotid 
artery, the posterior part by the vertebral artery which is a branch of the 
subclavian. The internal carotid divides at the external border of the optic 
chiasm into the anterior cerebral artery, which runs forward and medially 
and into the Sylvian or large middle cerebral artery, which passes into the 
Sylvian fossa. 

At the same point the posterior communicating and the choroid arteries 
are given off posteriorly from the internal carotid. The vertebral arteries 
on both sides unite at the point where the pons passes into the medulla and 
form the basilar artery, which, after giving off a number of branches for the 
pons and the cerebellum, divides near the anterior end of the pons into the 
two posterior cerebral arteries which pass on laterally. The anterior cerebral 
arteries are united in front by the anterior communicating artery. The pos- 
terior communicating artery on each side passes into the posterior cerebral 
artery, and produces the well known circle of Willis which unites the arterial 
regions of the carotid and vertebral arteries, being composed of ten arteries, 
as follows: 1, Anterior communicans; 2 and 3, the anterior cerebral arteries; 
4 and 5, the arteries of the Sylvian fossa (the middle cerebrals) ; 6 and 7, 
the posterior communicating arteries ; 8 and 9, the posterior cerebral arteries ; 
10, the basilar artery. It is true many variations occur here. Of the most 
important arteries of the brain, the anterior cerebral supplies a large portion 
of the frontal brain, the gyrus fornicatus, the paracentral lobe, and the pre- 
cuneus as well as the corpus callosum. The large, middle, cerebral artery 
(artery of the Sylvian fossa) supplies to a wide extent the area of the large 
ganglia and the internal capsule, and is of the greatest clinical importance 
as the usual seat of cerebral hemorrhage. It then gives off numerous col- 
lateral branches of which the first passes to the third frontal convolu- 
tion, the second supplies the central convolution and the operculum, the third 
the gyrus supramarginalis and angularis, and the fourth and fifth extend to 
the temporal convolutions, while special branches supply the island and, 
at the same time, the claustrum and the external capsule. The posterior 
cerebral artery encircles the pedunculus, and then passes posteriorly across 
the medial wall of the occipital lobe. It supplies almost all of the walls 
of the ventricle, and also the posterior divisions of the optic thalamus, the 
corpora quadrigemina, and the hippocampus major. The calcarine artery 
is the most important of those arteries which pass to the cortex and supply 
the occipital lobes, the gyrus hippocampi and the third temporal convolu- 
tion; this supplies the area of the fissura calcarina and the posterior divi- 
sion of the optic radiation of Gratiolet, an occlusion of which seriously impairs 
the sense of sight. 

Among the arteries visible upon the base of the brain we see the vertebral 
and basilar arteries and their branches, also the circle of Willis which is the 
most important; the middle cerebral artery just after its origin passes under 
the lowest part of the temporal lobe and disappears in the depth of the brain. 

Diseases of the cerebral arteries and hemorrhages, thromboses and emboli 
with resultant secondary softening of extensive areas of the brain form the 
largest proportion of all diseases of the brain. Therefore it is important for 
lis to recognize that the distribution of the individual arteries throughout 



Insula 



A. cornmunicans 
anterior 

^ A. cerebri anterior 




Operculum 



chorioidea. 



A. communi- 
cans posterior 



Rami ad 
pontem 

A. cerebelli . 
inf. anterior 

, A. auditiva 
interna 



A. cerebelli inferior — 
posterior 



^A. cerebelli 
superior 



A. basilaris 



A. spinalis posterior 

- — .Radix anterior of 
N. cervicalis I 



" ' Rami spinales of A. vertebralis 

/ 

/ 



Fig. 18. — The Distribution of the Arteries at the Base of the Brain and the Circle 

of Willis. 

The frontal lobes have been slightly separated to show the course of the two anterior cere- 
bral arteries as far as the genu of the corpus callosum; the right lateral cerebral fissure has 
been somewhat widened to show the branching of the middle cerebral artery. On removal 
of the left cerebellar hemisphere, the distribution of the posterior cerebral artery upon the 
basal surface of the occipital and temporal lobes becomes visible. 

27 



28 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

the cortex of the brain coincides in the main with the areas of the cerebral 
centers, so that, following hemorrhages or embolisms which are not too exten- 
sive, isolated symptoms of the absence of definite functions may appear. The 
most freqnent are hemorrhages from branches of the middle cerebral artery 
into the internal capsule and the basal structure surrounding it, and this 
produces paralyses, motor irritative symptoms, anesthesia and hemianopsia, 
also hemorrhages which by rupture into the ventricles are often the cause of 
death. 

We must now devote some attention to the spinal cord which develops 
gradually without a line of demarcation from the medulla, this structure 
tapering in its downward course, the pyramidal decussation being regarded 
as the beginning of the cord, therefore that area where the fibers of the pyra- 
mids running dorsally cross each other, and, with the exception of the small 
pyramidal anterior column tracts, extend to the lateral columns. As the 
nuclei of the posterior columns disappear, the characteristic formation of the 
spinal cord is seen, a white medullary mantle surrounding the substance which 
in shape resembles a butterfly and only at the entrance of the posterior roots 
reaches the periphery. The human spinal cord from the first ceryical vertebra 
to the first lumbar vertebra or, at most, to the upper border of the second, 
attains a length of from 40 to 50 cm. ; the lowest portion of the spinal cord in 
man, as well as in anthropomorphic apes, is quite undeveloped but is analogous 
to the tail in animals. The spinal cord in two areas is markedly enlarged 
showing a cervical enlargement at the fifth or sixth cervical vertebra and a 
lumbar enlargement in the region of the lowest thoracic vertebrae, both corre- 
sponding to the spinal centers for the muscles of the extremities. We differ- 
entiate the cervical cord with eight cervical roots, the thoracic cord with twelve 
dorsal roots, the lumbar cord with five lumbar roots, the sacral cord with five 
sacral roots and, finally, a pair of coccygeal nerves. The sacral cord terminates 
in the lowest portion of the spinal cord, the conns medullaris, which then 
separates into thin, terminal threads about 25 cm. long, the filum terminale. 
The sulcus longitudinalis ventralis medianus is situated ventrally in the 
middle, and as the anterior sulcus penetrates into the medullary mass. Cor- 
responding to it is the sulcus longitudinalis medianis dorsalis which is merely 
a narrow fissure. Alongside of this, near the point where the posterior roots 
enter, we note the sulcus lateralis dorsalis and a very indistinct sulcus lateralis 
ventralis at the point of exit of the anterior roots. In consequence of the 
shortening of the spinal cord which takes place in man the spinal roots must 
run toward the cauda in order to reach the corresponding intervertebral open- 
ings, and the deeper their origin the longer their course. Hence, in the region 
of the conus medullaris and below it there is a rich sheaf -like collection of 
nerve roots running downward, the so-called cauda equina. The furrows of 
the spinal cord permit us to differentiate an anterior and a lateral column, 
which, however, are only very indistinctly separated from one another, also 
a posterior column. The latter is divided in the cervical cord by a furrow, 
the sulcus paramedianus dorsalis, into Groll's or the medial column, and 
Burdach's or the lateral column. The gray substance is divided into the 
anterior horn which contains the larger portion of the motor ganglion cells, 
and the much smaller posterior horn the apex of which passes close to the 
periphery, and into the lateral horn which is not everywhere equally developed. 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 29 



A narrow, central canal extends down the middle of the spinal cord, but in 
adult man this is often obliterated: the anterior and posterior halves of the 
spinal cord are united b} r commissures, the anterior white commissure and the 



Conus medullaris 



Ventriculus terminalis 



Filum terminate 




Radices n. coccygei c: j^ ' (|jj| 



Dura mater sp 



-Radix posterior n. lumbalis HI 



•/----•Cauda equina 



— Radix posterior n. sacralis I 



Ganglion spinale n. sacralis I 



^ Ganglion spinale n. coccygei 



Fig. 19. — The Lumbar Cord, Pars Lumbalis Medull.e Spinalis, with the Conus Medul- 
larts, the fllum terminale, axd the cauda equina, seen from behind. 

posterior gray commissure, which encircle the central canal. The proportion 
of white to gray substance differs in almost every transverse section; but the 
latter appears in greatest bulk in the lowest portions of the spinal cord. 

For the finer structure of the spinal cord and its microscopic relations, the 
reader is referred to the article upon " Histology of the Nervous System " in 



30 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 



this volume, and here we will consider only the vascular supply of the spinal 

cord. This is formed partly by branches of the vertebral, partly by branches of 
the intercostal, the lumbar, and sacral arteries. At each side a vertebrospinal 
branch passes down from the ventral artery; these unite in the upper cervical 
cord and form the anterior spinal artery, which sometimes alone, sometimes 
as a tract composed of anastomosing branches of the intercostal, lumbar and 
sacral arteries, extends down to the conus. Thence the arteria* sulci pass 



Sulcus medianus posterior 



Sulcus intermedius posterior. 



Radix posterior 



Septum posterius 

/ -Septum intermedium 

/ / 

jCommissura posterior 



Sulcus lateralis posterior 



Substantia 
gelatijnosa 
(Rolandi) 



Pia mater 
•spinalis 



External 
glia sheath 




Sulcus lateralis anterior- 



Radix anterior 



•■ Vi 

Fissura mediana anterior 



Column a anterior 
' Substantia grisea centralis 

Commissura anterior grisea 
\ 
\ 

Commissura anterior alba* 



Fig. 20. — Transverse Section through the Cervical Enlargement, Intumescentia Cer- 
vicalis, of the spinal cord at the point of exit of the roots of the slxth cer- 
VICAL Nerve. 

through the anterior sulcus into the spinal cord, especially into the gray 
substance, where they divide as terminal arteries. The dorsal vertebrospinal 
artery (the posterior spinal artery), which originates from the vertebral and 
anastomoses with branches of the intercostals, etc., passes along the dorsal 
side of the spinal cord at each side of the posterior roots. A number of smaller 
arteries which radiate into the white substance of the spinal cord, and are also 
terminal arteries, are designated vasocorona. Their occlusion produces wedge- 
shaped infarcts in the white substance, while occlusion of the arterise sulci 
leads to almost complete exclusion of the gray substance. 

The shortening of the human spinal cord, and the pushing of its individual 
segments upward to correspond with the, intervertebral openings, is practically 
important for two reasons. In the first place, the entire canal below the 
second dorsal vertebra contains no spinal cord, hence without fear of injury 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 31 

to the latter we can introduce a needle into the vertebral canal through the 
wide intervertebral openings of the lower lumbar vertebras and withdraw 
cerebrospinal fluid for diagnostic and therapeutic purposes. Spinal puncture, 
introduced by Quincke, has already been much practised, and it has lately 
become possible by this means to inject drugs into the arachnoid sac. Here 
analgesia produced by injections of cocain soon conquered an extensive field. 
But in other ways also important advances have been made ; above all, by the 
successful injection of tetanus antitoxin as advised by v. Leyden. 1 Further- 
more, exact knowledge of the relations of the individual segments of the spinal 
cord to the vertebra* has become of vital importance in the surgery of the 
spinal cord, which has lately developed to an extraordinary extent with an 
increasing number of successes. Diagram- have been made, especially by Reid, 
which minutely portray these relations and their variations. 

Here we reach a point in cerebral localization, namely, the relation of the 
individual portions of the brain, especially of the cortex, to the bony skull, 

Punct. Rol. sup. 



Fiss. parieto occ. < 




Fig. 21. — Topographic Relations Between the Surface of the Brain and the Skull. 

(After Thane.) 



which, with the increasing importance of cerebral surgery, is rapidly becoming 
of greater practical significance. The frontal lobe is situated under the frontal 
bone and extends posteriorly somewhat below the parietal bone; under the 
latter are the central convolutions, the parietal lobes, and a portion of the 

i See article " Tetanus " in volume on " Infectious Diseases." 



32 MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 

occipital lobes, while the temporal bone covers the temporal lobe, and the high- 
est point of the squamous suture reaches the Sylvian fissure. Under the plate 
of the occipital bone lie the occipital lobes and the cerebellum. A number of 
methods have been proposed by which to determine the fissures which should 
be traced in operations, especially the Sylvian fissure, the central furrow, etc. 
Waldeyer, in a very instructive article, " The Topography of the Brain," * 
recently described the most important relations of these, combining the chief 
features in the systems of Thane, Poirier and others. Here I shall cite only 
the main points enumerated b}^ Waldeyer. 

(a) During life, the lower end of the central furrow may be traced exter- 
nally on the skull by drawing a German horizontal (a line from the deepest 
point of the infraorbital border through the highest point of the upper border 
of the porus acusticus externus) at a right angle from the tragus close behind 
the jaw and upon this a line extending 5 to 6 cm. upward ; 

(b) The upper end of the central sulcus is found by tracing a perpendicular 
line upon the posterior circumference of the base at the point where the mastoid 
process intersects the edge of the hemisphere; 

(c) The Sylvian point, the point of division of the Sylvian fossa, is situ- 
ated at a point 4 to 4.5 cm. perpendicularly above the middle of the zygo- 
matic arch. 

(d) The upper end of the Sylvian fissure corresponds to the middle of the 
lower border of the parietal tuberosity; 

(e) The parieto-occipital fissure is found on the lambda where the lamb- 
doid and sagittal sutures unite. 

Besides the direct topographic relations of the brain and skull an inter- 
esting question is, in how far the development of definite portions of the brain 
is manifested by the shape of the bony skull, and in how far an examination 
of the latter permits us to draw conclusions as to the development of the brain 
of any particular person. 

It is known that, at the beginning of the nineteenth century, the discussion 
of this point led the great investigator, Gall, to entirely erroneous conclusions ; 
he divided the skull into various regions, and the greater or less development 
of these areas was supposed to indicate greater or less mental development 
along certain lines. Even during the lifetime of Gall there was great opposi- 
tion to this teaching, which actually impaired the value of Gall's otherwise 
great services, for subsequent exact investigation of the brain and its faculties 
proved these theories to be absolutely incorrect. Lately Mobius has reverted 
to Gall's teaching inasmuch as he regards an unusually marked development 
of the upper external angle of the orbit (which, according to Gall, is the seat 
for the sense of figures) as a sign of especial mathematical power, as a " mathe- 
matic organ," due to an abnormal and increased development of the anterior 
end of the third frontal convolution. This view which Mobius proposed has 
not as yet been generally accepted nor has it been confirmed by others. On 
the other hand Schwalbe, in the Festschrift, issued upon the occasion of Kuss- 
maul's 80th birthday, described the " relations of the internal and external 
conformation of the skull " and has shown that a part of the rilievo of the 
brain is recognizable upon the external surface of the skull, best in those 

i Deutsche med. Wochenschr., 1901, Nr. 26 et seq. 



MACROSCOPIC ANATOMY OF THE CENTRAL NERVOUS SYSTEM 33 

parts covered by muscles, but roost clearly in the temporal region where, occa- 
sionally, four convolutions on the surface of the brain, the lower or third 
frontal, and the three lateral temporal convolutions may be detected as promi- 
nences. But Schwalbe himself expressly warns us not to take this as a basis 
for new phrenologic calculations, especially as the development of the promi- 
nence of the third frontal convolution is by no means an indication of mental 
power; on the contrary, particularly in the skulls of the lower races, this is 
usually well developed. Here a lesson for the future appears — we must seek 
to acquire more accurate knowledge of the relations of the brain to the skull. 
We approach the end of our discussion. It is most unsatisfactory to 
attempt to describe the macroscopic anatomy of the central nervous system 
within the compass of a short article. If I have succeeded in giving a general 
picture of the relations here existing, and have interested the practitioner by 
considering the physiologic importance of this somewhat dry subject, I have 
fulfilled my object. The structure of the brain can. of course, only become 
more interesting as our knowledge of these individual tracts and their func- 
tional activity increases. All the relations which have been disclosed by 
experiment and microscopic study have been thoroughly discussed in this 
article 



NORMAL AND PATHOLOGICAL HISTOLOGY OF THE 
. CENTRAL NERVOUS SYSTEM 

WITH SPECIAL REFERENCE TO THE NEURON THEORY 
By H. ROSIN, Berlin 

It is well known that the conspicuously rapid advance of the last few 
decades in the science of neurology is attributed to a sharper limitation and 
to an increase of clinical pictures, to a more comprehensive study of their 
symptom-complex, and, above all, to the important findings which histology 
has added, particularly in the study of the structure of the nervous apparatus. 
Although our knowledge is to-day still incomplete, nevertheless we have ad- 
vanced so far beyond the mere composition of the central nervous system that 
we are enabled to study a number of systems of nerve tracts coursing from 
the center to the periphery, and to determine the origin and source of stimu- 
lation of certain sensory and motor impulses. In pathology, histology has also 
led us to conclusions regarding anatomical changes which disclose to us the 
seat of many diseases of the nerves. 

By the comparison of the histologic rinding with the clinical symptom- 
complex neurology has given us important data; and it may, therefore, be 
interesting to review the normal and pathological histology of the nervous 
system from the present standpoint of scientific investigation, after which 
many symptoms, many a pathologic picture, will become intelligible. 

In the following I shall attempt such a portrayal without, on account of 
the limited scope of this article, entering further into details; nevertheless, a 
prerequisite is a knowledge of macroscopic relations, those relations in the 
structure of the spinal cord and, above all, in the brain, which should be 
familiar to all physicians from their studies of anatomy and embryology. In 
regard to these structures views have not materially changed in recent years. 
The old nomenclature is employed for the cerebral convolutions, for the central 
ganglia in the interior of the brain, for the cavities and fossae, for the varying 
arrangement of the gray and white substance, and these are sufficiently ex- 
plained in the various text-books upon anatomy and embryology. To any one 
especially interested in these subjects, I would commend the text-books of 
Edinger and Obersteiner, which are accurate and excellent treatises. Those 
who desire to investigate closely the theme under discussion may well study 
v. Bechterew's " Conduction Tracts in the Brain and Spinal Cord." 
34 



THE HISTOLOGIC COMPONENTS OF THE NERVOUS SYSTEM 



35 



I. PRELIMINARY REMARKS: THE HISTOLOGIC COMPONENTS OF 
THE NERVOUS SYSTEM 

The nervous organs are composed of the true nervous and of the gray or 
vesicular substance (including the vessels). 

The nervous tissue consists of nerve-cells and cell processes. 

The vesicular or cineritious substance — apart from the true connective 
tissue in the gross coverings, the adventitia, and the actual walls of the vessels 
— is composed of a peculiar connective substance which, since the time of 
Virchow, has been designated by the term neuroglia. 

A. THE NERVE TISSUE 



(1) THE XERVE-CELLS 

I shall first state what we at present know of the structure of the nervous 
substance, and I shall begin with the nerve-cells. Recently the finer structures 
of these tissues have been revealed to us as of such exceeding richness and pecu- 
liarity that, so far as 
we know, they may 
justly be included 
among the most com- 
plicated cells of the 
organism. 

Even the nucleus of 
the nerve-cell (Fig. 22, 
a) is characteristic on 
comparison with the 
nuclei of other cells; 
first, by its size, par- 
ticularly in those large 
cells which are found 
in the motor cortex of 
the brain, in the nuclei 
of the medulla oblon- 
gata, and in the ante- 
rior horns of the spinal 
cord. Here the germi- 
nal vesicle has the ap- 
pearance of an ovum. 
But the nucleus of even 
the smallest nerve-cells 
is always relatively 
large. 

The structure and 
the tinctorial property 

of the nucleus is also typical. In the large cells it is separated from the 
body of the cell by a sharp contour. On a light background, comparatively 




Fig. 22. — Nerve-cell from the Anterior 
Spinal Cord of a Rabbit, Hardened in 
cohol. Neutral red staining. 



Horn of the 
formalin-al- 



36 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

few but dark, more markedly refractive staining bodies are visible, among 
which one, the nucleus (Fig. 22), is conspicuous among the large nerve- 
cells and resembles the germinal spot of the germinal vesicles. The reac- 
tion of the nucleus to certain stains is noteworthy. While cell nuclei have 
in common the property of taking up basic stains with their chromatin 
substance (and, therefore, are called nuclear stains), and while the nuclear 
corpuscle (if present) frequently selects a stain which has an acid prop- 
erty, the reverse of this takes place in the nucleus of the nerve-cell : Its 
chromatin, scant in amount, shows an affinity for acid stains even when 
used in neutral staining mixtures ; for instance, in staining with triacid, with 
methylene-blue, eosin, etc.; on the other hand, the nuclear corpuscle (at least 
in man) stains uniformly and readily with both colors, and reveals to neutral 
color mixtures a neutrophilic property ; in some animals this is even basophilic. 

The body of the nerve-cell is, however, much more interesting than the 
nucleus. Recently attention has been specially called to the so-called Nissl's 
bodies or tigroid substance (Fig. 22, b), the importance of which must be 
pointed out. 

Formerly, when the only agent for preserving and hardening the nervous 
tissue was Midler's fluid, i. e., a mixture of potassium chromate, chromic acid 
and sulphuric acid, it was impossible to get a clear idea of the structure of the 
body of the cell even by employing several stains; Muller's fluid makes only 
the nucleus structurally distinct, rendering the substance of the body of the 
cell homogeneous. By using alcohol for hardening (Mssl), and recently for- 
malin which was introduced into histology by Blum, the individual parts of 
the body of the nerve cell have been revealed. In addition to the old methods 
of hardening, other stains are necessary for the detection of Nissl's bodies. 
In place of carmin, which Gerlach considered most important for staining in 
general, especially for nerve staining, Nissl used the more powerful basic 
anilin colors. If material that has been hardened, embedded, and cut in 
alcohol is stained with methylene-blue soap solution (or neutral red solution) 
coarse granules appear in the body of the cell and cover a considerable portion 
of it, extending almost to the periphery; they cluster around the nucleus in 
groups which penetrate to some extent^ into the processes of the cell ( Fig. 
22, d), and, under powerful magnification, are seen to be composed of indi- 
vidual threads and granules which have absorbed the basic stain. Mssl's 
method, therefore, embraces two different processes, the hardening in alcohol 
and the utilization of the basophilia (Rosin) of the granules in the choice of 
suitable staining substances. 

This appearance of basophilic substances in the body of the cell is quite pe- 
culiar, and is not found in other cells of the human body (except in mast-cells 
and, to a certain extent, in the plasma cells and lymphocytes) ; it is evident, 
therefore, that the histologic characteristics of the nerve-cells just described 
are of peculiar nature, particularly shown in their standing and analysis. 

Between Nissl's bodies in the substance of the nerve-cell a basic substance 
(Fig. 22) remains which, on staining according to Mssl's method, does not 
show the color. On staining with different mixtures (the triacid, according 
to Rosin, the methylene-blue erythrosin, according to Held), in contrast to 
the granules, it shows a preference for acid colors, upon employing the red in 
acids and the yellow neutral red in alkalies it becomes yellow, but it shows 



THE HISTOLOGIC COMPONENTS OF THE NERVOUS SYSTEM 



37 



no affinity for basic stains (unprepared). In this " oxyphilic " basic substance 
yellow granules are deposited which stain intensely with fatty staining sub- 
stances, such as osmium, sudan and scarlet, and which are to be included 
among the lipocliromes, i.e., stained fatty substances (Rosin), and" in adults 
these deposited in dense clumps often form a large portion of the basic sub- 
stance (Fig. 23, A and B). 

The remaining portion of the basic substance was formerly supposed to be 
structureless, but Apathy and Bethe, by a peculiar process of maceration and 
subsequent staining, have enabled us to make a differentiation which may possi- 
bly be of great importance in the study of the structure of the nervous system 
and the course of nervous stimulation ; this will be considered later. These au- 
thors noted that the ground substance is composed of a fine fibrillary net which 
passes through the body of the cell between Mssl's granules. The fibrillar pass 
from one process to another through the protoplasm, thence into the fibrillae 
(see below) which compose the axis cylinders as well as the dendrites. 

The nerve-cell is composed of a small, non-granular peripheral zone (Fig. 
22) and a delicate structureless covering; the point where the nerve process 
originates, as well at its beginning, appears to be free from granules (Fig. 22, c) 
which is in contrast with the dendrites (Fig. 22, d) ; in these statements I 
have mentioned the most important and the most complicated and peculiar 
features of the nerve-cell. 

(2) THE CELL PROCESSES 

Since the advenl of microscopy of ili<' nervous system, the processes have 
quite properly been regarded as the important and characteristic constituents 
of the nerve-cell. The familiar designations, unipolar, bipolar and multipolar 
nerve-cells, are now almost discarded; formerly every point of exit of a cell 




Fig. 23. — A, Sudan staining of the anterior horn of the spinal cord (man) ; B, Sudan staining 

of the cerebral cortex (man). 



process was designated as a pole of the nerve-cell. But the teaching of cell 
processes has recently undergone a change, and is now based upon a more 
comprehensive foundation, which, in its completeness, at present dominates 



38 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

the theory of the histologic structure of the central nervous organs, namely, 
Waldeyer's neuron theory, which will suhsequently be explained. I shall now 
attempt to present briefly the most important of these views regarding the 
behavior of the cell processes. 

The importance of suitable methods of staining, which has always been 
appreciated, is particularly evident in the histology of the nervous system. 

It was a matter of paramount importance to find stains which would 
clearly reveal the distribution of the cell processes. 

One of these stains is used in the so-called Golgi method, with which 
and with its modifications this author and other pioneers have made brilliant 
discoveries. Among them we must mention Ramon y Cajal, further van 
Gehuchten, Kolliker, v. Lenhossek, and Bechterew. The Golgi process is 
based upon a sometimes rapid, at other times slow, impregnation, of the nervous 
organs (hardened in chromium) with a silver, gold or mercury salt, and the 
subsequent reduction of the salt to the metal that was contained in the salt. 
This metal is deposited externally upon the cells and their processes, where, 
as a black substance, it gives the structure prominence. A fault in the method, 
namely, that only certain isolated cells are stained, others remaining uncol- 
ored, proves an advantage in the investigation, since, in suitable preparations, 
instead of a net that cannot be disentangled, a few cells only with their entire 
processes become distinctly visible. 

A second method enables us to stain cells and their processes through- 
out their entire extent. This is designated as vital methylene-blue staining. 
It was introduced by Ehrlich. The methylene-blue solution is either injected 
into the living animal until the tissue of the nervous system is saturated with 
the color (usually resulting in a white reduction product which again turns 
blue on exposure to the air) , or into " tissue kept alive outside of the body," 
i. e., the tissue of freshly killed animals, until a definite intensity of staining 
is attained. As the color is prone to disappear rapidly, it is retained by fixa- 
tion; for example, with ammonium picrate or ammonium molybdate. Ret- 
zius, Dogiel and S. Meyer have successfully used this Ehrlich stain which 
requires considerable practice. 

By these methods the relation of the nerve-cell to its processes has been 
revealed as follows: Every nerve-cell of the central nervous system has a 
process which, as a rule, is subsequently medullated (always when it leaves 
the gray substance, but in the sympathetic ganglia the majority of the nerve 
processes are non-medullated), the so-called nerve process (formerly axis- 
cylinder process) (Fig. 22, c; Fig. 24, b; Fig. 25, *). The other processes, 
the number of which varies greatly in the different cells, are called dendrites 
(formerly protoplasm processes). They are usually thicker than the nerve 
processes, are never medullated, and are never as long as the nerve processes 
(Fig. 22, d; Fig. 23, a). 

While the nerve process usually extends forward horizontally from the 
cell (Fig. 25, *, 26, a, and 27, a) and gives off many lateral branches (col- 
laterals) which pursue a varying convoluted course (Fig. 26, b), the dendrites 
usually soon bifurcate, occasionally into extraordinarily dense tree-like 
branches (Fig. 24, a, 25, 26, c, and 27, c) (for example, the pyramidal cells 
of the cerebral cortex, Purkinje's cells of the cerebellar cortex). This branch- 
ing of the dendrites finally ceases, and they either have free terminations, 



THE HISTOLOGIC COMPONENTS OF THE NERVOUS SYSTEM 



39 



being contiguous to similar terminal branches of other dendrites, or they are 
wrapped basket-like around other nerve-cells and their processes. The nerve 
process itself has a varying length; sometimes it is quite short, and after 
giving off many or few collaterals it divides near its origin into terminal 
twigs similar to the dendrites. Thus it is brought into contact with other 
cells and terminal twigs, and the same occurs with its collaterals. Often, 
however, the nerve process is of great length, it traverses wide areas of the 

brain or spinal cord, constantly giving off col- 
laterals, often extends from the brain into the 
spinal cord, or leaves the central nervous sys- 
tem and passes by peripheral nerves to those 
organs which it supplies. Such nerve proc- 
esses, for example, the fibers of the cerebral 





Fig. 24. — Motor Anterior Horn 
Cell with its Processes. (Af- 
ter Toldt.) 



Fig. 25. — Ganglion Cell of the Anterior Horn, Golgi 
Staining. (After Lenhossek.) 



nerves or those of the anterior and posterior roots, often have a length of 
many centimeters. Those nerve processes which extend to the periphery, for 
example, the motor nerves, terminate in the muscle (Fig. 19) by splitting 
into minute primitive fibrillar The sensory fibers have a similar termination. 
The dichotomic division appears to be characteristic for the termination of 
all nerve processes. 

The course of the nerve process, therefore, may he almost microscopically 
short, or of extraordinary length considering its minute size at the point of 
origin. 



40 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



Those who remember former views regarding the branching of processes 
of the nerve-cells must recognize the significance of the decided change in 
view. The nerve process histologically differs from the dendrite. Often it 
alone is destined for a long course, it alone is medullated, it never unites 
with another cell, as was once believed, but invariably terminates by splitting 
into fibrillar The old scientific principle of the indivisibility of nerve fibers 
has been forever abrogated. Even the so-called unipolar nerve-cells of the 



d 




spinal ganglia (Fig. 20, a) 
show a division of the nerve 
process: Shortly after its 
exit from the cell it divides 
T-shaped into two long 
branches, one of which be- 
comes the posterior root 
fiber (Fig. 20, b), the other 
the peripheral sensory nerve 
fiber (Fig. 20, /) (see be- 
low). 

In the main there has 
been no addition to our 
knowledge of the structure 



S) 



/ 



/ 



\. 







Fig. 26. — Sensory Nerve-cell. 
(After Golgi.) 



Fig. 27. — Nerve-cell of the Sym- 
pathetic. 



of the peripheral nerve fibers, but the sympathetic system has not yet been 
thoroughly studied in man. Here comparatively little has been added to the 
knowledge acquired in the earlier microscopic period. What Ranvier, Remak 
and others believed is to-day still accepted. 

We must now briefly discuss the histology of the peripheral nerve fibers. 
In the interior is the axis cylinder (Fig. 30, c), an albumin substance, the 
fibrillary structure of which was once doubted but is now acknowledged. The 
axis cylinder (axon), which is not visible in the fresh structure, has an 
especial affinity for acid stains (oxyphilia). It is surrounded by the myelin 
sheath (Fig. 30, b) which, traversed by the delicate neurokeratin structure, 






- d 



f 




Fig. 28. — Diagram of a Motor Xeurox; Fig. 29. — Diagram of a Sensory Neuron 
First Division. (After Toldt.) First Division. (After Toldt.) 

41 



42 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 




a 

Fig. 30.- 

Nerve 

RATED 

Fluid. 



- Medullated 
Fiber, Mace- 
in Muller's 



consists of semi-fluid myelin, and is probably identical 
with or closely related (Wlassak) to Liebreich's pro- 
tagon. The medullary sheath is surrounded by 
Schwann's sheath (Fig. 30, d), a structureless cover- 
ing which contains oblong nuclei. 

The medullary sheath shows here and there deep 
annular constrictions, Ranvier's nodes (Fig. 30, a), 
which almost completely divide it. Schwann's sheath 
covers the axis cylinder which is somewhat decreased 
in size. The nucleus is situated between these two 
constrictions in Schwann's sheath, and the medullary 
sheath at brief intervals shows clefts which are known 
as Lantermann's segments ; these pass obliquely almost 
through the myelin, thus forming furrows one above 
another. The limits of this chapter upon the cen- 
tral nervous system do not alloAV us to discuss the 
peripheral termination of the nerves in the mus- 
cles, skin, internal organs, and the organs of special 
senses. 



B. THE SUPPORTING SUBSTANCE. (THE GRAY OR VESICULAR 

SUBSTANCE.) 

The soft, nervous pulp is held together by a peculiar, widely branching 
basic tissue. Only the coarsest fibers originating from the pia mater (Fig. 



b — 




\ 

Fig. 31. — Transverse Section through the Cervical Enlargement. (After Toidt.) 



THE HISTOLOGIC COMPONENTS OF THE NERVOUS SYSTEM 



43 



31, b, and 32, b) and from the vascular sheaths of the arachnoid (Fig. 

32, c) which form, septa between the convolutions and folds (Fig. 31, a, 
Fig. 32, b, and Fig. 32, g) are formed of connective tissue; these are carried 
from the larger vascular trunks a certain distance within the gray substance. 
The vessels themselves 
with their walls, and 
even the capillaries, form 
a trabecular structure 
which is not without im- 
portance. 

The chief support is 
given by a specific sub- 
stance, the glia tissue. 
This has a characteristic 
structure and a peculiar 
tinctorial property. Wei- 
gert, and subsequently 
other authors (especially 
Benda), succeeded in iso- 
lating the glia tissue 
from other tissues, espe- 
cially the connective, and 
staining 




Fig. 32. — Transverse Section through the Upper Lumbar 
Cord. (After Toldt.) 



it. Like the 

nerve substance originating from the ectoderm (according to His, neuroblasts 
and spongioblasts), the neuroglia presents embryologicallj a compact net of 
trabecular which vary greatly in their caliber as well as in their density and 
arrangement. A thickly woven network of trabecular surrounds the brain 
and spinal cord, another covers the walls of the ventricles and the central 

canal of the spinal cord. 
It forms a network (Fig. 
33) in the gray substance 
that surrounds the nerve- 
cells and their processes. 
The glia of the white sub- 
stance is less dense; it 
passes between the nerve 
tubules in a loose fold, en- 
closes isolated bundles 
(Fig. 34) of these, and to 
some extent penetrates into 
the anterior and posterior 
nerve roots. 

Many of the nuclei 
which are sometimes sur- 
rounded by a layer of pro- 
toplasm are deposited in 
the midst of the glia net, preferably in the white substance, where several glia 
trabecular cross each other. It is still a mooted question whether these fibers 
unite with the glia cells (glia nuclei, Deiter's cells, spindle cells) (Fig. 36), 




Fig. 33. — Transverse Section through the Normal 
Gray Substance of the Anterior Horn op the Spi- 
nal Cord. 



44 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



or only pass along side them. But the adhesion of glia fihers to connective 
tissue septa and vascular sheaths has heen proven. 




Fig. 34. — Transverse Section through the Normal 
White Substance op the Spinal Cord. 



II. THE GROUPING OF THE NERVOUS SUBSTANCE AND 
THE NEURON THEORY 

From the preceding description of its elements, it is apparent that the 
tissue of the nervous system is of very complicated composition, of which 

we have now obtained a 
somewhat clear idea. 

The majority of the 
nerve-cells are found only 
in the gray substance, rare- 
ly in those areas where the 
gray and white matter are 
intimately admixed (in the 
medulla oblongata, in the 
pons, in the mid-brain and 
inter-brain). The white 
substance itself contains 
none of them. In many 
areas of the gray substance 
the nerve-cells are arranged 
in groups. 

In the spinal cord, es- 
pecially in the dorsal por- 
tion, segmentation may be recognized from above downward, a metameric 
arrangement. In a horizontal 
section there is seen a three- 
fold grouping of the anterior 
horn cells, especially in the 
cervical, cord, to which is added 
the cell group of Clarke's col- 
umn in the thoracic cord. 
There are also numerous nerve- 
cells which are apparently 
distributed irregularly through- 
out the gray substance of the 
spinal cord. 

The entire cortex of the 
brain shows the nerve-cells 
arranged in layers, which dif- 
fer from each other in shape 
and size. The nerve-cells ac- 
cumulate near the origin of 
the nerves of the brain, also FlG 35 
in its central ganglia and in 
the gray nuclei of its trunk. The majority of these send nerve processes 
from the gray substance into the white; a few nerve-cells, usually the smaller 




-Glia Cells (Spindle Cells). (After Kolliker.) 



THE GROUPING OF THE NERVOUS SUBSTANCES 



45 



whose nerve processes are very limited in extent, remain wholly within 
substance. 
As the nerve-cells are partially 



ones 
the gra\ 



grouped 



and partly isolated, 



so also the 

nerve fibers of the white substance either unite with well known nerve tracts 
and form columns, or pursue their own paths which are still unknown. 

The columnar nerve tracts within the central nervous system pass from 
one structure to another by longer or shorter paths. At certain points upon 
the same side of the brain and spinal cord they unite with one another {asso- 
ciation system), or at corresponding points on different sides {coordination 
system), or they pass caudalward from the cortex of the brain to the central 
ganglia, or even lower down into the spinal cord {projection systems). Some 
of the latter unite directly or indirectly with those coining from the brain 
and spinal cord and with the nerves extending into these structures. These 
are the centrifugal 
and centripetal fibers, 
among them the mo- 
tor and sensory, the 
paths of which are 
best known, while we 
know little of the 
course of numerous 
other tracts, particu- 
larly of those which 
lead to the internal 
organs, nor of the vaso- 
motors, many of which 
pass to the sympa- 
thetic, and reflex 
tracts. 

If, in the descrip- 
tion of the normal 
course as well as of the 
pathological changes of 
the nervous tracts, 
some areas are more or 
less obscure, I shall en- 
deavor in the follow- 
ing to describe briefly 
all that has thus far 
been discovered in neurology, all that has 




of 



general interest. 



Fig. 36. — Diagram of the Structure, the Course, and the 
Branching of Motor Neurons of the First and Second 
Divisions. (After Obersteiner.) 

become common knowledge and is 
My description will necessarily be incomplete. 

First, however, I must state the fundamental principle regarding the 
structure of the nervous system which is generally accepted to-day and is des- 
ignated as the neuron theory. Previously, as a hypothesis rather than based 
upon histologic investigation, the conception of- the relation of individual 
nerve tracts to each other differed little from our present view. 

By the term neuron we understand the totality of>a nerve-cell and all of 
its processes (Fig. 36, I). All nervous processes, all dendrites including their 
branches and collaterals, are merely parts of the body of the cell, the totality 



46 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

(Fig. 36, I, II, III, Fig. 28, and Fig. 29) therefore forming a histologic and 
functional unit. The nerve is so intimately connected with the cell that it 
perishes if detached from it or if the cell dies. Inversely, the nerve-cell sus- 
tains injury if robbed of important parts of its processes, and signs of degen- 
eration subsequently appear, with permanent atrophy unless there is regenera- 
tion of the fiber. 

The individual neurons of which the entire nervous system is composed 
are in such close contact that, as a rule, the terminal twigs of one neuron 
closely approach the cell body of the other or surround it (Fig. 36, 2 and 3). 
It is possible that the terminal branches of the two neurons approach so closely 
as to be contiguous (Fig. 36, J/.). 

The long tracts of the nervous system are always composed of several neu- 
rons. Thus we differentiate between neurons of the first, second, and third 
division, etc., and the peripheral neuron is invariably designated as the -first. 

In the motor tract we usually differentiate only two neurons (Fig. 36, 
2 and 3), in the sensory at least three (Fig. 36, 4). Other associated and 
coordinated tracts within the central nervous system may be composed of still 
other groups of neurons, but of this we have no accurate knowledge. 



III. THE FIBRILLiE THEORY. (NEURO-FIBRILS.) 

That the neuron theory is based upon exact data histologically obtained 
by the Golgi method has already been stated. That, so far as we are aware, 
it sufficiently explains the structure of the nervous system, its " fiber sys- 
tems," the course and the communication of its tracts, and, above all, certain 
of its histologic changes in disease, will perhaps become evident in our later 
description. Notwithstanding all this, I must here at least mention the most 
recent theory of the histologic composition of the nervous substance, since 
it is based also upon microscopic findings which its founders (Apathy and 
Bethe) repeatedly demonstrated. By a peculiar process of hardening, macer- 
ation, and staining, these authors succeeded in separating the basic substance 
of the nerve-cells as well as their processes into fibrillar. They made prepara- 
tions which showed that the fibrils pass uninterruptedly from the nerve fibers 
through the body of the cell into the dendrites, that they merely pass through 
the body of the cell, not stopping there, and that subsequently, by the com- 
munication of their processes, they run through other nerve-cells without 
terminating there. These histologic findings, if applied to the guiding 
nervous substance, must vitiate the law of the discontinuity of neurons and 
nerve fibers, that is, the fibrillar, and must be assigned a substantive position 
which, according to the neuron theory, is in sharp contrast with their non- 
uniformity. In consequence, the terminal ramifications are said not to ter- 
minate free in front of the cell but, as was maintained some years ago by 
Held, to enter into the body of the cell or into the interior of the processes 
which it surrounds, there to unite with the fibrillar. Hence, nerve stimulation 
must necessarily pass through an uninterrupted fibrillary substance, and the 
nerve-cell is relegated to a very subordinate position, much more so than that 
it held prior to the development of the neuron theory. I think it necessary 
to mention this fibrillge theory, but we must await further investigations 



NEURON SYSTEMS AND NEURON DISEASES 



47 



before substituting it for the neuron theory, the conceptions of which, obtained 
by means of very subtle researches, are entitled to the closest consideration. 
The majority of neurologists are at present decidedly inclined to adhere 
to the neuron theory, although in a somewhat modified form. 



IV. NEURON SYSTEMS AND NEURON DISEASES 

Our knowledge of the structure of the nervous system has not yet reached 
a point which enables us systematically to divide the countless, intertwining 
nerve fibers, to assign to each of these its definite path, and to every tract 
its cellular origin and its termination. This has been possible to only a com- 




Fig. 37. — Diagram of the Conduction Tracts of the White Substance of the Spinal 

Cord 

paratively slight extent. Here we follow the neurons through their entire 
course, and here we are enabled to recognize certain pathologic changes prin- 
cipally confined to the individual neurons affected, and which are therefore 
appropriately called the neuron diseases. 

We shall first consider the normal relations of the neurons. 

At present but two of the large and important neuron systems have been 
comprehensively studied: The centrifugal motor and the centripetal sensory 
tracts including the paths of the organs of special sense. Embryologic inves- 
tigation, the animal experiment (severing and degeneration), and, finally, 
microscopic investigation of the pathologic changes by approved methods 
among which those of Weigert- and Marchi are in the front rank, have given 
us the results at present attained, and by these means we may hope to achieve 
additional successes. 



48 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



A. THE MOTOR TRACT 

(1) THE FIRST MOTOR NEUEOX 

(a) The First Motor Neuron in the Spinal Cord 

The anterior roots of the peripheral nerves spring from the spinal cord at 
either side of the anterior longitudinal space (Fig. 31, c, Fig. 32, a, Fig. 37, a, 
Fig. 38) ; they are accompanied for a short distance by a glia sheath which 
after passing through the soft membrane (Fig. 40. a) is separated from them 
by a connective tissue sheath. This extends along the anterior side of the 
spinal ganglion (Fig. 41, b) without entering it (Fig. 41, a) and, in common 
with the posterior sensory root, passes a sheath of the dura (Fig. 40, b) and. 
while giving off fibers to the sympathetic plexus, assumes the part of a mixed 
nerve (Fig. 41, c) and proceeds to the peripheral muscles which it supplies. 



Sensory collaterals to the opposite side 



Column cell 

Golgi's cell 
Commissure cell 
Commissure fiber 
Column cell 



Collaterals of 
the column fibers in the 
terminal arborization 




Branches of the 
posterior root fibers 



Peripheral nerve fiber 



u. Ganglion cells of the 
spinal ganglion 

Posterior (sensory) 
root fibers 



Sensory collaterals with 
terminal arborizations 

Central continua- 
tion of a cell of 
Clarke's column 



Central fibers 
to the pyramidal 
lateral column 



Motor cells of the 
anterior column 



Anterior (motor) 
root fibera 



Column fiber with collaterals 



Central fibers to the pyramidal column 
Fig. 38. — Diagram of Various Tracts and Neuron Systems in the Spinal Cord. 



It has been determined that the majority of fibers in the anterior roots, 
principally motor nerve fibers, run to the muscles (vasomotors?) but we are 
not certain that this is the case with all. We must first follow the central 
path of the motor anterior root fibers to the cells from which they originate, 
and of which, according to the neuron theory, they are constituents. 

Tracing the anterior roots backward, they are seen to pass through the 
white substance with a slight ascent, isolated by their own medullary sheath 
they proceed between longitudinally rising nerve fibers which extend to the 




"Fia. 39. — From the Anterior Horn of the Gray Substance of the Spinal Cord. 

(After Kolliker.) 




Pig. 40. — The Anterior Roots and 
their Relations to the Mem- 
branes. (After Toldt.) 



Fig. 41. — Transverse Section through the Inter- 
vertebral, Disc, between the Third and Fourth 
Cervical, Vertebrae. (After Toldt.) 

49 



50 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



anterior columns, and pass to the lateral column at their boundary (Fig. 
37, a, Fig. 38, Fig. 39, a). Thus they reach the anterior horns of the spinal 
cord, and prove to be nerve processes from some of the large anterior horn 
cells situated there (Fig. 39), particularly from the anterior median group 
(Waldeyer). It must be remembered that in the anterior horns there are 
many cells which have nothing in common with the anterior roots, and which, 
as Ramon maintains, belong to altogether different neurons. 

The arrangement of the first motor neuron of the spinal cord is therefore 
simple and readily comprehended. It is composed of a number of large ante- 
rior horn cells with their dendrites, and of anterior roots which emerge from 
the lateral motor nerve. At the points of cervical and lumbar enlargement 

the number of neurons is 
much greater, because there 
is a larger number of mus- 
cles to be supplied than is 
the case in the thoracic 
cord. 



(b) The First Motor Neu- 
ron in the Brain 

The origin of the first 
motor neuron of the brain 
is somewhat more compli- 
cated. Here we must first 
determine the origin of the 
motor cerebral nerve, the 
nuclei of which are identi- 
cal with the neuron cells 
under consideration, and 
are similar to the anterior- 
horn cells of the spinal 
cord. I take it for granted 
that the peripheral course 
of the nerve fibers is under- 
stood, and shall refer mere- 
ly to the fact that the fol- 
lowing nerves will be con- 
sidered (Figs. 42 and 43) : 
The oculomotor nerve (the 
third nerve), the trochlear 
(the pathetic or fourth 
nerve), the abducens (the 
sixth nerve), the facial (the 
seventh nerve), the spinal 
accessory (the eleventh 
These, as is well known, are 




Fig. 42. — Medulla Oblongata, Pons, Peduncles, and 
Adjacent Parts Seen from the Base. (After Toldt.) 



nerve), and the hypoglossal (the twelfth nerve). 

purely motor nerves. In addition are the mixed nerves, the trigeminal (fifth 

nerve), the glosso-pharyngeal (the ninth nerve), and the pneumogastric (tenth 



NEURON SYSTEMS AND NEURON DISEASES 



51 



nerve). We know of the last three that they possess a motor root besides 
their main sensory root. We will now discuss the origin of this first motor 
tract of the cerebral nerves in so far as it has been revealed bv recent inves- 



tigations. 



We seek the origin of the oculomotor nerve toward the top of the brain, 
but still in that portion which we designate the middle brain; here also we 




Fig. 43. — Base of the Braix axd Cerebral Nerves. 



look for its chief divisions in man, the corpora quadrigemina (see Figs. 44 
and 45) above, the fissure of Sylvius below, and beneath these the fiber masses 
of the superior peduncles of the cerebellum and of the pedunculi cerebri (Figs. 
46, 47, 48, 49). Its nuclei (neuron cells) lie along the entire length of the floor 
of the aqueduct of Sylvius, from its frontal origin almost to its caudal end; 
5 



52 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



the nuclei of the trochlear nerves are situated posteriorly where the aque- 
duct opens into the fourth ventricle. We differentiate several groups of 



Corpor. quadrigem 



, Locus coeruleus 



Corp. geniculat. mediale 




Colliculus facialis 
Striae acusticie 



- Ala cinerea 
Calamus scriptorius 



Funicul. gracilis 

Fig. 44. — Prolonged Cord with the Rhomboid Fossa and Corpora Quadrigemina from 

Above. (After Toldt.) 



oculomotor nuclei, the anterior small-celled nucleus, the large-celled, the long 
posterior nucleus, and, finally, that situated in the median line, the medial 
nucleus. The nerve processes of all these cells converge there, without cross- 
ing, and form the oculomotor 
nerve. The processes of a 
small proportion of the dorsal 
cells of the posterior nucleus 
cross to the other side. Clin- 
ical observations and autopsy 
findings show that the centers 
for individual ocular muscles 
have been approximately deter- 




Mid-brain. 



por- 



Anterior 
tion of the 4th 
ventricle. 



Middle portion 
of the 4th 
ventricle. 



Posterior por- 
tion of the 4th 
ventricle. 



— Medulla oblon- 
gata. 




Fig. 45. — Diagram of Fig. 44. (After Toldt.) 



Fig. 46. — Section through the An- 
terior Corpora Quadrigemina. 



M. obliquus superior — 



$mall cell median nucleus (for the M. sphincter pupill.-r) > 

i 
Large cell median nucleus (for the M. ciliaris) , J 



Aqua?ductus cerebri (Sylvii) 



_\ — -. M. sphincter pupillae 
M. ciliaris 

M. rectus lateralis 



For the internal ocular muscles 
(Radix brevis ganglii ciliaris) 



Colliculus superior of the 
corpora quadrigemi 



Tract of the ; 
fibers of the N 



Corpus geniculat 
mediale 

Thalamus 



Corpus genicu 
atum laterale 



Tractus opticus 



'/. c 3 

S ~ E 



1 o 







Obliquu* inferior 
Rectus inferior ' 

Nucleus n. trochlears 
Nucleus n. abducentis 

Fasciculus longitudinalis medialis 



Fig. 47. — The nuclear origin of the oculomotor ami trochlear in the middle brain; their central 
tract (blue) and their union with each other, as well as with the nucleus of the abducens 
through the median longitudinal bundle (red). The division of the nucleus of the oculo- 
motor in the lateral principal nucleus, the small-celled median nucleus, and the large-celled 
median nucleus; the localization of the different fiber groups of the oculomotor in this 
nuclear area. The central course of the so-called pupillary fibers of the optic nerve (tract 
for the reflex contraction of the pupil). (Diagram based on Bernheimer's description.) 

53 



54 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



mined, the reader being referred to the works of Edinger, Westphal, Pick and 
Starr. The united fibers (among these a few crossed ones also) pass trans- 
versely through the tegmentum of the cerebral peduncles, ventrally downward, 
and subsequently appear as a nerve upon the inner side at the base of the brain 




Nucl. mot. n. V - — , 
Nucl. n. VI - 
Nucl. n. VII — 

Nucl. mot. n. IX et X 



.-- Nucl. n. II 
- Nucl. sens. n. V 

.-Nucl. n. vestib. (VIII) 



Nucl. n. XI -~ 
Nucl. n. XII-' 



Nucl. n. cochl. (VIII) 
- Nucl. sens. n. IX 

Nucl. sens. n. X 

Tract, solitarius et 
Nucl. tract, solitarii 

Rad. ascend, n. V 



Fig. 48. — Nuclear Origin of the Cerebral Nerves. (After Toldt.) 

where the cerebral peduncles converge to an angle before disappearing below 
the pons. 

The nuclei of the trochlear nerve unite (Figs. 47-49) behind the nuclei 
of the oculomotor nerve at the height of the posterior part of the posterior 
corpora quadrigemina. The nerve processes of its cells now follow a peculiar 
course. For a short distance the} r run horizontally and caudally, then ver- 
tically (dorsaHy), and internally, until they reach the velum medullare anti- 
cum which, as is well known, covers the fourth ventricle anteriorly, while the 
lingula of the cerebellum extends beyond it. Here in the velum (Fig. 48) the 
fibers cross those of the other side, run alongside the velum, and emerge 
as a nerve at the point where the fourth ventricle forms an angle anteriorly, 
because the superior peduncles of the cerebellum by their divergence from 
the corpora quadrigemina to the cerebellum mark the anterior structure of 
the rhomboid fossa (fourth ventricle). 

The origin of the other motor cerebral nerves must be sought in the 
medulla oblongata, where they are distributed throughout its entire extent. 
Some are in the anterior part of the rhomboid fossa above the pons, others 
in the posterior part which is characterized by pyramids from the base, while 
a third area for their origin is in the medulla oblongata below the fourth 
ventricle and extending caudally far down into the cervical cord. 

We would digress too far were we to trace accurately the topography of 
these various origins. I refer all those who are interested to the above men- 



NEURON SYSTEMS AND NEURON DISEASES 



oo 



tioned text-books of Edinger, Obersteiner and v. Bechterew. I shall only 
attempt to review briefly their complicated relations. 

The motor root of the trigeminal or trifacial nerve (the nerve for the 
muscles of mastication. Figs. 48 and 49) rises in the extreme anterior part 
of the medulla oblongata (therefore frontally). In contrast to the very 
prolonged sensory nucleus its origin is somewhat circumscribed, lying ver- 
tically above the place where the nerve emerges from the pontal fibers. We 
find its nucleus not far below the anterior third of the rhomboid fossa at its 
lateral border in the region of the locus cceruleus. Naturally the locus coeru- 
leus must be divided, i. e.. a part of the sensory trigeminal nucleus which 
has been stained by pigment, and which during its prolonged course lies lat- 
erally in the rhomboid fossa, is situated just below the surface. The fibers 
pass from the motor nucleus without crossing and almost horizontally (a 



Nucl. n. Ill 
Nucl. n. IV 
Rad. n. IV s 



Nucl. rad. desc. n. V ^ 
Nucl. mot. n. V 
Nucl. sens. n. V v 
Pars sec. rad. n. VII _ 
Nucl. n. VIII. 
Genu int. n. VII — 
Nucl. n. VI- 
Nucl. n. VIII 
Pars prim. rad. n. VII 
Nucl. n. VII' 

Nucl. sens. n. IX ' 
Nucl. sens. n. X -' 

Nucl. n. XII S 
Nucl. mot. n. IX et X ' 

Nucl. n. XI ' 
Tract, solit.-et Nucl. tr. solit.'' 

Rad. asc. n. V / 



„ Aquicd. Sylvi 

uci. ruber 




n. Ill 
Fascic. longitud. 

Brach. pontie 
. Port, major n. V 
Port, minor n. V 
--^g,-^ Gangl. Gasseri 

V>Ram. n. trigm. I, II, III 

..Rad. n. VII 

,„.-' N. interm. 

Gangl. spirale et rad. n. VIII 
— Gangl. vestibul. et rad. n. VIII 
Rad. n. VI 
angl. petros. et rad. n. XI 

Gangl. jugul. et Rad. sens. n. X 

mot. n. X 
XII 



Rad. post. n. spin. * 
Fig. -43. — Nuclear Origix of the Cerebral Nerves. (After Edinger.) 



little outwardly) through the pontal fibers at the point where these run to 
the cerebellum as its superior peduncles. Its nerve fibers are situated within 
this longitudinal fiber layer through which they pass to those of the sensory 
trigeminal roots; thus we see at the base of the brain between the pons and 
superior peduncles a common root emerging from the cerebellum. 



56 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



Close to the motor origin of the fifth nerve, and upon about the same 
plane, but extending rather caudally than ventrally, is the lengthy origin of 
the facial nerve (Figs. 48, 49, 50). Its nerve-cells extend caudally to that 
point upon the surface where the rhomboid fossa terminates. Here it is 
divided by the sensory root of the trigeminal nerve, and posteriorly by that 
of the pneumogastric nerve. 1 Its nerve fibers run a peculiar course. They 

















Fig. 50. — Facial Origin and its Surroundings; Namely, Nucleus and Fibers of the 
Auditory Nerve. (After Edinger.) 

at first rise dorsally above the long series of nuclear origins until immediately 
below the floor of the fourth ventricle. Here nearly all make a right-angled 
curve to the front, and thus form a bundle which passes for a short distance 
anteriorly, then suddenly forms another angle, the crus of which again curves 
ventrally and somewhat outwardly, thence runs directly and without cross- 
ing to the base, where (after passing through the pyramidal tract) it appears 
behind the pons at that point where the posterior olive, the border of the 
pons, and the restiform body form a small triangular groove. Therefore, 
the facial nerve in its peculiar course forms a double bend. 

In the convexity of this knee, immediately under the floor of the fourth 



i I shall at present not discuss the other masses which force themselves between 
the origin of the facial and that of the superficial sensory nuclei; for example, the 
fibrse arcuatae, and the reticular substance. 



NEURON SYSTEMS AND NEURON DISEASES 



57 



ventricle, about at the boundary of the anterior and posterior half of the 
same, and quite close to the median line we find in a narrowly limited 
area the nuclear origin of the abducens (Figs. 48, 49, 50). They, therefore, 
have a different plane from the nuclei of the motor trigeminal and facial 
nerves (Fig. 48). The nerve fibers of the abducens run horizontally, without 
crossing ventrally, somewhat outwardly but again inwardly after passing the 
pontal fibers, and thus at the height of the facial nerve, they appear at the 
base at just the point where the posterior olive and the pyramids unite with 
the posterior margin of the pons (Figs. 42 and 43) and form a groove. 

The motor nucleus of the pneumo gastric, also called the nucleus ambiguus 
(Figs. 48, 49, 52), lies caudally behind the facial, at the point where the 
rhomboid fossa reaches its posterior end. This nerve, like the facial, is over- 
lapped by the sensory nuclei of the fifth nerve, above which (therefore dor- 




Fig. 51. — Section in the Region of the Origin of the Abducens. 

(After Edinger.) 



The Vestibular Nerve. 



sally) and immediately below the floor of the fourth ventricle, the nuclei of 
the sensory glossopharyngeal and vagus are arranged. Its nucleus extends 
caudally about as far as the posterior olive, but much further anteriorly, 
about half-way to the origin of the facial. Its nerve fibers follow a knee- 
shaped course similar to that of the facial, although they do not form a double 
knee, but merely pass dorsally and somewhat frontally to the region of the 



58 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



sensory vagus nucleus, there bending and forming an angle to unite with the 
sensory main root which rises here. Together they pass outwardly and a 
little downward through the region traversed by the nuclei of the fifth nerve 
and their fibers (ascending trigeminal root), and emerge at the point where 
the main branch of the vagus also appears conjointly with the glossopharyn- 
geal nerve; namely, in a space formed by the junction of the posterior olive 
with the remains of the lateral column and the restiform body (Figs. 4? 
and 43). 

The spinal accessory (Figs. 48, 49 and 53) springs from behind the nucleus 
ambiguus in the same plane of the transverse section of the medulla oblongata. 




Fig. 52. — Section through the Medulla Oblongata. (After Edinger.) 

In its lengthy course its cells are found distributed as low as the sixth cervical 
vertebra. Its nucleus, therefore, has its frontal beginning at the pyramidal 
decussation just where the latter end of the posterior olive is visible upon 
transverse section, but it extends caudally far above the pyramidal decussation 
into the cervical cord (Fig. 49). The characteristic gray substance of the 
spinal cord is for some time distinctly visible, yet the original cells of the 
spinal accessory are still perceptible. We find them in the upper cervical 
cord, in those areas in which the lateral horns are more conspicuous than in 
the caudal cervical cord. Thence the fibers of the spinal accessory pass hori- 
zontally through the lateral column and outward. Still higher, at the height 
of the lateral horns, where, by manifold constrictions and changes of the gray 
substance, and especially by the pyramidal decussation, its characteristic but- 
terfly appearance becomes obliterated, the nerve continues its course, and on 
the same frontal plane as the ambiguus vagi, the facial, and the motor tri- 
geminal (Figs. 48, 49). In a simple bend the cells of the spinal accessory 



NEURON SYSTEMS AND NEURON DISEASES 



59 



send their fibers outwardly, first to the front, then horizontally and laterally. 
Thus these root fibers of the spinal accessory combine and form the nerve which 
passes out of the cervi- 
cal cord, between its an- 
terior and posterior 
roots, into the lower por- 
tion of the medulla oblon- 
gata at the point where 
the remains of the lat- 
eral columns extending 
caudally from the olive 
may still be recognized 
externally ; from its low- 
est point of origin the 
canal of the spinal cord 
rises vertically, and its 
branches from the cer- 
vical cord and medulla 
passing to it. shoot-like, 
finally enter the base 
of the brain close to the 
trunks of the glosso-pha r- 
yngeal and pneumogas- 
tric nerves (Fig. 43). 

The hypoglossal nerve remains as the last motor cerebral nerve (Figs. 48, 
49, 53, and 54) to be discussed. Its nucleus, like that of the abducens (Fig, 




nst 



N30L ^ ■ f "NXH 

decussated fibre* ascending 
from the spinal cord 

Fig. 53. — Section- through the Medulla Oblongata at 
the Poixt of Exit of the Pxeumogastric. (Diagram 
after Edinger.) 




y-J /~\ >* Lnt.JuLu m n n ucleu? 



JC hypoylos 



Fig. 54.- 



-Section through the Medulla Obloxgata at the Height of the Posterior 
Hypoglossal Roots. (Diagram after Edinger.) 



60 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

48), is external to that chain of nuclei with which we have just become 
acquainted. Although a motor nerve, we find it in the region of the sensory 
nuclei of the medulla. Functionally it resembles the anterior horn cells of 
the spinal cord pressed into the median line and dor sally (by the pyramidal 
decussation through the reticular substance and the lemniscus tract), while 
the anterior motor cerebral nerve nuclei retain their position like the ante- 
rior horns of the spinal cord. Its caudal beginning, at about the height 
of the pyramidal decussation, occupies a corresponding position with that of 
the anterior horns of the spinal cord, ventrally close beside the nuclei 
of the spinal accessory. Frontally, however, the hypoglossal turns to the 
median line, and simultaneously into the dorsal area of the medulla, so that 
at the height of the fourth ventricle it is already upon its floor (Fig. 49). 
The central canal of the spinal cord, upon the anterior side of which the 
nucleus of the hypoglossal was originally situated, opens into the fourth ven- 
tricle. Thus we find the nucleus of the hypoglossal, when it has reached the 
region of the fourth ventricle, upon the floor of the same structure upon both 
sides of the median line, occupying the posterior quarter. The nerve fibers 
of the hypoglossal pass in a straight line without crossing, obliquely outward 
and downward, and form a number of bundles between the lower olive and 
the pyramid. 

If, in connection with this description, we bear in mind the branches 
of the motor cerebral nerve passing to the periphery, we have a summary 
of the entire first motor neuron of the brain. 

(2) THE SECOND MOTOR NEURONS 

The cerebral nerve nuclei, as well as the motor cells in the anterior horns 
of the spinal cord, are united with the terminal twigs of other nerve fibers 
which, with the nerve-cells belonging thereto, form a second neuron which 
has also a motor function. This second centrifugal tract originates in the 
cortex of the brain, and passes in a course now to be described from a definite 
area of the same to the cells of the first neuron. In diseases of either of the 
two motor neuron tracts, the effect upon muscle movements invariably differs 
to a certain extent. 

Degeneration of the first neuron leads to flaccid paralysis of the affected 
muscle which is total and irreparable, and atrophy rapidly appears in these 
muscles (see below). 

It is different with the second motor tract, through which are conveyed the 
conscious impulses of the will. Without it, even with an intact first neuron, 
active contraction of the muscle is impossible, and only unconscious and reflex 
movements are produced. A permanent interruption of this second neuron 
does not produce flaccid paralysis, but spasms and contractures in the mus- 
cle areas affected by disease of the second neuron are induced, and these are 
attributed to the absence of control, therefore the inhibition, of impulses of 
the will, and to the preponderance of reflex irritation. Often, however, the 
paralysis is complete. 

The second motor neuron originates in those portions of the cortex of 
the brain which we designate as the motor. From animal experiments (Ewald 
and Hitzig, H. Munk) as well as by embryologic investigation and clinical 



NEURON SYSTEMS AND NEURON DISEASES 



61 



research combined with the findings at autopsies, we know that the motor 
cerebral cortex is situated in the posterior and lower portion of the frontal 
lobe, and in the anterior portion of the parietal lobe, especially in the anterior 
and posterior ( ?) central convolutions. Here also there are isolated fields for 



certain motor regions. 



Thus, the center for the lower extremities is situated in the central con- 
volutions on both sides of the upper portion of the central furrow (Figs. 




Fig. 55. — Convexity of the Brain from Above. (After Toldt.) 

55-57); those for the upper extremity in the lower portion; for the facial 
and hypoglossal nerves the center is quite low down; that for the larynx is 
in the operculum; the center for movements of the trunk is in front of the 
centers for the extremities in the posterior part of the two upper frontal con- 
volutions; that for speech beside the center for the larynx in the posterior 
lower frontal convolution close to the operculum. The center for the move- 




Fig. 56. — Convexity of the Brain Seen from the Side. (After Toldt.) 




Ftg. 57. — Motor Region of the Cerebral Cortex in Man. (After Oppenheim.) 
62 



NEURON SYSTEMS AND NEURON DISEASES 



63 



ments of the eye is behind the center for the 
portion of the parietal lobe. 

Here it is worth while to examine the cha 
cerebral cortex which is particularly rich in 
fibers and cells. 

As is well known the entire cortex of 
the brain is composed of several layers. 
Layers of cells of various kinds alternate 
with rows of fine fibers which are arranged 
radially, horizontally or vertically. In dif- 
ferent areas of the cortex of the brain this 
structure is sometimes simple, sometimes 
complicated, and perhaps is most compli- 
cated in the hippocampus major. 

But the motor cortex of the brain also 
is characterized by manifold layers (Figs. 
58, 59). First we have a layer of numerous 
small pyramidal cells below a tangential 
fiber mass composed of a few small cells. 
Then comes a broad, deep layer composed 
of less numerous but very large pyramidal 
cells : all have their apices pointing upward. 
Then follows a fourth layer of small, and 
not very numerous pyramidal cells, irregu- 
larly placed between dense vertical fibers. 
In all these layers there is a considerable 
number of disseminated polygonal cells and 
glia nuclei. The latter are particularly 
dense between the third and fourth layers. 
Any one interested in the structure of the 
cerebral cortex is referred to the excellent 
description of Ramon ( Studien iiber die 
Hirnrinde des Menschen. Leipzig. 1900. 
Ambros. Barth). 

Of all the cells of this part of the cor- 
tex (which with their dendrite nerve fibers 
and collaterals form a very dense network 
that has been explored by Golgi and Ra- 
mon), the large pyramids (Fig. 59. III. i) 
of the third layer are the only ones which 
appear to be regarded as cells of the second 
motor neuron. They are characterized by a 
many-branching dendrite net which extends 
to the surface as well as to the sides, and is 
intimately related to other dendrites. The 
nerve processes rise vertically or radiate 
from this while sending numerous collat- 
erals downward into the white medullary 
mass of the centrum semiovale. 



upper extremities in the anterior 
racteristic structure of the motor 




Fig. 5S. — Sectiox theough the Cor- 
tex of a Frontal Convolution; 
to the Left Cells Staixed by 
Golgi's Method, to the Right 
Fibers Staixed by vVeigert's 
Method. (After Edinger.) 



64 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



Here they take part in that projection fiber system which passes from all 
sides of the cerebral cortex to each hemisphere, just as the so-called fibrilla- 




Fig. 59. — A Partly Diagrammatic Presentation of the Cortex of the Cerebrum After 

Staining with Silver. 

I, Molecular layer; II, small pyramidal cells; III, large pyramidal cells; IV, polymorphic cells 
with medullary layer. (After Obersteiner.) 

tion of the corona radiata passes to the caudally situated areas of the brain 
(Figs. 61 and 62). While a large portion of these fibers pass to the root 

ganglion (corpus striatum) or to the inter- 
r~ ' ■''-'' ' v '-~"^1^ brain (thalamus opticus), the nerve fibers of 

those pyramidal cells in the deeper parts of 
the medullary layer of the hemispheres form 
a distinct bundle, the motor or pyramidal 
trad (Fig. 61). At the level of the corpus 
striatum this enters into the medullary layer 
of the same called the internal capsule, which 
divides the corpus striatum into the caudate 
nucleus and the lenticular nucleus, and shows 
upon horizontal section an obtuse angular 
bend, which opens outwardly (Figs. 51, 55). 
Therefore, upon horizontal section the inter- 
nal capsule reveals an anterior and a poste- 
rior crus. In the anterior third of the poste- 
rior cms is the motor bundle, which contin- 







Fig. 60. — Section through the 
Internal Capsule. 



NEURON SYSTEMS AND NEURON DISEASES 



65 



ues ventrally and caudally and at the base of the brain enters the cerebral 
peduncle running under the thalamus and the corpora quadrigemina (Figs. 
61, 62), the ventral portion of which it forms separated from the dorsal 
through the substantia nigra by the tegmentum. In the fibers of its foot are 
the projection fibers from the central convolutions, as well as branches from 
the frontal, parietal and temporal lobes. 



Fissura longitudinalis cerebri 
Radiatio corporis callosi 



Septum pelluciduru 



Plexus chorioi- 
deus ventricuti 
lateralis 



Gyrus frontalis superior 

Truncus corporis callosi 

Cornu anterius ventriculi lateralis 
Caput nuclei caudati 



Radiatio cor- 
s poris striati 



Putamen 



Capsula 



Plexus chori- 
oideus ven- 
triculi tertii 

Capsula inter- 
na (Peduncu- 
lua corona? 
radiatse) 



Insula 




Cornu infe- 
rius ventriculi"^ 
lateralis 



Pedunculus 
cerebri 



Brachium 
pontis 

Fasciculi longi- 
tudinalis (pyra- 
midales) pontis 

Facies inferior cerebelli 

Fibra? pontis superficialis 

Pyramis medullas oblongata? 

Fig. 61. — The Pyramidal Tract. 



N. glosso- 
pharyngeus 



N. vagus 
Nucleus olivaris inferior 



Decussatio pyramidum 
(After Toldt.) 



66 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



The tracts of the second motor neuron are in the foot; these run side 
by side for a short distance (Figs. 42, 43, 46) but soon diverge, and only a 
comparatively small number of them continue caudahvard. The others ex- 



Lobulus 
paracentral^ 



Corona radiata ^ 



-?- Gyri centrales anterior et posterior 
(upper portion) 



Radix anterior n. spinalis 




Central fibers of the 
pyramidal tract 



Crossed central tract 

of the N. hypoglossals"""---..^ J 



Crossed central tract 

of the N. facialis 



Basis 
peduncuii 



Pons (Varoli) — ^— 



Nucleus 
n. faeialis 

Radix 
n. facialis 



Radix "-- \^f\A 

n. hypoglossi J^\^J \ 

Nucleus n. hypoglossi " \ \\ 



Pecussatio pyramidum ** 



- Gyri centrales 
/ (lower portion) 



: Pars opercu- 
lars of the Gyrus 
frontalis .inferior 



\ Capsul a. interna 



Pyramis (medulla? oblongata?) 



Fasciculus cerebrospinalis (pyramidalis) lateralis 
(pyramidal lateral column) 



•Fasciculus cerebrospinalis (pyramidalis) anterior 
(pyramidal anterior column) 



Motor ganglion cells of the anterior column 



Fig. 62. — The Pyramidal Tract (Red) Beside the Tract of the Hypoglossal and Facial 

Nerves (Black). (After Toldt.) 



NEURON SYSTEMS AND NEURON DISEASES 



67 



tend no deeper than the area of the pons (Figs. 42, -13, 51. Gl) and these 
are mostly transverse fibers which pass from one hemisphere of the cere- 
bellum to the other and also cover from below those fibers which had previ- 
ously centered in the cerebral peduncles. The majority of the motor fibers 
from other peduncles (Fig. 62) are lost in the pons. We are familiar with 
only a part of this divergent tract. By analogy, as well as by direct histologic 
observations (Spitzka), it appears reasonable to assume that these tracts 
diverge only in the pons from those which are situated deeply because they 
unite with the cerebral nerve nuclei in the mid-brain and medulla, and sur- 
round these cells with their own terminal arborizations ; at present we know 
the tracts of only the facial and hypoglossal nerve- (Edinger). At all events. 
at the upper end of the pons where the peduncles are inserted a thick bundle 



Corpus anterius ventric. 
lateralis 
Nucl. 
caudatus > 

Capsula in- 

terna 
. z Puta- 

| s men -J 

5 '— < rlobua 

= 1 palli- 

— • clus 

Capula 

externa .» 

Claustrum JH 

Insula - 

(' immissura 

anterior — p 

Ansa 
lenticularis 

Ansa pedun- 

cularis -""^= 
Substant. 
perforata 
anterior 
Trigonum 
olfactor 



|-» Corpus 
" callosum 




C'ommissura supe- ^ 
rior (Meynerti) 

Fjg. 63. — Frontal Section through the Hemispheres Behind the Optic Chiasm. 

(After Toldt.) 



Nervus 

opticum 



passes dor sail u from the foot to the tegmentum, and this after previous decus- 
sation in the raphe may carry the motor neurons to the cerebral nerve nuclei, 
particularly to both of the above mentioned nuclei. Other fibers of the pedun- 
cles which do not form this bundle, and which extend no further caudally, 
are lost in the numerous nerve-cells of the pons which lie between the fibers 
passing through the cerebellum (the fibers of the middle peduncle of the 
cerebellum ) . 

The remainder of the ventral portion of the crus cerebri which passes 
transversely through the pons as a closed bundle (Figs. 61. 62) and then ex- 
tends caudalward is called the pyramidal tract. This unites at the posterior 



68 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



border of the pons in the form of the so-called pyramids at the base of the 
medulla (Figs. 42, 43, 61, 62), and subsequently with the so-called pyramidal 



Truncus corporis callosi 
Septum pellucidum 

Corpus fornicis 




Uornu inferius 
ventriculi la 
teralis 



Glomus chorio 
ideum 



Radiatio occipi 
to-thalamica 
(Gratioleti). 



Splenium corporis callosi 



Fig. 64. — Horizontal Section through the 

Right Side). 



Eminentia 
collaterals 

Calcar. avis 



Colum posterius 
ventriculi lateral! 



Fissura calcarina 
Hemispheres (Somewhat Deeper upon the 
(After Toldt.) 



NEURON SYSTEMS AND NEURON DISEASES 



69 



Sensory collaterals 



decussation below the point where the lower olives are laterally attached. 
The majority of the fibers in this tract cross to the other side, and simul- 
taneously extend to the ventral surface on that side of the cord, which they 
continue to traverse as the pyramidal lateral column tract (Fig. 37, b). In 
man a few of these fibers do not cross but remain in the anterior columns, 
the so-called Turk's bundle (Fig. 28, i) ; subsequently they also pass down- 
ward into the spinal cord. In the spinal 
cord itself these fibers of the pyramidal 
tract pass from time to time to the large 
motor anterior horn cells with their ter- 
minal filaments (Figs. 38, 65). The 
fibers for the lateral column pass direct- 
ly into the gray substance, those for the 
anterior columns only after the fibers of 
the anterior commissure have crossed. 
It is therefore the property of the sec- 
ond motor neuron (Fig. 62) to termi- 
nate on the side of the antral nervous 
system opposite to that in which it 
originates, and subsequently to sur- 
round the nerve-cells of the first motor 
neuron which, on its part, continues its 
course through the spinal cord and, to 
a great extent, through the brain. 

In reviewing this description of 
the path of the second motor neuron in 
the spinal cord it is evident thai its 
course has been quite accurately deter- 
mined; in the brain, however, it- ter- 
mination near the origin of the cranial 
nerve is still to be found. 

Recently it has been demonstrated that the pyramidal tract in the lateral 
columns of the spinal cord, which had previously been regarded as an entity, 
contains a special variety of fibers, discovered by Monakow, and, according 
to the investigations of Rothmann, this promises to be a valuable addition 
to our knowledge of the processes of motion. This tract, designated as 
Monakow's bundle, extends backward and gradually diverges from the pyram- 
idal tract in such a way that in the pons and in the ventral portion of the 
crus cerebri it enters the tract of the tegmentum, crosses near the corpora 
quadrigemina, and is finally lost in the region of the red nuclei. Correspond- 
ing to this we have also a special motor neuron for the second division, the 
cells of which in all probability also originate in the cerebral cortex; but con- 
cerning this we have no absolute knowledge. 



Fasciculus cerebro- 
spinal lateralis 



Central motor path 
of conduction in 
the lateral or 
crossed pyrami- 
dal tract 



Central motor path 
of conduction in 

the anterior or 
direct pyramidal 
tract 



Motor ganglion cells 



Motor root libers 




Fig. 65. — Course of the Second Motor 
Neuron in the Spinal Cord; Diagram. 
(After Toldt.) 



B. DISEASES OF THE MOTOR TRACT 

Although nerve-cells and processes actually form a histologic and biologic 
unit, nevertheless it is not absolutely necessary that the neuron theory should 
simultaneously stand or fall with this, and that the entire neuron should 



70 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

invariably and uniformly be attacked by disease. Of course, when the nerve- 
cell is destroyed we may quite properly assume that the processes originating 
in it also immediately degenerate. But inversely, when there is destruction 
of one or several processes anywhere in its course and consequent interrup- 
tion of function, we cannot assume that the cell itself and the central por- 
tion of the nerve process must likewise undergo degeneration. On the 
contrary, this is true only of the peripheral end ; cell and process form a histo- 
logic and physiologic entity. Hence, as we maintain that every cell may 
continue to live if the damage which it sustains does not injure too great 
a part of its body, so the nerve-cell also may retain its properties if not too 
large a portion of its processes, that is of itself, is destroyed, and this is 
especially true whenever subsequent restitution is possible. Naturally if the 
degeneration of the nerve fiber is very extensive, and particularly if this be 
permanent, the cell itself will suffer. This has been proven by pathologic 
histology, as well as by experiment. 

The observation is frequently made that a nerve tract is primarily only 
partially diseased, and later wholly degenerates; this does not conflict with 
the neuron theory. On the contrary, such diseases of the nerves are the 
strongest proofs that an entire neuron may be attacked or several individually. 

Such primary diseases of neurons — after the foregoing we are interested 
only in the motor and sensory, other neurons having been as yet but little 
investigated — may quite properly be designated as neuron diseases, and may 
be differentiated from other affections of the nervous system which are caused 
secondarily by the vessels, or by the supporting tissue, or by the effect of 
traumata and tumors affecting the nerve tracts. 

I shall attempt in the following to select from the whole series of dis- 
eases of the nerves those neuron diseases with which we have become familiar, 
and to portray these as such. It is an initial effort, and this must excuse its 
incompleteness. 

I shall begin with neuron diseases of the motor tract, chiefly from a histo- 
logical standpoint, without minutely discussing their clinical aspects. The 
subject of sensor}?- neuron diseases will follow the description of the normal 
sensory neuron. 

There must be no misapprehension if a comparatively small number of diseases 
follows the preceding theoretic explanation. On the contrary, I must state that we are 
only groping in the dawn of scientific research, and that this subject still contains many 
dark problems. Above all, autopsies and investigations with approved modern methods 
are most necessary; for the great abundance of material, the diseases often running 
an exceedingly slow course, do not permit the clinical and larger institutions to investi- 
gate these as distinct diseases because many carefully observed cases die outside the 
hospital from intercurrent affections, and thus afford no opportunity for the autopsy 
which is so necessary for the scientific elucidation of affections of the nerves. 

(1) DISEASES OF THE FIRST MOTOR NEURON 

(a) Its Multiple Affections in the Spinal Cord 

The Spinal Form of Progressive Muscular Atrophy. — Clinically we are 
all familiar with the characteristic disease which Duchenne and Aran first 
designated as progressive muscular atrophy, beginning very slowly in the 
upper extremity, and finally distributing itself to other parts. 



NEURON SYSTEMS AND NEURON DISEASES 71 



Autopsies conducted with the aid of recent methods — notwithstanding 
the frequency of the disease but few reports are at hand — have shown that a 
typical neuron disease here forms the basis of the muscular atrophy. The 
motor cells of the anterior horns of the spinal cord (probably with their 
dendrites) and the nerve fibers originating from them (through the anterior 
roots), even the atrophied paralyzed muscles, are primarily degenerated. In 
place of the absent or atrophic nervous substance proliferated glia tissue 
appears, and the walls of the vessels are thickened. The affection is therefore 
not a primary disease of the muscle, but a degeneration of the neurons sup- 
plying the muscles, with secondary atrophy of the muscles. 

(b) Multiple Motor Neuron Diseases in the Brain 

(i) Chronic Progressive Bulbar Paralysis. — This disease of the spinal 
cord is closely related to that of the medulla oblongata which exclusively affects 
the cells of the motor nuclei and their fibers in this region; therefore the 
first motor neuron. The elective affinity for the motor tract which this 
process here displays is the more marked because the sensory nuclei and their 
tracts often lie in the immediate vicinity, yet are not implicated. Here also 
we rind, as in the preceding form, destruction of the motor nerve-cells: Degen- 
i ration of the motor cerebral nerves and disappearance of their root fibers. 
In place of the absent nervous structure there is a proliferated glia tissue. 
The disease of the spinal cord sometimes changes into the bulbar form so 
that the entire peripheral motor tract is involved. 

(ii) Progressive Ophthalmoplegia. — In this disease the process chiefly 
attacks the motor nerve nuclei of the medulla oblongata, while the nuclei 
of the muscles of the eye with more frontal situation are exempt or are only 
secondarily implicated. 

On the other hand, there is a quite rare affection in which the more 
frontal nerve nuclei are predominantly involved, especially the nuclei of the 
oculomotor and the trochlear nerves in the mid-brain, occasionally also those 
of the abducens, and in very severe case- there is a partial implication of the 
nucleus of the facial nerve. Both of the latter, as is well known, belong to 
the anterior divisions of the medulla oblongata. 

Here we are dealing with a lesion of the anterior portion of the first 
motor cerebral neuron, and therefore this affection, clinically named pro- 
gressive ophthalmoplegia, may be included among the neuron diseases. 

(Hi) Circumscribed Diseases in the Area of the First Motor Neuron. — 
The affections previously mentioned are diseases of the spinal cord or the 
brain which implicate a great number of neurons. The more diffuse affections 
are contrasted with those which occur in a circumscribed area, in an isolated 
nerve nucleus, or which implicate only the motor conduction tract belonging 
thereto. Among those of the brain are isolated nuclear paralyses, and of the 
spinal cord those paralyses designated as monomuscular, which, however, are 
in part not of peripheral origin but central, their origin being in a corre- 
sponding portion of the anterior horns of the motor nerve, the actual focus 
of the disease. 

There is a great paucity of literature on this subject, not because the cases 
are rare, but because, for reasons above mentioned, they have not been studied. 



72 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

There are no cases of undoubted disease of the spinal cord which may be 
regarded as central monomuscular paralysis or primary affection of the neuron ; 
of course, secondary disease following tumors and inflammatory processes of 
the membranes of the brain, and particularly after injuries, is often reported 
in literature. 

Idiopathic nuclear paralyses have, on the contrary, been histologically 
determined in the brain. The oculomotor nerve furnishes an example of such 
a circumscribed neuron disease, which is frequently seen clinically, and more 
rarely anatomically. Here we do not consider the progressive form of paral- 
ysis mentioned above, but that dependent upon a different etiologic base 
(migraine, syphilis, etc.), and which is often of benign type. 

Other nuclei of the nerves of the muscles of the eye, especially the abducens, 
occasionally suffer from similar attacks. 

Recently the neuron diseases of the spin?.! accessory have been studied, 
but the material for investigation is exceedingly scant, and additional re- 
searches are necessary. 

No further description of the histologic picture is necessary ; its prominent 
features remain the same : Disappearance of the nucleus and fibers in the 
entire course of the neuron. 

(2) DISEASES OF BOTH MOTOR NEURONS 

Atrophic Spastic Bulbospinal Parahjsis (Senator); (Amyotrophic Lateral 

Sclerosis, Charcot). 

We as yet know little of primary diseases which attack the second motor 
neuron exclusively, either in its entire extent or in any part of its course. 
Naturally, the most common and best known are secondary lesions and inter- 
ruptions of continuity in various areas of these tracts (see below). The histol- 
ogy of recent cases of acute polioencephalitis, the counterpart of poliomyelitis, 
which is most often observed in children, has not been sufficiently studied 
for us to pronounce it a neuron disease. 

Charcot has described under the name of spastic spinal paralysis an affec- 
tion with a characteristic symptom-complex, and, because of these symptoms, 
has postulated a primary degeneration of the pyramidal tracts of the spinal 
cord; therefore, according to our conception, the tract of the second motor 
neuron; wherefore, if the neuron theory be otherwise correct, it must also 
include a degeneration of the motor cells of the cerebral cortex belonging 
thereto, a process which has as yet been too little studied. 

So far no conclusive case has been anatomically investigated — the case 
Striimpell reported also showed disease of the posterior column; instead of 
the looked-for changes, the necropsy revealed very different conditions (multi- 
ple sclerosis, combined system diseases, tumors, meningitis). It still remains 
a mooted question whether there really is an isolated affection of the entire 
second motor tract. 

But we recognize distinctly a not very rare disease which was first graph- 
ically described by Charcot; its anatomical course conclusively proves the 
existence of disease of the neurons. Charcot gave to this the anatomico-clinical 
designation of amyotrophic (myatrophic) lateral sclerosis. Senator has de- 
nominated it by a purely clinical term, atrophic spastic bulbospinal paralysis. 




NEURON SYSTEMS AND NEURON DISEASES 73 

Histologically, the affection in its full development reveals the following 
conditions (Fig. 66) : 

(1) Primary degeneration of the first motor neuron similar to that of 
progressive muscular atrophy and chronic bulbar paralysis. 

(2) The same primary degeneration of the pyramidal tracts which was 
postulated by Charcot for spastic spinal paralysis, but which has not yet been 
proven. 

The implication of the motor cerebral cortex, that is, of the cells in which 
the motor tract originate, has not jet been certainly demonstrated and requires 
confirmation ; it is questionable in how far disease of 
these greatly disseminated cells may be histologically 
determined by our ordinary methods. At all events, 
it is not too far fetched a hypothesis to regard these 
cells as implicated in the process. Thus we have a 
picture which most clearly portrays the symptoms of 
disease of both motor neurons. 

In enumerating the diseases of the first motor 
neuron, I stated that our knowledge of these cir- 
cumscribed affections is very limited, and we are Fig. 66.— Traxsverse Sec- 
absolutelv ignorant concerning the changes of this TIOX THROrGH THE Spi - 

i • -i . "l-i -, tVt i o i • , nal Cord in Atrophic 

kind in the second neuron. \\ e know ot no histo- Spastic Bulbospin u 

logic alteration in a cortical area which can be re- Paralysis. 

ferred to an isolated and circumscribed affection of 

a definite muscle or muscle group, which would produce the symptoms of a 

spastic monomuscular paralysis. Perhaps in the future we shall acquire more 

definite knowledge concerning this. 



C. THE SENSORY TRACT 

(1) THE FIRST SENSORY NEURON 

It has been embryologically determined, especially by the investigations 
of His, that the centripetal tracts in the spinal cord and brain which we 
designate as the distribution of the entering posterior roots and of the sensory 
cerebral nerves are at first adjacent and external to the central organ, and 
that after only a definite period of development do they become a part of the 
spinal cord. This makes it clear that even after full development the nerve- 
cells belonging to these fibers are situated outside of the central nervous 
system; such are the cells of the spinal ganglia (Fig. 29) in which the sensory 
cerebral nerves resemble the spinal ganglia, the included ganglia nodules, 
this knowledge being also based upon the experimental severing of the pos- 
terior roots. Here the following ganglia must be included (Fig. 73) : In the 
vagus and the glossopharyngeal nerve the jugular and petrosal ganglia; in 
the auditory nerve the spinal ganglion (cochlea?) and the vestibular branch 
of the same, the vestibular ganglion ; in the trigeminal the Gasserian ganglion ; 
in the optic nerve the ganglia of the retina ; in the olfactory those of the 
mucous membrane of the nose. The nerve centers external to the central 
nervous system are the origin of the peripheral centripetal, apparently the 
entire, sensory tract. They are, therefore, the cells of the first sensory neuron, 



74 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



and the posterior roots of the spinal cord and the sensory cerebral nerves are 
the nerve processes belonging to it. 

The cells of the centers just mentioned differ markedly in their structure 
and in the nature of their arborizations from the motor cells. In the first 
place, they are deficient in dendrites; indeed, it is not certain that the cells 
of the spinal ganglion possess dendrites. Furthermore, they are usually not 
angular and polygonal like the motor cells, but are round or oval, like vesicles. 
A covering of connective tissue with nuclei protects them from injury in their 
peripheral position. The branching of the nerve processes is also characteristic 
of the sensory neuron. 

Near its origin this divides into two parts, one of which by a longer or 
shorter route soon passes to the sensory end organ where it terminates in vari- 
ous ways, while the other proceeds toward the 
central nervous system, and there pursues a 
course which is soon to be described. 

(a) The First Sensory Neuron in the Spinal 
Cord 

We shall first trace the course of the nerve 
fibers of the first sensory neuron in the spinal 
cord. The nerve process passes only a few 
millimetres from the cells of the spinal gan- 
glia when it divides into a peripheral and a 
central branch (Fig. 29). The peripheral 
branch, after leaving the spinal ganglion and 
uniting with fibers of the anterior root, passes 
the processes of the first motor neuron in the 
spinal nerve (Fig. 40) between the vertebral 
canal and the periphery, where it separates 
from the motor fiber and extends to the skin 
jgj\ iWi P anc ^ ^ ne °ther organs which the sensory nerve 

supplies. After forming a dense net in the 
cutis by the division and branching of its 
fibers, it terminates in various end-apparatus 
(which need not here be further described 
(Fig. 29). 

The central process (Fig. 29) within the 
posterior roots passes to the spinal cord. We 
shall now trace the somewhat complicated 
course of these sensory posterior root fibers 
in the spinal cord. 

Every posterior root after entering the 
spinal cord divides into two groups of fibers 
(Figs. 67, 68) each of which pursues a dif- 
ferent course. The group of thicker fibers 
(Fig. 70, rpi) passes to the posterior column, the other passes to the pos- 
terior horns (Figs. 67, 69). Immediately after entering the spinal cord 
(in the angle formed by the junction of the periphery of the posterior 




Fig. 67. — Diagram Showing the 
Branching of the Dorsal 
Roots. (After Ramon.) 



NEURON SYSTEMS AND NEURON DISEASES 



75 



columns and the posterior horns) the fibers separate into two branches a 
short descending and a longer ascending branch (Figs. 68, 69). These last- 
named branches pass toward the brain as far as the medulla oblongata (see 
below). During their course they constantly give off collateral branches to 
the gray substance, particularly those which penetrate the same side of the 
posterior horn to the cells of Clarke's columns, or by the posterior commissure 
upon the opposite side (Fig. TO). A few collaterals transversely describe an 
arc through the posterior horn of the same side, in front of the gelatinous 




Fig. 68. — Diagram of a Transverse Section of the Spinal Cord; Tracts of the First 
Division Continuous; Tracts of the Second Division Interrupted. (After Edinger.) 



substance and outwardly into the lateral column, thence into unknown regions 
(Bechterew) (Figs. 68, 69). The descending roots in the posterior column 
follow a similar course (Schultze's bundle?) and finally appear to terminate 
in the gray substance of the posterior horns. Each root as it ascends (Fig. 
37, e ) inserts its fibers laterally beside those which have entered below, thus 
medially forcing the preceding ones away from the zone of entrance. Thus 
it happens that the posterior roots which effected an ingress low down in the 
lumbar cord are found medially high up in the cervical cord, in G oil's columns 
Figs. 37, e, 38, 68, and 69) while those which entered high up are still in 
Burdach's column (Fig. 37, d). Hence Ave find that the posterior root fibers 
and the posterior lateral area, filled by the same, are together designated the 
entrance zone of the roots. 

The other, more lateral group of posterior root fibers (Figs. 67, 68, 69, 
V0,rpe) which entered the posterior horn is composed of fibers having a two- 



76 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



fold course. One group passes horizontally through the gray substance directly 
to the large cells of the anterior horn (Figs. 69, 71). The other group, usually 



Fasciculus anterior propnus 



Fasciculus lateralis 
proprius 



Collaterals 

Ascending and descending 

branch of the sensory 

root fiber 



Spinal ganglion 
Sensory root fiber 




Fasciculus cerebello- 
spinalis 



— Tract fibers with 
collaterals 



Central path from Clarke's 

column (passing upward 

in the direct lateral 

cerebellar tract) 



Fig. 69. — Diagrammatic Course of the First Sensory Neuron. (After Toldt.) 



thin fibers, divide at the apex of the posterior horn, or perhaps just within it, 
occasionally in front of the gelatinous substance, into an ascending and de- 
scending branch. The finest fibers, which separate as soon as they enter the 




Fig. 70. — Diagram Showing the Origin and Termination of the Nerve Roots in the 
Gray Substance of the Spinal Cord and the Distribution of the Nerve Cells in 
the Latter. (After Bechterew.) 



NEURON SYSTEMS AND NEURON DISEASES 



77 



apex of the posterior horn, form upon transverse section a small area, the 
so-called border zone of the posterior horn, or Lissauer's zone (zona terminalis) 
(Figs. 68 and 70, z). The branches of this group of posterior root fibers also 
give off numerous collaterals some of which extend anteriorly far into the gray 
substance while others terminate in the small cells of the posterior horn (Fig. 
68). The mode of termination of all these 
processes and collaterals is invariably the 
same, and occurs by splitting up, so that 
the terminal twigs envelop the cells which 
they approach, or split up in the vicinity 
of their dendrites (Fig. 61). 

The fibers which ascend to the medulla 
oblongata terminate there. At the begin- 
ning of the prolonged cord numerous 
nerve-cells appear in the posterior column, 
the nuclei of GolVs columns (funiculi gra- 
ciles) and of Burdach's columns (funiculi 
cuneati) which may be followed to the 
beginning of the fourth ventricle (Fig. 71). 
Here all the fibers of the posterior roots 
split up, and here the first sensory neuron 
of the spinal cord terminates in a web of 
cells. 

(b) The First Sensory Xeuron of the 
Cerebral Nerves 

The intramedullary course of the first 
neuron of the sensory cerebral nerves 
which, corresponding to those of the pos- 
terior roots of the spinal cord, possess a 
root for each isolated sensory nerve, is a 
varying one. In the main, it resembles 
that of the spinal cord. Its origin is in 
the peripheral ganglia (apparently only 
included in it) (Fig. 73, see above) which, 
in the nerves of special sense (the optic, 
the auditory, the olfactory, is situated 
in the end organs of special sense. After 
they have entered the brain they terminate 
by splitting up around the clumps of 
nerve-cells which correspond to those nuclei of the funiculi gracilis and cuneati 
around which the ends of the spinal cord roots divide. 

Formerly these nuclei were considered to be the origin of the sensory 
cerebral nerves. We now know, from the results of Golgi's method, that 
they terminate there, 1 and that from these cells other fibers originate which 
form a second neuron that we shall discuss later. 




Fig. 71. — Diagram of the Coerse of the 
Sensory Tracts from the Posterior 
Roots to the Prolonged Cord. (Af- 
ter Edinger.) 



1 In the nerves of special sense (the olfactory, the optic, the auditory) there are also 
fibers with a centrifugal course and of unknown function whose neuron cells also ter- 
minate in the above mentioned cerebral nerve nuclei. 



78 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



If we remember these important relations of the sensory cranial nerve 
roots to their peripheral ganglia and to their cerebral nuclei, in a description 
of their intracerebral course we may for special reasons disregard the earlier 
view, that the fibers originate from the nuclei. I shall hereafter follow this 
plan. 

I shall therefore begin with the sensory nuclei in the medulla oblongata 
around which the sensory nerves split up. If we recall the position of the 
motor nuclei, it will be easy to follow the sensory, particularly their relations 
to the motor. I therefore refer the reader to what has been said above, and to 
Figs. 72 and 73. 

The trigeminal nerve has the longest, most extensive termination of all 
the cerebral nerves. Its nucleus begins far down in the cervical cord, even 
below the origin of the spinal accessory. The gelatinous substance of the 
posterior horns and their cells are the ones with which it unites. In the 
medulla oblongata the nuclei of the posterior columns and subsequently also 
other cerebral nerve nuclei force the gelatinous substance and the nucleus 
of the trigeminal nerve from their dorsal position and inward somewhat 
ventrally. The nucleus, however, retains its lateral position (FigS. 74, 75, 
76), therefore remains external to the portions named above, the long motor 
cells iiHhe medulla oblongata which form the origin of the first motor neurons 
of the brain. In the region of the rhomboid fossa it follows a deep lateral 
course up to the anterior middle of this structure, where the nerve itself from 




Nucl. mot. n. V -■ — 
Nucl. n. VI - 
Nucl. n. VII — 

Nucl. mot. n. IX et X 



=- Nucl. n. II 
- Nucl. sens. n. V 

.-Nucl. n. vestib. (VIII) 



Nucl. n. XI 
Nucl. n. XII- 



— Nucl. n. cochl. (VIII) 
- Nucl. sens. n. IX 
Nucl. sens. n. X 

Tract, solitarius et 
Nucl. tract, solitarii 

Rad. ascend, n. V 



Fig. 72. — The Nuclear Origin of the Cranial Nerves. (After Toldt.) 



without joins the nucleus as a thick bundle (from the Grasserian ganglion) 
accompanied at first by the motor root which we have already described, and 
passes from the base obliquely, from without and ventrally inward, and hori- 
zontally toward the dorsum through the external divisions of the pons (Fig. 



NEURON SYSTEMS AND NEURON DISEASES 



r 9 



77). After the fibers of the sensory trigeminal root have reached the nucleus 
just described, like all sensory nerve roots they divide into two branches (see 
above, the spinal cord roots). The shorter one passes to the nerve-cells near 
its point of entrance, while the other descends and gradually becomes smaller, 



Nucl. n. Ill ^ 
Nucl. n. IV 
Rad. n. IV s 

Nucl. rad. desc. n. V ^ 
Nucl. mot. n. V >. 
NucL sen?, n. V^ 
Pars sec. rad. n. VII ^ 
Nucl. n. VIII.^ 
Genu int. n. VII—- 
Nucl. n. VI"- 
Nucl. n. VIII - 
Tars prim. rad. n. VII ■"■* 
Nucl. n. VII - 

Nucl. sens. n. IX "' 
Nucl. sens. n. X 

Nucl. n. XII 
Nucl. mot. n. IX et X 

Nucl. n. XI 

Tract, solit.-et Nucl. tr. solit. 

Rad. asc. n. V 
Rad. post. n. spin. 



^ Aquacd. Sylvii 
- Nucl. ruber 

r Rad. n. Ill 

s Fascic. longitud. 

, Brach. pontis 

Port, major n. V 
A' \"/ Port, minor n. V 

<- Gangl. Gasseri 

^Ram. n. trigm. I, II, III 

^Rad. n. VII 

___-" N. interm. 

Gangl. spirale et rad. n. VIII 
""—Gangl. vestibul. et rad. n. VIII 
~~~~ Rad. n. VI 
*" Gangl. petros. et rad. n. XI 

Gangl. jugul. et Rad. sens. n. X 
' Had. mot. n. X 
% Rad. n. XII 
N. XI 
1 Rad. cerebr. n. XI 

Rad. spin. n. XI 
Rad. ant. nn. spin. 




Fig. 73. — The Nuclear Origix of the Cranial Nerves. (After Edinger.) 

supplying all the cells of the gelatinous substance down into the cervical cord 
(ascending 1 trigeminal root) (Fig-. 73-76). 

Now there is also an ascending trigeminal root (Figs. 72, To, 96, 97. 98). 
This is distributed about nerve-cells which may be traced to the middle brain. 
Its most frontal position is in the region of the corpora quadrigemina, later- 
ally from the aqueduct of Sylvius, therefore laterally to the nuclei of the 
oculomotor and trochlear, and at the same time somewhat toward the dorsum. 
Thence the nucleus of the descending trigeminal root extends caudally up to 
the lateral borders of the anterior portion of the fourth ventricle where it 
approaches the surface and where, by pigmentation of its cells, it becomes 
visible as the locus cceruleus (Fig. 78). There it unites with the main nucleus 
of the trigeminal nerve. 



i According to the neuron theory, the designation 
correct one. 



descending root " would be the 



80 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



We are still in doubt as to the relations of the descending trigeminal roots 
to this nucleus. It is uncertain whether the fibers of the nerve here split, or 




Fig. 74. — Section through the Medulla Oblongata. (After Edinger.) 

whether they rise at this point, and therefore constitute a descending cen- 
trifugal tract. In fact, the nerve fibers of this tract unite with those of the 
motor, not with the sensory root (radix mesencephalica). 




NXt 



Decussated fibres ascending 
from the spinal cord 

F; g. 75. — Section through the Medulla Oblongata at the Point of Exit of the Pneu- 
mogastric. (Diagram after Edinger.) 



NEURON SYSTEMS AND NEURON DISEASES 



81 



We next consider the nuclei of the pneumo gastric, the sensory root of which 
arises from the jugular ganglion combined with that of the glosso-pharyngeal 
which originates in the petrosal ganglion in the medulla oblongata close 
behind the auditory nerve (see below) between the olive and the restiform 
body (Fig. 77). The motor origin of the vagus in the nucleus ambiguus has 
already been described (Figs. 72, 73, 74). It is situated in that chain of 
motor cells the position of which about corresponds to the lateral horn of 
the cervical cord. Dorsally from this, in the position in which the posterior 
horns were formerly situated, and simultaneously forced to the interior by 
the nuclei of the posterior columns pressing to the side, is the sensory nucleus 
of the pneumogastric (Figs. 72, ^3, 74. 76). It originates in the medulla 



Xoelf-3 raC - 




\ / 

J/ll^culumn nucleus 



Fig. 76. — Section through the Medulla Oblongata at the Height of the Posterior 
Hypoglossal Roots. (Diagram after Edinger.) 

about at the point where the olives trend somewhat more ventrally, and passes 
frontally as far as the beginning of the second third of the fourth ventricle. 
Its anterior point is. of course, to be distinguished as the nucleus of the 
glosso-pharyngeal nerve. The nucleus of the vagus is situated upon the floor 
of the fourth ventricle laterally to that of the hypoglossal nerve (Figs. 72, 73, 
74, 76), and here its dark pigmented glistening cells form the ala cinerea 
(Fig. 78). 

The accumulation of cells just described does not, however, form the only 
nuclei of the pneumogastric. The fibers of the vagus root, like the fibers of 
the trigeminal with their descending branches, the so-colled ascending root, 
unite with cells which, like the ascending nucleus of the trigeminal nerve, 
may be followed down into the cervical cord, although less far, and commu- 
nicate with the cell group fibers of the ascending root of the glosso-pharyngeal 
nerve. These cells, like those of the trigeminal nerve and parallel with them, 



82 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



lie embedded in a gelatinous substance with a somewhat more dorsal and more 
medial inclination and penetrate close to the nuclei of the posterior columns. 
This ascending glosso-pharyngeal-pneumogastric root also bears the name 
tractus solitarius (Figs. 73, 74, 92, 93, 94). 

The auditory nerve consists of two nerve roots which pass to the cochlea 
and to the vestibule of the ear. The first branch only appears to be connected 




Fig. 77. — Bask of the Brain and Cranial Nerves. 



with the sense of hearing, while the second is probably the chief nerve of tonus 
and coordination. One, the cochlear nerve, springs from the cells of the 
spiral ganglion of the cochlea. Like the spinal ganglion cells, the cells of 
this ganglion also send out a process which soon divides into two parts. The 
short peripheral end runs to the auditory cells of the cochlea, the other, the 



NEURON SYSTEMS AND NEURON DISEASES 83 

ventral, forms a part of the root of the auditory nerve. The vestibular nerve 
originates from the ganglia of the labyrinth and from others which lie in its 
central course; both its processes are divided like those of the cochlear. The 
peripheral process extends to the cells of the ampulla, and the central forms 
the other trunk of the auditory nerve. Thus the combined roots of the eighth 
nerve pass laterally to the central organ alongside of the facial at the posterior 
border of the pons between the external border of the olives and the restiform 
body (Fig. 78). 

The divisions of the auditory nerve do not unite in the interior of the 
medulla oblongata. On the contrary, the cochlear nerve, whose root fibers 



Corpor. quadrigem. k 

V 

Corp. geniculat. mediale \/j^^ 



> Locus crrruleus 



"F^wftti" fm 'i kA* -» £■& -* Collieulus facialis 

Brachium pontis v^- ^^MJ^IudfmW 1 ^tffl^^MLiSlI^^- *"^ ^tria? acustica? 

Corpus restifortae *>i s ~j(f£--^.l' JTj^HLJ Wr\ -XS^ 

*> 1 V^ Jl \Mjz%M- - - ' -^-l a cinerea 

"" — fcV-i^iyi *W ~~~ — Calamus scrip torius 



Funicul. gracilis . [± 

Fig. 78. — Medulla Oblongata avith the Fourth Ventricle and the Corpora Quadri- 
gemina, Seen from Above. (After Toldt.) 

branch posteriorly and outwardly from those of the vestibular nerve soon after 
its entrance into the posterior border of the pons, penetrates the ventral acoustic 
ganglion (Figs. 78, 79. 94. 95) which upon transverse section is found to be 
situated in the most lateral region of the pons ventrally and somewhat out- 
wardly from a section through the restiform body, in an area which may be 
recognized from the flocculus of the cerebellum by a small protuberance; a 
few fibers of the cochlear nerve pass for a short distance toward the cerebellum 
and then enter a second small nucleus situated there, the tuberculum acousti- 
cian (Edinger). The vestibular nerve (Edinger, Freud) passes to a second 
acoustic nucleus, the dorsal (Figs. 94. 95). "While the ventral nucleus is 
external to the other sensory nuclei of the medulla, the dorsal is within the 
area of the latter structure (Figs. 64, 85, 86). It is situated directly upon 
the floor of the fourth ventricle in its most lateral portion. It runs forward 
laterally from the anterior division of the nuclei of the glossopharyngeal and 
pneumogastric nerves to the anterior third of the fourth ventricle, so that 
nearer the front it lies outside of the nucleus of the abducens and the genu 



84 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



of the facial. The strice acousticce (see below) are still within its area. The 
ascending trigeminal root is invariably situated below them ventrally, while 
the large nucleus has a more frontal situation. 

The first neurons of the organs of sight and smell are still to be described. 

The optic nerve which originates from the ganglion cells of the retina is 
divided into two branches of which the peripheral goes to the rods and cones 




while the central passes toward the brain, and after forming the well-known 
chiasm at the base of the brain (in front of the tuber cinereum) encircles 
the cerebral peduncles as the optic tract. 

The posterior portion of the optic tract disappears under the gyrus hippo- 
campi and the uncus of the temporal lobes which must be lifted if we would 



Capsula interna 
Cauda nuclei caudati - 
Globus pallidas 

Corpus j kterale 
geniculatum | mediale 

Pulvinar 

Sulcus lateralis mesencephali 

Brachium quadrige-. 
minum inferius 

Trigonum lemnisci 
Brachium 
conjunctivum 
Velum medullare 
an ter ius (Tegmen 
ventriculi quarti) 
Ventriculus quartus - 

Nn. glosso- 
pharyngeus et vagus 
Clava 
Tuberculura cuneatum 

Tuberculum cinereum ^ \\ 
Funiculus gracilis 
Funiculus cuneatus ' 



Fig. 80. — Region of the Corpora Q 

Right 




Commissura anterior 
^Caput nuclei caudati 
. Cornu anterius 
ventriculi lateralis 

Putamen. 



Tractus opticus 
-N. opticus 

Tractus olfactoriua 



Chiasma opticum 
x Corpus mamillare 
\ N Pedunculus cerebri 
^ \ . s Pons (Varoli) 

\ N Fila lateralia pontis 

N Fasciculus obliquus pontis 
Brachium pontis 
v Flocculus 

Oliva 

\ \ Fibrae arcuata externa? 
x v N ^ Bundles of the posterior root of the 

\ nrsf cervical or suboccipital nerve 
\ Sulcus lateralis posterior 

N Funiculus lateralis 

uadrigemixa, Thalamus, and Medulla, from the 
Side. (After Toklt.) 



Commissura habenularum 



Trigonum habenultp 
Corpus pineale 



Colliculus superior 

Brachium quadrigemi- 
num superius 

Brachium quadrige-v 
minum inferius 

Pedunculus cerebri ^ 



Colliculus inferior 

Frenulumveli medull. 
anter. 

Velum medullare anterius 
Nodulus vermis - 

Velum medullare — 
posterius 
Pedunculus flocculi 

Recessus lateralis 
ventriculi quart 
Apertura lateralis ventriculi 
quarti 
Plexus chorioideus ventriculi 
quarti 
Apertura mediana ventriculi 
quarti 
Fig. 81. — Corpora Quadrigemina, Thalamus 




., Thalamus 
lamia chorioidea 
Stria terminalia 

' Cauda corporis striati 
• Pulvinar 



Corpus geniculatum 
mediale 



Corpus geniculatum 
laterale 



- Corpora quadrigemina 

N. trochlearis 

Brachium conjunctivum 
— Brachium pontis 
Corpus restiforme 

Flocculus 
Ventriculus quartus 



- Tela chorioidea ventriculi 
quarti 
Medulla oblongata 



and Medulla, from Above. (After Toldt.) 
85 



86 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

follow the tract farther (Fig. 43). We then perceive that after the optic 
tract has passed around the cerebral peduncles (Fig. 80) it sinks dorsally 
into the roof of the middle brain, the characteristic portions of which are 
formed by the corpora quadrigemina (Figs. 81, 82). Just before it enters 
the middle brain it shows upon its lateral aspect a small protuberance, the 
corpus geniculatum laterale. A few of the fibers enter the nerve-cells, but 
the majority pass on in two directions. Some run through the anterior supe- 
rior peduncle which leads from the superior peduncle to the internal capsule, 
and thus enter the nerve-cells of the superior peduncle itself. The other enters 
the pulvinar and the stratum zonale of the thalamus opticus. 

The olfactory nerve receives its neuron cells in the cells of the olfactory 
mucous membrane. Its processes pass as fibers of the nerve of smell through 
the lamina cribrosa at the base of the brain, and there enter the bulbus olfac- 
torius (Fig. 77) . Here the first division of the tract of smell terminates in cells 
which are situated in the gray substance in the interior of the bulbus. Much 
of the interest and importance of the neuron theory centers about the nature 
of the termination, namely, the terminal filaments which enter into the bulbus, 
the primary neurons of smell combined with the dendrites of the cells of the 
second division (see below) in the bulbus. This communication takes place 
in the so-called glomeruli, which form a layer in the bulbus immediately below 
its external white medullary covering, and which is dimly perceptible macro- 
scopically (Fig. 83). I shall discuss later the complicated structure of the 
tract of smell. 

(2) THE SECOND SENSORY NEURONS 

The peripheral neuron is always designated as the first. In describing the 
second sensory neuron I shall delineate the course of those nerve processes 
of the cells which we have learned to recognize as the terminal nuclei of the 
first sensory neuron, and around which they split up. 

Before describing the path of the sensory fibers, I must call attention to 
one point. In the description of the first sensory neurons I stated that many 
collaterals are constantly given off by the fibers. In the motor tract we recog- 
nize the same tendency to unite with the branches of other functionating 
neurons. In the sensory tract, however, especially in the first sensory neuron, 
the conditions are complicated by a multiplicity of collaterals and communi- 
cations. 

It will be remembered that, while giving off lateral branches (collaterals), 
some of the posterior roots of the spinal cord pass through the gray posterior 
horns, others through the white posterior columns, in a manner which has 
been described. 

The fibers which pass into the posterior columns send collaterals, or even 
terminal distributions, into the motor anterior horn cells. These are the 
reflex tracts (reflex collaterals) (Figs. 68, 69, 84, 85), which unite the first 
sensory with the first motor neuron (Fig. 88) and which, therefore, have 
no central sensory continuation. Other posterior root fibers and collaterals 
of the gray substance communicate with cells of Clarke's columns (columna 
vesicalis) ; this is particularly marked in the dorsal cord, and takes place at 
the base of the posterior horn just where the posterior gray commissure arises. 






NEURON SYSTEMS AND NEURON DISEASES 



87 



A second sensory neuron does not develop from the cells which here surround 
it. On the contrary (Figs. 85, 86), the fibers of these cells pass upward in 
the spinal cord as a neuron of the cerebellum, they enter the lateral column, 
and emerge at its external periphery as the cerebellar lateral column tract 
(Figs. 85, 86). Finally, some posterior root fibers from the posterior horn 
terminate in cells in but a few of which short tracts develop inside of the 
spinal cord (Figs. 38, 84) (column cells, association tracts). 



Bulbus ocul 
sinister 



(Guddeni) 
Tractus opticus 
dexter 



Thalamus 



Radiatio occipi- c- 
to-thalaniica 
(Gratioleti) 



Lobus occipitali 




Uncrossed bundles of the optic t 



Crossed bundles uf the optic tract 



Radix medialis 



Radix lateralis 



Nucl. corporis 

geniculati 

lateralis 



~^ Colliculus 



"" Corpora quadrigemina 



'" Cornu posterius ventriculi lateralis 
'^ Tapetum 

~~ Cuneus 



Fig. 82. — Tract of the Optic Nerve (First Nextrox Red). (After Toldt.) 



88 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



The ramifications of the sensory cranial nerve roots are similar, in so far 
as they have been investigated. 

The first sensory neuron, with manifold combinations, finally communi- 
cates with the actual second sensory neuron. It arises from those nuclei which 

have already been described as 
terminal nuclei of the first sen- 
sory tract, and the course of these 
fibers we will now follow. 

The cells from which the sec- 
ond sensory neurons originate are 
found in large numbers in the pos- 
terior horns of the spinal cord. 
Their processes pass transversely 
through the gray substance, first 
anteriorly into the anterior com- 
missure, and subsequently upon 
the other side (Figs. 84-86) they 
enter the white substance of the 
lateral columns, and pass through 
that structure which we desig- 
nate the lateral column basic bun- 
dle. Thence they pass upward 
into the medulla oblongata. In 
consequence of the alteration in 
the parts due to pyramidal de- 
cussation they make a bend toward the middle, and pass between the lower 
olives which lie at either side of the median line (Fig. 86). 




Fig. 83. — The Bulbus Olfactorius of the Mouse. 
(After Obersteiner.) 




Fig. 84. — Diagram of the Origin and Termination of the Nerve Roots in the Gray 
Substance of the Spinal Cord and the Distribution of the Nerve-cells of the 
Latter. (After Bechterew.) 



NEURON SYSTEMS AND NEURON DISEASES 



89 



Here they receive the name lemniscus (or laqueus, formerly olivary inter- 
mediary layer) and pass on to the frontal region. 

Before following the path of the lemniscus we must return to those pos- 
terior root fibers which ascend in the posterior columns of the spinal cord. 
As has been mentioned, they extend to the medulla oblongata where they 
terminate in numerous cells which arise 
at the height of the pyramidal decussa- 
tion, the nuclei of Burdach's columns 
and of GolPs columns (Figs. 53, 87, 91, 
92). These nuclei may be traced as far 
as the beginning of the fourth ventricle. 
After this the fibers in the posterior col- 
umn gradually become more sparse, and 
finally disappear completely. 

From the cells of these nuclei nerve 
fibers originate which form the sensory 
neurons of the second division (Figs. 
85, 87). They pass to those fibers which 
extend from the lateral column of the 
spinal cord to the median line at the so- 
called lemniscus (olivary intermediary 
layer). In order to reach these fibers those 
from the dorsal side of the medulla must 
reach a zone situated more ventrallv and 
medially, and thus they pass in an arc 
ventrallv and inwardly from the nuclei 
of the posterior columns. At the same 
time they all cross the median line in the 
" raphe." unite with the lemniscus upon 
the opposite side, and with this they as- 
cend. Until these fibers of the nuclei of 
the posterior column enter the lemniscus 
they are called fibrce arcuata 7 internee 
(Figs. 85, 91, 92). Thus we find a second 
sensory crossing above the pyramidal de- 
cussation. All sensory fibers of the second 
neurons, like the motor, find their way to 
the brain by crossing over. Those which 
pass from the lateral columns of the spinal 
cord into the lemniscus without cross- 
ing have passed through the anterior 

commissure of the spinal cord from the posterior horn of the other side, while 
the fibra? arcuatae interna? cause the crossing of the sensory tract from the pos- 
terior columns which until then had not been made. Hence the second sensory 
neuron of the posterior roots of the spinal cord ascends to the medulla oblongata 
in the lemniscus on both sides of the raphe. We must trace this through the 
pons and the middle brain. 

The fibers of the second sensory neuron of the cranial nerves now unite, 
and, the lowest, the nucleus of the pneumogastric nerve (Fig. 87) whose cells 




Fig. 85. — Diagram of the Course of the 
Sensory Tracts from the Posterior 
Roots to the Medulla. (After Edin- 
ger.) 



90 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



form the second neuron, sends its fibers through the raphe to the most dorsal 
region of the crossed lemniscus. The same is true of the nucleus of the glosso- 
pharyngeal nerve. (Probably the ascending roots of the glosso-pharyngeal 
and vagus send crossed fibers into the lemniscus tract; no positive knowledge 
concerning this is at hand.) 

The relations of the auditory nerve are more complicated. These arise 
at the height of the pons in the median portion of which, above the pyramidal 



Radix anterior 

Central motor path of conduction 
Motor nerve cell 



Sensory collaterals (reflex collaterals) 

Peripheral motor nerve fiber 



Peripheral motor 
nerve terminal 



Peripheral sensory nerve 
fiber 



Peripheral sen 

sory nerve 

terminal 




^JRadix posterior 



Ascending and de- 
scending branch of 
the sensory root 
fibers 



Sensory collaterals 



5* Tract cell and tract 
fiber 



Fig. 86. — Diagram of the Motor and Sensory Conduction Tracts (Reflex Arcs). 

(After Toldt.) 

tract, the lemniscus tract passes upward. The nuclei of the acoustic nerve 
have been described (Figs. 88, 89, 94, 95). The ventral nucleus, situated 
ventrally and laterally near the entrance of the nerve, contains some of the 
second neuron cells. These fibers pass horizontally and medially through the 
pons between the pyramidal and lemniscus tracts, and cross to the other 
side. This tract is called the corpus trapezoideum (Figs. 88, 89, 95). 
Large numbers of nerve-cells are here deposited. Directly above them dor- 
sally and immediately below the nucleus of the facial nerve is a small col- 
lection of ganglia, the upper olive, into which large numbers of these fibers 
penetrate. 

Many fibers of the corpus trapezoideum appear to split up around their 
own cells as well as around the cells of the upper olive, upon the same side 
as well as after crossing to the opposite side. 

From the nuclei of the corpus trapezoideum and the upper olive other 



NEURON SYSTEMS AND NEURON DISEASES 



91 



fibers pass to the nerve of hearing where they mix with the remaining fibers 
of the corpus trapezoideum in so far as these have not united with cells, and 
subsequently as the lateral lemniscus (see below) they pass to the middle 
brain (Figs. 87, 96). 



Lobus parietal 



Corpora ( Colliculus superior \ x 

quadrigemina \ Colliculus inferior V| 



Thalamus 



Corpus geniculatum mediale 



Central tract of the N 
Lobus acusticus \ 

temporalis , 



w§H^/' "^ Capsula 
interna 




Exit of 
the 
Lem- -=, 
niscus 

lateralis 




pal! id us 



Putamen 



Lemniscus lateralis 

(acusticus) / 

Nucleus lemnisci 
lateralis 

Nucleus olivaris superior ' 
Corpus trapezoideum 



Exit of the lemniscus medialis 

Funiculus posterior "^ 



Fiber tract running to the 
Basis pedunculi 



Lemniscus medialis 
(sensitivus) 



Stria medullaris (acustica) 
~j Z.-~-' m Decussatio lemniscorum 

J 

' "~ Substantia gelatinosa 

""" Nucleus funiculi cuneati 
"* Nucleus funiculi gracilis 

~" Funiculus cuneatus 
"*■ Funiculus gracilis 



Fig. 87. — Diagram of the Lemniscus Tract (the Medial in Black, the Lateral in Red). 

The tuberculum acusticum of the nerve of hearing sends its nerve fibers 
dor sally around the corpus restiforme into the striae acousticce of the rhomboid 
fossa (Fig. 94). They pass through the middle of this, and then sink ven- 



92 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



trally in the raphe down to the level of the corpus trapezoideum and ascend 
in the lateral lemniscus together with fibers from the corpus trapezoideum 
and its nerve nuclei. 

The path of the fibers of the dorsal nucleus of the auditory nerve (vestib- 
ular nerve) is still unknown. 

As to the second neuron of the trigeminal nerve, i. e., the fibers of its 
elongated sensory nucleus which pass out centripetally, and which for a long 




Pig. 88. — Origin of the Facial Nerve and its Surroundings, the Fibers of the 
Auditory Nerve and its Nuclei. (After Edinger.) 

time could not be traced, the subject has been illumined by the investigations 
of Edinger, Wallenberg and Ramon. On the opposite side of the medulla 
oblongata they pass upward into a region which is designated as the substantia 
reticularis or as Bechterew's central tegmental tract (Figs. 89, 94-96) ; that 
is, such a tract has been located in animals. It extends dorsally and somewhat 
laterally from the median lemniscus up to the middle brain and through this 
to the thalamus. 

In the middle brain, therefore in the region of the corpora quadrigemina, 
the lemniscus appears as the continuation of the second sensory neuron of the 
spinal cord, the glosso-pharyngeus-vagus, the auditory, and probably also of 
the trigeminal nerve. Here it extends dorsally from the substantia nigra, but 
ventrally below the decussation of the peduncles, to the cerebellum, and fron- 
tally below the red nucleus (Fig. 99), the origin of these peduncles. The 



NEURON SYSTEMS AND NEURON DISEASES 



93 



lateral lemniscus disappears in the region of the anterior corpora quadri- 
gemina. 

I must now state the difference between the medial and lateral fibers of 
the lemniscus. The medial (upper) lemniscus contains the continuations of 




Fig. 89. — Section in the Region of the Origin of the Abducens. The Vestibular Nerve 

(After Edinger.) 

the fibra? arcuatae interna 3 , therefore rises from the nuclei of the posterior 
columns (Fig. 87). To these are added the fibers from the lateral column 
basic bundles of the spinal cord. In the tegmentum it passes below the 
corpora quadrigemina to the inter-brain, in the thalamus, where its ventral 



Fissura mediana posterior 
Funiculus gracilis 
Funiculus cuneatus 



Substantia gelatinosa 
(Rolandi) 
Fasciculus cerebrospinal]"? 
(pyramidalis) lateralis 
Canalis centralis 



Vena spinalis interna 

Columna anterior 



Basi- 




Cervix 
Caput 



/ Columnar 
( posterioris 



— ■ Radix spinalis n. accessorii 



Processus reticularis 



Decussatio pyramidum 
Fissura mediana anterior 



Fig. 90. — Transverse Section through the Pyramidal Decussation. (After Toldt.) 
8 



94 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



nucleus breaks up; this is, therefore, one of the ends of the second sensory 
neuron. 

The lateral (lower) lemniscus probably contains the fibers of all sensory 
nerve nuclei (glossopharyngeal, vagus, acousticus and trigeminal nerves) , 



Vena spinalis interna 



Fibrae arcuatae internae- 



Columna anterior 
Fissura mediana anterior 




Nucleus funiculi gracilis 

Nucleus funiculi cuneati 
^. Tractus spinalis n. trigemini 
Tuberculum cinereum 



Formatio reticularis 
Pyramis "" ^SgaagS/ ^5^ Lemniscus (medialis) 

Fig. 91.— Transverse Section through the Medulla Below the Olives. (After Toldt.) 



Calamus scriptorius 
Tractus solitarius 



Corpus restif orme — ~ 

Tractus spinalis n. — , 
trigemini 

Nucleus n. hypoglossi-""' 

Nuclei laterales* 2 ^ - 

Nucleus olivaris inferior "" 



Nucl. olivaris accessorius 
medialis 
Decussatio lemniscorum 




Nucleus funiculi gracilis 
r Nucleus funiculi cuneati 

Fibrae arcuatae externae 
(posteriores) 

Substantia gelatinosa 

^Fibrae arcuatae internae 

Raphe 

— Fibrae arcuatae externae 

( anteriores) 
Pyramis 



Nucleus arcuatus 

Fig. 92. — Transverse Section through the Lower Portion of the Olives and the 
Fourth Ventricle. (After Toldt.) 



Nucleus alae cinerae 
Tractus solitarius ^ 
Corpus restif orme 



Tractus et Radix spinalis 

n. trigemini 
Nucleus n. hypoglossi 

Nucleus ambiguus 

Nucl. olivaris j dorsalis - 
accessorius } med ialis ~- 

Nucleus olivaris inferior 

Decussatio lemniscorum 




___ — — Tela chorioidea ventriculi quarti 

Fossa rhomboidea 
(Ependyma) 
Fila radicularia n. vagi 



— Substantia reticularis 
grisea 

— Substantia reticularis 
alba 

• Fila radicularia n. 

hypoglossi 
Raphe 



Hilus nuclei olivaris 



Pyramis 
Stratum interolivare lemnisci 



Fig. 93. — Transverse Section through the Middle of the Oltve and the Lower Por- 
tion of the Fourth Ventricle. (After Toldt.) 



from the medulla oblongata. It forms the external side of the tegmentum, 
and in the area of the corpora quadrigemina it invariably passes in a dorsal 



NEURON SYSTEMS AND NEURON DISEASES 



95 



direction until it terminates at various places in the region of the anterior 
corpora quadrigemina. Its fibers enter partially into both corpora quadri- 
gemina, in part they are arrested beforehand in the ganglia which are simul- 
taneously deposited within it (lateral and upper lemniscus nucleus). A few 



Eminentia medialis 
Ependyma fossae rhomboideae N 

Corpus restiforme 

Radix vestibularis 

(medial root of the 

N. acusticus) 
Tractus solitarius - 

Radix n. glos 
sopharyngei 
Radix spinalis n. ci 

trigemini 
Fibrae cerebelloolivares 
Substantia reticularis grisea 

Nucleus olivares inferior 

Pyramidal nucleus " 
Pyramis 



Sulcus longitudinalis fossae rhomboideae 
t Sulcus limitans 

Area acustica 

Radix cochlearis (late- 
rale) Root of the N. 
acusticus 
Striae medullares 
(acusticee) 

•» Nucleus dorsalis n. 
acustici (Nucleus 
n. vestebuli) 
■»- Nucleus ventralis 
n. acustici (Nu- 
cleus n. cochleae) 

Nucleus n. glosso- 
pharyngei 
Nucleus eminentiae medialis 
Fasciculus longitudinalis medial 

Lemniscus (medialis) 
Fibrae arcuata? external (anteriores) 
^ Nucleus arcuatus 




Fissura mediana anterior 
Fig. 94. — Transverse Section through the Middle of the Fourth Ventricle. 

(After Toldt.) 



Colliculus facialis 



Fasciculus longitudinalis medialis 



Nucleus n. abducent is 
Nucleus n. vesticuli laterales 
(Deiter's nucleus) 



Fiber tracts from 
the Nucleus n. 
vestibuli to the 
cerebellum 

Corpus restiforme 



Radix vestibular. 
(medial root of 
the N. acusticus) 

Substantia reticu- 
laris grisea 



Lemniscus (mediales) 



Fibrae pontis superficiales 



Pars basilaris pontis 



Genu (internum) n. facialis 
Fovea superior 
/ Radix n. facial 

(Pars secunda) 




Radix n. facialis 

(Pars prima) 
Nucleus sensibilis 
n. trigemini 
(Substantia 
gelatinosa) 
"~ Radix spinalis n. 
trigemini 
Nucleus n. 
facialis 



Fila radicularian. 
abducentis 
""" Nucleus olivaris 

superior 
Corpus trapezoideum 



Raphe pontis 



Fasciculi longitudinalis 
(pyramidales) pontis 



Sulcus basilaris 



Fig. 95. — Transverse Section through the Lower Border of the Pons Immediately 
Above the Olives and through the Middle of the Fourth Ventricle. (After Toldt.) 



96 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



fibers (fibers of the acoustic) terminate in the medial geniculate body. The 
course of the lemnisci may well be followed in the illustrations from 90 to 99, 
which clearly portray the general position of the tracts in the trunk of the 
brain. 



Decussatio nn. trochlearium 
Ventri cuius quartus (upper portion) 

Lemniscus lateralis 
Brachium conjunctivum 

Substantia reticularis grisea 
Lemniscus medialis 



Bundle passing - — - 
from the fillet 
into the crusta 
of the cerebral 
peduncle 

Raphe pontis 



Velum medulare anterius 
Radix n. trochlearis] 



Nuclei pontis 




Radix descendens (mesencephalica) 
n. trigemini 

Substantia ferruginea 

Fasciculus longitudinalis 

Pars dorsalis frontis 



Fibrae pontis 
profunda? 



Fasciculi longi- 
tudinalis (pyra- 
midalis) 



Pars basilaris pontis 
Fibrce pontis superficiales 



Fig. 96. — Transverse Section through the Middle of the Pons and the Anterior Med- 
ullary Velum. (After Toldt.) 

We must now study the continuation of the optic and olfactory nerves 
after leaving their nuclei. For a reason to be immediately mentioned, I 

shall omit the secondary optic tract, and 
shall pass at once to the olfactory. This 
rises from the cells of the bulbus olfac- 
torius which have already been described 
as their "glomeruli." From this point the 
fibers run but a short distance only. They 
gradually pass into the gray substance of 
the optic tract which immediately joins the 
bulbus posteriorly and terminate in its cells. 
Another part appears to run on posteriorly 
to the point where the tractus olfactorius 
arises from two roots, one a distinctly vis- 
ible white lateral root, and a medial one 
which is grayish and can be recognized only 
with difficulty. This latter enters the cells of the gray substance in this 
region which is designated as the substantia perforata anterior (Fig. 77). 




Fig. 96-4. — Section through the 
Pons. 



NEURON SYSTEMS AND NEURON DISEASES 



97 



The fibers of the lateral root pass on posteriorly into the temporal lobes. Later 
we will again follow them. 

In regard to the secondary optic tract, we must assume that the external 
geniculate body, the anterior corpora quadrigemina, and the pulvinar of the 
thalamus neurons of the second division develop from the three terminal stages 
of the optic tract, like those in the lemniscus. 

The fibrillations of the first terminal stages of this nerve, so far as we 
know at present, pass directly to the cortex of the brain without ending in 
nuclei extending toward the cauda like the fibers of the lemniscus. There- 
fore this higher optic tract does not correspond to the second, included within 
the temporal tract, but to that third sensory neuron which is now to be de- 
scribed. 

(3) THE THIRD SEXSOEY XEUROXS 

The complicated mechanism of the sensory conduction tract compared 
with the motor, which is evident from this description, is explained by the 
fact that in man as well as in all vertebrate animals there is a third sensory 
neuron in addition to the two neurons we have discussed. The marked devel- 



Stratum zonale 



Lemniscus lateral 

Radix descendena 
(mesenoephalica) 

n. trigemini 



Nucleus lemnisci 
lateralis 




Nucleus colliculi inferiores 
»« Stratum griseum centrale 



Aquaeductus cerebri 

(Sylvii) 



Sulcus lateralis 
mesencephali 



Lemniscus—-""] 
medialis 



Lemniscus tract to 
the basis pedunculii 



Nucleus n. troch- 
learis 



Fasciculus lon- 
gitudinals 
medialis 



Decussatio 
brachii con- 
junctiva 



"•* Recessus pos- 
terior fossae 
interpeduncu- 
laris (Tarini) 



Fibrse pontis su- 
perficiales 



Fig. 97. — Transverse Section through the Upper Margin of the Pons and the Poste- 
rior Corpora Quadrigemina. (After Toldt.) 

opment of the anterior brain (cerebrum) in the primates, the higher stage of 
conscious sensation, are probably intimately related to this. It is scarcely 
necessary to state that collaterals in an unentwined web pass to other centers 
of other neuron groups with unknown functions, especially in the cortex of the 
brain itself, and are combined in various ways with others in the sensory tract. 



98 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



The third sensory neuron is combined with the second in the cortex of 
the brain. The fibers of its origin must be sought in those nuclei in which 
the lemniscus ends (Fig. 97) and also at the terminal ends of the primary 
optic and secondary olfactory fibers which have been described. In the 
optic nerve the second neurons must be considered the equivalent in func- 
tion of the third neurons of the remaining sensory tracts (Fig. 82). The 



Stratum album profundum 
Aquaeductus cerebri (Sylvii) 

Stratum griseum centrale 



Radix descendens 

(mesencephalica) n 

trigemini 



Stratum zonale 

Stratum griseum colliculi 



Brachium quadrigemi- 
num inferius 



Sulcus lateralis 
mesencephali 



Fasciculus lon- 
gitudinalis 
medialis 

Brachium 
conjunctivum 



Substantia nigra 




Nucleus n. 
oculomotorii 



Lemniscus 
medialis 



Basis pedunculi 



Tegmentum 



Decussationes teg- 
mentorum 

Fossa interpeduncularis 
(Tarini) 



Fig. 98. — Transverse Section through the Upper Corpora Quadrigemina, the Teg- 
mentum, and the Crus Cerebri. (After Toldt.) 

lemniscus is supposed to carry all of the second sensory neurons from the 
spinal cord and the medulla oblongata up to the nuclei of the nerves of the 
mid-brain and to the thalamus. The second sensory neuron of the trigeminal 
nerve, certainly in the rabbit, is the only one that leads somewhat dorsally 
and laterally up from the median lemniscus, chiefly through the thalamus; 
in man this is still uncertain. 

The relation of the lemniscus to the cortex of the brain must now be 
outlined. This is brought about by the corona radiata fibrillation which has 
already been referred to. It extends as a large projection tract into the medul- 
lary mass of the hemisphere which we designate as the centrum semiovale. 
The motor cortical tract also runs through the corona radiata bundles. 

The path that the sensory tract pursues from the terminal nuclei in the 
middle brain and in the inter-brain to the cortex is, therefore, a devious one. 
Its extent over the cortex is much greater than that of the motor tract. Hence 
we find united here the fibers of the lemniscus which come from the nuclei 
of the mid-brain (Figs. 98 and 99) (medial lemniscus nucleus and nucleus 
of the corpora quadrigemina, geniculate body). They pass through the so- 
called regio-suhthalmica in the tegmentum under the thalamus (Fig. 63), 
obliquely outward and upward into the internal capsule, and there terminate 
(Fig. 60) close behind the motor tract. 

Fibers from the nucleus of the optic, from the geniculate body, from the 



NEURON SYSTEMS AND NEURON DISEASES 



99 



anterior corpora quadrigemina and the thalamus are added to this tract 
originally coming from the nuclei of the medulla oblongata. The optic tract 
and the auditory tract are in the posterior part of the internal capsule (see 
above) behind the remaining sensory bundles. The central olfactory tract 
only has a different course. Urontally. however, the sensory fibers diverge in 
various directions from the internal capsule of the cortex of the brain. The 
majority of the fibers pass longitudinally upward into the corona radiata 
fibrillation of the parietal lobes, and this is designated as tegmental radiation. 
It was formerly believed that the sensory fibers in this cortical region of the 
tegmentum (tegmentum. Figs. 98, 99) of the cerebral peduncles passed unin- 
terruptedly into the medulla. We now know that they traverse the tegmentum 
for some distance but not unbroken!}-, for their course is interrupted by the 



Tela chorioidea ventriculi tert 
Vena cerebri interna j 
Radix descendens (mesencephalica) 
n. trigemini s 



Nucleus corpori 
geniculate med 



Corpus pineale 
.( Acervulus 



/ Aqusductus cerebri (Sylvii) 
/ Colliculus superior 



Tract from the commissura 
posterior 

Brachium quadrigemium 
inferius 

/ Lemniscus medialis 




Tractus opticus 



Basis pedunculi 

Substantia nigra 

Nucleus ruber (tegmenti) 



v Nucleus n. oculo- 
motorii 
Fasciculus longitudi- 
^ nalis medialis 

Fila radicularia n. oculomotorii 



H. oculomotorius 
Substantia perforata posterior 

Fig. 99. — Transverse Section through the Upper Corpora Quadrigemina in its Most 
Anterior Portion. (After Toldt.) 



ganglia of the middle brain and inter-brain. These fibers, which pass into 
the cortex of the parietal lobe, probably extend to the same region where the 
motor centers are also found. According to Ramon they there encircle the 
small pyramidal cells of the cortex. Doubtless these combinations form 
other cortical territories which are as yet unknown. 

The fibers of the optic nerve follow another path. Like the optic radia- 



100 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

tion, they run backward from the posterior capsule into the occipital lobes 
(Fig. 82). All of the auditory filers appear to reach the temporal lobes and 
to distribute themselves through its cortex (Fig. 79). 

The termination of the olfactory radiation still remains to be described 
but we will not discuss its peculiarities. Although the olfactory tract in 
man is comparatively atrophic, it nevertheless retains the complicated struc- 
ture of the fiber of those animals which have been designated macrosmatic, 
and in which the sense of smell is distributed extensively through a number 
of lobes. The olfactory tract becomes complicated by centrifugal fibers which 
the central neurons of other tracts of special sense (the auditory and, prob- 
ably, also the optic), and perhaps still other sensory cranial nerve tracts, appear 
to possess. I shall make no attempt to describe these tracts as Edinger and 
Bechterew have portrayed them in detail. 

As to the centripetal fibers, we must assume a complicated direction of 
the higher neurons of the olfactory tract; this has been lately made the sub- 
ject of studies which are not yet complete. It has been stated that a sec- 
ondary olfactory tract is known to extend from the bulbus to the tractus olfac- 
torius and the substantia perforata. From this point a tertiary tract arises. 
It passes in a double path to the cortex of the brain, particularly to that part 
which we designate the hippocampus convolution; first, through the lateral 
root of the olfactory tract, secondly, from the substantia perforata anterior 
through the anterior commissure to the hippocampus convolution of the oppo- 
site side. Now there are fibers in this lateral root which pass directly from 
the bulbus without touching the tract or its cells or other cells, chiefly as a 
secondary tract, to the hippocampus major of the same side. 

The other relations of the cortex of the hippocampus are very compli- 
cated, even that of the coordination fibers and association fibers with other 
parts of the brain. Without minutely discussing the subject I wish to men- 
tion that the fibers coming from the corpora mammillaria to the hippocampus 
major, particularly those connected with the psalterium, are especially impli- 
cated, that some of these fibers communicate with the thalamus and with the 
tegmentum of the middle brain; briefly, that the center for smell has been 
traced beyond the cortical tracts to the most distant regions of the brain, espe- 
cially in animals. 

D. DISEASES OF THE SENSORY NEURONS. TABES DORSALIS 
(LOCOMOTOR ATAXIA) 

So far as it is known we have seen how very complicated is the histologic 
structure of the sensory tract in comparison with the motor. Its functions 
also are more numerous and more complicated. There are several qualities 
of sensation. These must be conveyed through the same conduction tract, 
and on this point we are unable to decide whether, according to the nature 
of the stimulation, the same fiber is capable of developing various kinds of 
sensation, or whether in every sensory tract there are special fibers for differ- 
ent sensations. All sensory nerve tracts convey various sensations. This is 
not only true of sensory neurons, sensu strictiori, but also of the neurons for 
hearing, for sight, and for smell. In the auditory nerve we find the tract 
for equilibrium, in the optic there are tracts which permit the estimation 



NEURON SYSTEMS AND NEURON DISEASES 101 

of. distance, space, etc. There are fibers which act reflexly upon the nerves 
of accommodation and the pnpil (oculomotor, sympathetic). In the tract 
of smell, in many animals at least, there are numerous hut as yet unknown 
varieties of sensation. 

Thus diseases of the sensory tract produce greater disturbances than those 
of the motor. Tactile sense, the sensation of pain, of temperature, and coor- 
dination of movements, for example, are conveyed by the first sensory neurons 
of the spinal cord (v. Leyden), thence they are transmitted further by neu- 
rons of the sensory tract, and are brought to consciousness. How manifold 
must those symptoms of disturbance be which affect only the first sensory 
neuron ! The various sensations in disease are not even perceived in the 
same way; on the contrary, even with apparently similar histologic changes, 
we see that in one case this, in another a very different sensation, is expe- 
rienced. 

This sufficiently explains why we know so much less of neuron diseases 
of the sensory tract than of those of the motor tract. 

We have fairly accurate knowledge in only one disease, an affection of 
the first sensory neuron, and this is tabes dorsalis. According to present 
opinions (v. Leyden, Pierre Marie, Redlich, Eosin) it is a diffuse disease 
of the fibers of the first sensory neuron; i. e., of tliose tracts above all which 
enter into the posterior roots of the spinal cord, also of the roots of the 
sensory cranial nerves which, it is true, are as a rule less intensely implicated. 
All the fibers of this neuron are not diseased in tabes but invariably more 
or less of them, and we must trace their degeneration until they split and 
form the nucleus of the second neuron. The view that tabes is a disease of 
the posterior roots was maintained by v. Leyden at a time when the structure 
of the spinal cord was by no means so well understood as it is to-day, and 
when the neuron theory had not been formulated. 

Since tabes is, above all, a degeneration of the extramedullary and intra- 
medullary posterior root fibers of the spinal cord, we find in the severe forms 
of the disease, i. e., according to the number of posterior roots and their fibers 
which are diseased, degeneration in Burdach's columns following the entrance 
and course of the posterior root fibers, and in Golfs column following the 
fibers which penetrate more deeply. We also find a decrease in the fibers 
of the posterior horns, in the region of the reflex collaterals, and in the fibers 
of Clarke's columns following the course of the posterior roots. Optic atrophy 
is often added, also trigeminal disease (affection of the jaws), gastric crises, 
laryngeal crises, and impairment of the sense of taste, etc., all of which are 
to be attributed to diseases of the root of the vagus and of the glosso- 
ph a ryngeal nerves. 

Tabes is a primary degeneration of the nervous tissue without inflam- 
matory implication of the intermediary tissue and the vessels. In place of 
the degenerated fibers glia tissue with its cells and vascular adventitia appear. 
This leads to so-called sclerosis. Proliferation of vessels, hemorrhages, round 
cells, granular cells, etc., are entirely absent. In cases of pure tabes we find 
upon transverse section of the spinal cord merely degeneration in the poste- 
rior columns and the disappearance of fibers in the posterior roots (Fig. 100). 
With the naked eye we recognize the characteristic gray discoloration (Figs. 
101, 102, 103, 104) in the posterior column; in the stained preparations 



102 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



nerve fibers are absent in these areas and a dense glia tissue, the nuclei of 
which are not greatly increased, takes their place (Fig. 105). This glia 

tissue seems to lose 
its structure only in 
very old cases, when 
it appears to be trans- 
formed into true con- 
nective tissue. Glia 
staining by Weigert's 
method shows in these 
areas that the char- 
acter of the tissue is 
unchanged. 

It is difficult to 
diagnosticate disease 
of the cells of the pos- 
terior root fibers, for 
here we can consider 
only spinal ganglia 
cells, in the brain the 
ganglion of the vagus root, the trigeminal root (Gasserian ganglion), and 

the optic nerve-cells in the 
retina, etc. No disease of the 
spinal ganglion ceils has as 
yet been found in tabes. Nei- 
ther atrophy, nor destruction, 




Fig. 100. — Posterior Roots (a) and Anterior Roots (b) in Tabes. 






Fig. 101. — Tabetic Degeneration. 

cs, Upper cervical cord; c, posterior cervical cord; 

d, dorsal cord; I, lumbar cord. 



Fig. 102. — Tabetic Degeneration 
in the Lumbar Cord. 





Fig. 103. — Tabetic Disease of the Cervical Cord. 
(After Oppenheim.) 



Fig. 104. — Tabetic Disease of the 
Lumbar Cord. (After Oppenheim.) 




NEURON SYSTEMS AND NEURON DISEASES 103 

nor change in Mssl's granules has been positively determined ; of course, the 
basic substance has not been minutely investigated. There are authors who 
believe that tabes does not originate in a neuron but at the point where the 
posterior root enters from the pia (Obersteiner and Redlich). 

For the present we must withhold a positive opinion regarding these 
conditions; we have no methods with which to determine accurately whether 
a nerve-cell is histologically and functionally sound or not. The basic sub- 
stance, too, which Apathy and Bethe 
maintain may be divided into a fibril- 
lary structure, has never been sufficient- 
ly examined pathologically on account 
of a lack of approved methods. Accord- 
ing to the best authorities, the reten- 
tion of Nissl's granules does not abso- 
lutely prove the normal composition of 
the cells, as its destruction proves dis- 
ease. 

Tabes is the only pure neuron dis- 
ease of the sensory conduction tract 
with which we are as yet familiar; and 
no disease of the sensory neurons of the 

higher divisions is known. 

° . .-.,.. Fig. 10o. — Posterior Columns in Tabes, 

Histologically we are still m lgno- Greatly Magnified. 

ranee concerning the circumscribed and 

partial sensory diseases. Clinically, however, these are more or less familiar. 
The number of paralyses, especially of irritative conditions, of the sensory 
neurons is very great, and to these must be added the various forms of 
peripheral neuritis. But we have no accurate insight into the anatomical 
substratum. We believe the cells of the spinal ganglia to be the neuron cells 
of the peripheral sensory nerves, but we know nothing of their implication 
in the previously mentioned pathologic conditions. It is interesting to note 
that in cases of h erpes zoster changes have recently been observed in the spinal 
ganglion cells, but, as stated, the entire subject has been too little investigated 
for us to discuss it in this brief article. 

E. OTHER KNOWN TRACTS IN THE CENTRAL NERVOUS SYSTEM 
(1) IN" THE CEREBRUM AND PEDUNCLES OF THE BEAIN" 

The motor and sensory tracts are the ones with whose anatomical course 
and function we are most familiar, but they are not the only ones with 
which we are acquainted. Of some neuron systems we do not know the func- 
tion, of many others the exact cellular origin, nor has their termination as 
yet been sufficiently traced. These are tracts which belong in part to pro- 
jection systems, partly to coordination, and partly to association systems. 

I shall begin with the most important of these which are situated in the 
cerebrum. 

Here a number of fiber bundles unite the distinct and detached sections 
of the cerebrum, and then traverse extensive areas in the white substance 



104 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



of the hemispheres. The numberless short tracts which run from one con- 
volution to another or run within a convolution form a network of association 
fibers which cannot be disentangled. 

First we must mention those most important association bundles which 
unite areas of the same hemisphere with each other (Fig. 106). The fas- 



Lobus p: 


irietalis 

I 

\ \ 
\ \ 
\ \ 

\ J 


Fibrae arcuatse cerebri 
/S N Gyrus cinguli 




Cingulum 

Fasciculus longi- _^^\^ 
tudinalis superior *\^^^ \ 

Lobus f \ N x 
occipitalis ^^ >v N v 


1 /. / / // 

L 1 / j^ j / / / / / 

\ > — ¥^^ j y*J J / 


Fornix 

Lobus front. 

\ 1 



> 









m 



m 



I 



\ Fasciculus 
uncinatus 

Lobus temporalis 



Fasciculus longitudinalis inferior 
Fig. 106. — Association Bundle Projected upon the Median Surface of the Hemisphere. 

(After Toldt.) 

ciculus uncinatus runs from the lower external border of the frontal lobe 
to the anterior portion of the temporal lobe. The fasciculus longitudinalis 
inferior connects the temporal lobe with the occipital lobe. The fasciculus 
arcuatus extends from the posterior portion of the temporal lobe to the frontal 
lobe and also to the anterior portion of the parietal lobe. The fasciculus 
fronto-occipitalis runs from the frontal lobe to the occipital lobe. The cingu- 
lum runs above the corpus callosum in an arch from the cortex of the hippo- 
campus major of the temporal lobe, and reaches that area of the frontal lobe 
where the fasciculus uncinatus is inserted. 

Furthermore, we know there are important fiber masses which unite the 
areas on one side of the brain with those of the other side. They are called 
coordination fibers, and we assume that they serve the functions of both 
hemispheres for the purpose of coordination. The corpus callosum (Figs. 
107, 108) is the structure which unites the greatest number of these fibers. 
With the exception of a few longitudinal bundles it consists almost entirely 
of coordination fibers which emerge from one hemisphere and radiate into 
the other, closely uniting in the median line, and forming a compact 
mass. 



Yentriculus tertius Massa intermedia 



Tela chorioidea ventriculi tertii 
Corpus pineale 



Fissura transversa cerebri 
Aquseductus cerebri \ 

(Sylvii) \ 

Lamina quadrigemina 

Velum medullare 
anterius 

Vent, quart. 



Foramen interventriculare (Monroi) 
Columna forme's 

Septum pellucidum 

/ Lamini rostralis 




Commissura anterior 
Lamina terminalis 



v Chiasms opticum 
Infundibulum 

Tuber cinereum Hypophysis 

Corpus mamillare 
Corpus medull. cerebelli I » 

Medulla oblongata r ° ns CVaroU) 

Fig. 107. — Median Sagittal Section through the Brain. (After Toldt.) 



Lobus frontalis 



Radiatio corporis callosi ^— -j 



Corpus caliosum 




Nucleus lentiformis 



— Lobus parietalis 



Fissura cerebri 
lateralis (Sylvii) 



Lobus temporalis 



N. Substantia perforata 
anterior 



Commissura anterior . 

Pars anterior 

Fig. 108. — The Most Important Commissure Fibers. (After Toldt.) 

105 



106 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

If we trace the radiation of the corpus callosum into the hemispheres we 
see it distributed to various regions of the cortex; the anterior portion passes 
over the anterior division of the lateral horn to the frontal lobes; fibers of 
the corpus callosum which pass to the occipital lobe encircle the posterior 
horn from above; these are called the forceps major; others which do not 
run so far backward as laterally pass to the outer side of the posterior horn 
in the temporal lobes, and are known as the forceps minor. 

Some of the coordination fibers of the cerebrum do not pass through the 
corpus callosum but into the anterior commissure. We know from the macro- 
scopic anatomy of the brain that this passes from one side to the other quite 
anteriorly in the ventral portion of the third ventricle, in front of the crus 
of the fornix and under the septum pellucidum. It lies at the same time 
under the head of the corpus callosum. In the hemispheres the fibers radiate 
below the corpus striatum, quite close to the floor of the brain, and above the 
substantia perforata anterior, also posteriorly and downward as far as the 
posterior parts of the temporal lobes. Here they join the cortex of the hippo- 
campus major, and appear to form a coordination system for the sense of 
smell. A few fibers apparently enter the tractus olfactorius (olfactory lobe). 
There are a few other small commissure systems (commissura mollis and 
posterior, lyra Davidis) the importance of which I shall not discuss. 

Besides the association and projection fibers of the cortex of the cerebrum 
there are numerous projection fibers in the corona radiata, and these run 
caudally to the trunk ganglions (nucleus caudatus and corpus striatum), to 
the thalamus (inter-brain), directly downward to the middle brain (corpora 
quadrigemina), or to the medulla oblongata and the spinal cord. The second 
motor neuron and the third sensory neuron are known to be important parts 
of these projection fibers, but the origin and course of many of the fibers from 
this tract are still unknown and their function still obscure. 

I shall now leave the fiber systems of the cerebrum, and pass to the con- 
sideration of tracts in the caudal portions of the brain. 

The trunk ganglia (lenticular nucleus) and the thalamus are connected 
by a closed tract, the lower part of which is surrounded by the internal 
capsule. This is the ansa nuclei lentiformis. It forms the three links of the 
lenticular nucleus ; its function is still unknown to us. 

I must also call attention to the fasciculus longitudinalis posterior (Figs. 
94-98), a bundle of longitudinal fibers which may be traced caudally from 
the region of the corpora quadrigemina far down into the medulla. It is 
invariably situated in the sagittal plane of the lemniscus tract, extending dor- 
sally beyond it as a closed round bundle. During its course it constantly gives 
off fibers to the cranial nerve nuclei, and appears to form for these an asso- 
ciation center, made necessary by their complicated and numerous coordinate 
functions. 

In conclusion I must mention a net-like layer (fibers and nerve-cells 
combined) which may be followed from the most caudal portion of the 
medulla through the pons to the tegmentum of the cerebral peduncles. This 
tract, formerly designated the substantia reticularis (Figs. 91, 93-96), has 
been characterized b}^ Bechterew as the central tegmental tract. Its function 
is unknown. In the medulla and pons it usually lies laterally and above the 
lemniscus; it disappears in the region of the corpora quadrigemina. 



NEURON SYSTEMS AND NEURON DISEASES 107 

(2) IN THE CEREBELLUM 

We now come to the histology of the cerebellum, a neuron system of great 
extent and important in its unity. The course of its fibers has been only 
partially traced, and its connection with other portions of the brain has been 
more clearly revealed than its own complicated structure and the functions 
of its cells and columns. 

We know that there are centripetal and centrifugal cerebellar fibers; 
we do not accurately know the individual course of these, but the bilateral 
functions of some of them have been determined. 

Extending between the cerebellum and other portions of the brain we 
recognize the following principal fiber systems: (1) Columns from the 
spinal cord to the cerebellum (and vice versa): (2) From the nuclei of 
the medulla; (3) Pontal fibers of the cerebellum; (4) Tracts to the middle 
brain. 

We will first consider the tracts which unite with the spinal cord (Figs. 
109 and 110). The columna vesicalis of the spinal cord, the so-called column 
of Clarke, around which the fibers of the posterior roots split up (Figs. 67, 84), 
is already familiar to us; new fibers originate from these cells and pass into 
the lateral columns of the spinal cord, at the bolder of which they ascend 
without crossing (Fig. 85). Gradually forming a broader zone, they reach 
the medulla where they unite with the corpora restiformia and form the 
hemisphere of the cerebellum (Figs. so. Si. 85), and within its medullary 
mass upon either side of the corpus dentatum tiny pass to the upper vermis, 
and split up in its cortex while crossing in the median line. 

But we also distinguish a bundle descending from the cerebellum to the 
lateral columns of the spinal cord. These libers originate in Belter's nucleus 
(Fig. 109) of the cerebellum which is situated within the medulla of the 
hemispheres at the point where the corpora restiformia are inserted. 

Finally there is an ascending spinal cord cerebellar bundle, Gowers' bundle 
(Figs. 37, g, 38, 109, 110). This lies as a triangle or prism ventrally toward 
the cerebellar lateral column tract, joins the anterior column basic bundle, 
but lies also at the periphery of the spinal cord, passes through the medulla, 
upward through the dorsal and also the most lateral region of the pons, and 
in its most anterior division winds dorsally around the peduncles to the mid- 
brain, then passes through the velum anticum and into the cerebellum in the 
region of the superior vermis (Figs. 109, 110). 

These are the best known spinal cord ramifications of the cerebellum, and 
now we come to those of the medulla oblongata. Almost all of these pass 
through the corpus restiforme. The most important layer is the cerebellar 
olivary tract. This probably arises from the hemispheres of the cerebellum, 
the fibers passing arc-like through the posterior olives and the secondary olives 
of the same side to the nuclei of the olives and secondary olives of the oppo- 
site side (Figs. 109, 110). 

Some of the fibers which pass from the cerebellum through the corpus 
restiforme to the medulla reach the sensory nuclei, the glosso-pharyngeal- 
vagus, and the trigeminal. They originate in the middle portion of the cere- 
bellum, probably in the nucleus tegmenti. 

Another important fiber tract which originates in the cerebellum is that 



108 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



of the peduncles to the pons. They form that cerebellar fiber system in the 
pons which passes from unknown regions in the hemispheres of the cere- 
bellum to the cortex, first entering the ventral portions of the pons and encir- 



FCocu/t/^ 




Med. spi n 

Fig. 109. — Diagram Showing the Arms of the Cerebellar Tracts. (After Edinger.) 

cling the closed bundles of the pyramidal tract (Fig. 96). Many nerve-cells 
in which a portion of the fibers appears to end are here deposited, while, on 
the other hand, fibers of these cells ascend to the cerebellum, probably in the 
same tract. Some of these fibers, however, extend to the raphe of the pons, 



NEURON SYSTEMS AND NEURON DISEASES 



109 



which they partially cross, and then pass dorsally to the region of the teg- 
mentum where they may be traced toward the cerebrum. 

The communication of the cerebellum with both nuclei of the auditory 



Lobus parietalis 



Putamen v 



- Corona radiata 
Nucleus caudatus 

s Thalamus 

Capsula interna 



^~ Nucleus hypothalamicus (Corpus Luysi) 
— - Nucleus ruber 

Pedunculus thalami inferior 

Fibers of the Brachium conjunctivum 




Ansa peduncularis ' 



Lemniscus mediali 
(sensitivus) 



Brachium conjunctivum 

Raphe medulla? oblongatae - 
Nucleus olivaris inferior 



, Decussatio brachii conjunctivi 
)ra quadrigemina 
Brachium conjunctivum 
Vermis superior 

Nucleus dentatus 



Hemispaerium cerebelli 



Crossed f Fibrae cerebelloolivare:* 
(decussat- 
ing) tracts 



Fibrae arcuatae interna? X V 

/ 

Nucleus funiculi cuneati M 



Corpus restiforme (its entrance in- 
to the medullary center or white 
matter of the cerebellum) 



Nucleus funiculi gracili 



Fibrae arcuatae externa? 
'. (posteriores) 

Fibres of the posterior column 
passing direct to the cerebellum 
Fasciculus cerebellospinalis 



Direct 
(non-de- 
cussating) 
tracts 



Fig. 110. — Cerebellar Tracts. (After Toldt.) 



110 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



nerve deserves special mention. The origin of these fibers in the cerebellum 
is unknown. 

Another important communication is that of the cerebellum with the 
mid-brain by means of the anterior cerebellar peduncles (Figs. 97, 98) pass- 
ing to the corpora quadrigemina. Its fibers arise from the nucleus dentatus 
of the cerebellar hemispheres, traverse the tegmental region of the mid-brain, 
cross from the lemniscus layer dorsally and ventrally to the posterior corpora 
quadrigemina (Fig. 98), and terminate in the red nucleus (of the tegmentum) 
in front of the anterior corpora quadrigemina (Fig. 99). 

This forms a brief description of the many direct communications of the 
cerebellum with the middle brain and the spinal cord, and also with regions 
lying between these which contain very important nerve centers. The func- 
tion of these many communications is uncertain. Some serve for coordina- 
tion and assist in maintaining the equilibrium brought about by cerebellar 
combinations. 

As the portions of the brain which must still be considered are united in 
various ways (collaterals, posterior longitudinal fasciculus) the union with 
the cerebellum must be regarded as a second, perhaps more important, one. 

There appear to be no direct tracts from the cerebellum to the cerebrum, 
but the peduncles indirectly communicate with the pons and the corpora 
quadrigemina, just as tracts of the cerebrum enter into their terminations in 
the tegmentum. 

Our knowledge of the course of the neurons within the cerebellum is very 
meager; of many fibers we know neither the origin nor the path they pursue. 
But we may hope that the future will reveal this. 

I take this opportunity to mention a few peculiarities in the structure 
of the cerebellar cortex which differs markedly from the cortex of the cere- 
brum (Fig. 111). Here three layers may be dif- 
ferentiated; the external molecular layer which is 
deficient in cells and the internal granular layer are 
separated by a narrow band in which a large num- 
ber of peculiarly formed cells are arranged like a 
palisade. The cells in the middle layer are called 
Purkinje's or bottle cells. They are characterized by 
an extraordinarily large number of dendrites which 
are distributed chiefly in the molecular layer while 
the nerve process passes to the medullary mass of 
the cerebellum, in its passage often giving off nu- 
merous collaterals. In the molecular layer we find 
the terminal ends of the neurons whose origin is 
unknown, the fibers of which rise from the medul- 
lary layer and through the granular layer to the 
cortex. The scant cells of the granular layers them- 
selves, at least in great part, are connected with the dendrites of Purkinje's 
cells ; their nerve fibers and collaterals come in contact within the body of these 
cells. The granular layer, which was formerly not regarded as a nervous organ, 
is composed of numerous polygonal cells with a few short dendrites and a nerve 
process which extend upward into the molecular layer and there divide into two 
branches and become entwined. Besides these small cells of the granular layer 




Fig. 111. — Cortex of the 
Cerebellum. (After Ober- 
steiner.) 






Fig. 112. — Friedreich's Disease. (After P. Marie.) 
9 111 



112 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



there are a few large ones whose dendrites pass upward into the molecular layer, 
but the nerve processes within the granular layer after running a short dis- 
tance split up around many cells. Neuron fibers from the medullary mass 
of the cerebellum, whose origin is not yet known, end in the granular layer. 
We are chiefly indebted for our knowledge of the finer structure of the cortex 
of the brain to the investigations of Ramon and van Gehuchten, and this 
knowledge has been acquired by the Golgi method. 



F. NEURON DISEASES OF THE CEREBELLUM. FRIEDREICH'S ATAXIA 

We know as yet of no diseases in which the tracts which lead to the cere- 
bellum or originate from it are alone affected. Such pure neuron diseases 
of the cerebellum cannot arise independently, because the cerebellar tracts 
are most intimately connected both anatomically and functionally with 
other spinal and cerebral fiber systems, and in pathologic cases all must be 
affected. 

There is, however, a disease which implicates a definite cerebellar tract; 
namely, that situated in the periphery of the lateral column tracts of the 
spinal cord. This disease is Friedreich's ataxia; it is hereditary, and mani- 
fests itself by marked ataxic disturbances, yet, even when the patella reflexes 
are absent, sensation is not decidedly impaired. 

Anatomically, this disease produces degeneration in three different column 
systems. We find destruction of fibers in the posterior columns (but not in 

the roots or in their course), in the 
pyramidal tracts, and in the cere- 
bellar lateral column tracts (Figs. 
112, 113). It is possible that this 
degeneration in the posterior col- 
umns takes place in the area of the 
association tracts, the course of 
which we know to be shorter. As 
lias been stated, the posterior roots 
and their zone of entrance are nor- 
mal, probably also their entire 
course. The disease in the course 
of the pyramidal tracts appar- 
ently does not affect the motor 
fibers since muscular spasms and increased reflexes constantly appear here. 
It is still a mooted question which must be settled b}^ further researches 
which fiber tract is destroyed; I must call attention to Monakow's tract 
which unites with the pyramidal tracts and descends in the lateral co- 
umns. There is certainly degeneration of the cerebellar lateral column 
tracts and the cells of Clarice's columns which form their origin. Since 
various authors (Senator and others) believe in a primary implication 
(congenital atrophy) of the cerebellum, we may assume disease of the 
tracts of the cerebellum. We may hope that, in the future, thorough his- 
tologic investigations of this rare disease may give us more accurate infor- 
mation as to the origin and relation of the cells connected with the degen- 
erated fibers. 







jMlRiPBk. 




; J^^BHjy^^HP jBp *5k '-•"*'* 




** •' V ' *r 


' 



Fig. 113. — Friedreich's Ataxia. 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 



113 



G. COMBINED SYSTEM DISEASES 

There is till another group of diseases of the spinal cord in which the 
anatomical finding closely resembles that of Friedreich's ataxia. These cases 
have been designated combined system diseases. The name may be deemed 
fitting only when those areas in the columns of the spinal cord in which defi- 
nite tracts run are affected. In the posterior columns, GolPs columns in toto, 
Burdach's columns alone were found to be partially degenerated, in the 
lateral columns the region of the 
pyramidal tracts, but usually, as 
in Friedreich's ataxia, the cere- 
bellar lateral tracts, not rarely 
the region of Clowers' columns, 
and in the anterior columns 
Turk's bundles in the immediate 
vicinity are often implicated 
(Fig. 105). But these degener- 
ations are not strictly limited to 
the previously mentioned tracts, 
and in each of the individual 
cases now frequently observed 
the seat of the disease varies, at one time this, at another time that, column 
being implicated. The clinical symptoms vary exceedingly; they resemble 
those of other spinal cord diseases, and may be a- vague as the anatomical 
localization. Hence, the anatomical starting-point of the disease remains 
obscure, and we are in doubt whether a diffuse Leukomyelitis chronica, per- 
haps starting from the vessels, or a primary diffuse disease of the gray sub- 
stance is the cause. We cannot to-day positively assert that the affection is 
a neuron disease, as is maintained by some authorities. We may point by 
analogy to similar degenerations of the white substance, not in columns but, 
on the contrary, occurring in foci, particularly in severe anemias and in cer- 
tain intoxications. Here the assumption of processes which start from the 
vessels and only secondarily lead to degeneration of the nervous substances 
is much more reasonable. 




Fig. 114. 



-Combined System Diseases. 
(After Oppenheim.) 



V. OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 

I must now consider the pathological histology of those affections of the 
brain and spinal cord which do not depend upon the primary disease of 
one nerve tract or of one or more neurons, which, therefore, are not limited 
in their extent but are found in any area regardless of the nature and func- 
tion of the nerve elements there situated. The cause of such diseases is 
acute or chronic inflammation which may occur diffusely or in foci within 
the central nervous system, or which may attack the membranes, and sec- 
ondarily implicate the nervous substance ; at times we are dealing with degen- 
erative processes which may originate in the vessels, or from the interstitial 
tissue and may develop in various areas. Finally, tumors or injuries may 
simultaneously implicate the various nervous elements. 

All the tracts which are damaged by diffuse or circumscribed (focal) 



114 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

disease of the nervous system must show degeneration beyond the focus 
since that portion of the tract immediately degenerates which is cut off by 
the pathologic focus from the neuron cells belonging to it. Therefore, de- 
generation will follow the direction of the affected tract either peripherally 
from a descending focus or centrally from an ascending one. 

It is evident that secondary degenerations always occur in primary foci 
of the central nervous system, and these are often extensive if long tracts are 
interrupted. At the same time the study of such secondary degenerations 
after primary focal diseases is invaluable, not only for the full comprehension 
of paralyses present but also for the recognition of the structure of the cen- 
tral nervous system. In a certain sense they correspond to experimental sec- 
tions to which we owe much of our knowledge of the course of fibers. 

In the following I shall attempt to describe the most important inflam- 
matory focal diseases while I must refrain from considering tumors and in- 
juries, since these would lead us too far afield. Secondary degeneration fol- 
lowing inflammatory foci, tumors and injuries, in so far as its course is known, 
will be discussed. 



A. ACUTE MYELITIS, ENCEPHALITIS, AND ACUTE SOFTENING 

Acute myelitis presents manifold clinical pictures. Acute encephalitis, 
which has been less accurately studied, occasionally produces most unlike 
symptom-complexes according to the seat of the affection and whether it is 
circumscribed or diffuse; for an accurate portrayal of the different pathologic 
types text-books upon nervous diseases should be consulted. 

The histologic picture to be described is uniform. In most of these proc- 
esses two characteristic changes are invariably found in acute myelitic foci. 
First, the invariable alteration in the walls of the vessels which is conspicuous 
in the histologic picture; the dilatation and the abnormal filling of the ves- 
sels which not infrequently causes the smallest branches to rupture, the exu- 
dation of cells and lymph from the same, and the proliferation of " round 
cells " from the vascular wall and the glia tissue. Occasionally the degen- 
erative changes of the nervous elements in the region of the focus are of great 
histologic interest. The acute inflammatory changes in the nervous apparatus 
do not differ essentially from those of other organs. 

Certain individual differences are evident. These are mainly the conse- 
quence of the soft consistence of the tissue, and this we refer to the presence 
of myelin, a substance readily dissolved and absorbed. Added to this are 
deviations from the conditions perceived in other organs which are often evi- 
dent at some distance, and produce secondary alterations in the nervous tissue. 

The consistence of the tissue frequently leads to softening. Every exten- 
sive myelitic and encephalitic focus in the white substance may be reduced 
to a pappy consistence so that in making microscopic preparations it is diffi- 
cult to keep the destroyed elements in position even by careful embedding 
in hardening substances. When the processes begin acutely we find in the 
white substance in the area of greatest inflammation a disintegrated field in 
which only the tissue elements of greatest resistance, the vascular walls, and 
the glia tissue (fibers and cells) are visible, although they may be displaced. 
In the main, however, we find here myelin granules, swollen and broken up 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 115 

axis cylinders in irregular arrangement which cannot always be recognized 
as such. Hemorrhages readily occur in these softened tissues, the greatly 
distended vessels finding no external resistance rupture, and the effusion of 
blood is rapidly distributed throughout the necrotic, softened, and destroyed 
tissue. 

The cell proliferation in the surroundings of the vessels is of a somewhat 
different character in the white substance of the nervous system from that 
in other organs. We usually find the body of such cells enormously swollen 
and completely filled with remains of tissue, which, because of their softer 
consistence, are more readily absorbent than the necrotic elements of other 
organs. Therefore, these cells are filled with a granular substance which 
becomes black on the application of osmic acid and this is to be regarded 
as altered myelin. In contrast with normal myelin this blackens with Marchi's 
fluid (see below), stains deeply witli sudan red, scarlet or other dye-stuffs 
acting on fat, which is not the case with normal myelin. Such cells are called 
granular cells. According to the softness of the tissue they are often found 
quite remote from the vascular wall in which they appear to originate. They 
push forward in rows, palisade-like, to the border of the diseased area. 

Stress must be laid upon the fact that these granular cells are all mono- 
nuclear — not rarely the nucleus is still present — and it is noteworthy that 
cells are present in the inflammatory diseases of the central nervous system 
which cannot he regarded as wandering cells of the blood but as proliferated 
cells of the tissue, especially of the advent ilia of the vessels, and these, like 
the multinuclear phagwytes of the blood, have loaded themselves with gran- 
ules of myelin and other shreds of tissue. They are apparently identical with 
the "round cells " in the inflammatory foci of other tissues, but are more 
dense, and are visibly more distended with tissue masses than others. 

The farther from the center of the focus that we search for normal 
tissue, the more numerous do we find the retained nerve fibers to be in the 
white substance. At all events, we note in these areas numerous nervous 
elements in a transitional stage between the normal and the pathologic. In 
an unstained condition, or with ordinary methods of staining, these transitions 
are not readily perceptible, but in the Marchi method, which makes the granu- 
lar cells very distinct, we possess a fine means of determining the recent 
destruction of the medullary sheaths. 

Marchi's Method. — Here a brief statement of the importance of Marchi's 
method may be of interest. During the process of its destruction the chemical 
composition of the myelin apparently changes, for in Marchi's fluid, which 
consists of three parts of Miiller's fluid and one part of a one per cent, hyper- 
osmic acid, normal myelin sheaths are unstained; at most they change to 
a pale gray color. In beginning acute degeneration the nerve sheaths behave 
differently. Here they are stained black, i. e., the myelin is found in deep 
black granules around the colorless axis cylinder, and the globules are de- 
stroyed; this can be best seen in longitudinal sections. The medullary sub- 
stance which apparently shows chemical alteration now stains like the sub- 
stance of the granular cells with which it is perhaps identical, also like the 
dense granules which appear in the lipochrome of the nerve-cells, to which I 
previously referred. 

But if the gray substance has been attacked by acute myelitis, this struc- 



116 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

ture also suffers and shows marked pathologic changes. The most important, 
although not always the most obvious, is the degeneration of the nerve-cells, 
but, because of the firmer consistence of the tissue, no conspicuous softening 
is present. In intense myelitis the nerve-cells are either entirely destroyed 
or to such an extent that, robbed of their processes, they are detected as 
clumps which can no longer be differentiated from other cellular products 
of inflammation in their midst. Occasionally, however, their configuration 
is apparently normal and at first conveys the impression of a certain degree 
of atrophy, unless staining according to Mssl shows them to have been com- 
pletely destroyed in the interior. The body of the cell is filled by a fine gran- 
ular dust instead of the usual coarse granular substance, and this no longer 
stains intensely, the nucleus passes away from the center to the side of the 
cell, and only the lipochrome maintains its appearance and position as the 
most resistant substance. That the fibrous net of finer nerves in the gray 
substance is destroyed by the inflammation is shown by preparation stained 
according to Weigert's method, or with triacid (Rosin), or with van Gison's 
method, for we recognize a massive formation of new cells particularly in the 
surroundings of the vessels. These cells, however, are not so large as the 
granular cells in the white substance, for there are no masses of detritus 
in the myelin. Nevertheless their origin is the same as that of the adventitia 
of the vessels. Dilated vessels were everywhere observed and hemorrhages 
were numerous. 

In the recent foci of acute inflammation the glia tissue is only passively 
implicated. Where the inflammation is most severe much of it is softened 
although even there some fibers show greater resistance than the nervous tis- 
sue; in the mass of tissue detritus we readily find retained glia nuclei and 
fibers. The glia is usually quite well retained in the peripheral areas of acute 
inflammation even when there are hemorrhages, granular cells, and dilated 
vessels. The meshed spaces also are apparently in good condition when the 
nerve tubules of the white substance have disappeared, that is, have degen- 
erated. Often this network, instead of being filled by nervous elements, is 
made up of granular globules or well retained red blood-corpuscles from hem- 
orrhages. 

Thus we have the histologic picture presented by acute inflammation of 
the white and gray substance of the central nervous system. Microscopically 
only the picture of acute inflammation is recognizable; the clinical variations 
which appear during life are not seen ; and the manifold and varying etiologic 
factors which permit the differentiation of different forms of acute myelitis 
form no part of the histologic picture. 

From the microscopic appearance it is difficult to differentiate between 
acute inflammation and acute softening such as is produced by nutritive 
disturbances on the part of altered vessels, by occlusion or rupture of the same, 
or by toxic agents. 

Clinically the differentiation is generally eas}^. The anatomical picture 
is invariably the same, and in inflammation also we have the histologic appear- 
ance of softening. Both dilatation of the smallest vessels and hemorrhages 
appear in the focus of softening,, apparently because the broken up tissue 
exerts no power of resistance upon the vessels which are usually friable, and 
thus the vis a tergo, the internal pressure, immoderately enlarges the caliber 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 



117 



and finally causes the wall to rupture. Granular cells also are in the sur- 
roundings of the focus. But the quantity of mononuclear inflammatory 
cells in the vessels is more scant, there is no hyperemia of the large arterial 
vessels, and thus a slight difference is perceptible. Those who are especially 
interested in the subject are referred to my article upon acute myelitis in Which 
this condition is described somewhat more minutely (Zeitschrift f. hlin. 
Med., 1896). 

B. ACUTE POLIOMYELITIS 

We now come to a special form of acute myelitis which is chiefly seated 
in the anterior horns of the spinal cord or in the motor nuclei of the medulla 
oblongata. Its limitation to the seat of origin of the first motor neuron 
raises the question whether we are not here dealing with a pure neuron dis- 
ease which secondarily implicates the vessel, and this is the opinion of some 
authors. 

However, the histologic picture makes it clear that no pure disease of the 
nervous apparatus, as in the previously mentioned disease of the first motor 
neuron, is present (Goldscheider and others). On the contrary, the nervous 
tissue is so slightly implicated that it appears questionable whether in this 
affection the nerve-cells and the processes are not merely attacked secondarily 
and passively. To this must he added that not only are I he motor cells of the 
diseased areas chiefly affected, but even those in the area of inflammation 
which have other functions than motor are diseased. Therefore, in spite of its 
peculiar localization this form of most acute inflammation is included among 
the acute forms of myelitis. 

The process is disseminated 
throughout the entire length of 
the spinal cord, in some areas 
simultaneously and acutely, but 
the intensity of the disease va- 
ries, and it leads to irrepara- 
ble changes only in individual 
areas. Clinically there is pa- 
ralysis of one or more extrem- 
ities. Histologically, in recent 
cases, there is an acute inflam- 
matory dilatation of the ves- 
sels of the gray suhstance of 
the anterior horns, the branches 
of the anterior spinal artery, 
and simultaneously a marked 
proliferation of round cells. 
The process rarely extends into 
the white substance beyond the 
border of the anterior horns. 

Here granular cells are not infrequently observed and hemorrhages often 
occur. In the condition previously described the nerve-cells of the affected 
region are found to be in all stages of degeneration. The motor nerve fibers 
of the diseased anterior horn cells then secondarily degenerate, and the nerve 




Fig. 115. — Lumbar Cord After an Attack of 
Acute Poliomyelitis of the Left Side. 



118 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

fibers disappear peripherally from the anterior roots to the paralyzed muscle. 
In atrophic paralysis the muscles also degenerate. 

If the process is extremely intense, when it has run its course the nerve 
elements are permanently destroyed and the affected portion of the anterior 
horn becomes sclerotic. It decreases in size (Fig. 115), is filled with tense 
glia tissue, and is almost entirely devoid of nerve elements. 

It is quite generally known that the disease occurs in earliest childhood, 
but it is also met with in adults although much more rarely (Moritz Mayer, 
Duchenne, Erb, F. Schultze, F. M tiller, Strumpell). In these cases the ana- 
tomical picture is almost identical with that of infantile paralysis. 

C. ACUTE BULBAR PARALYSIS 

Inflammatory disease in the region of the motor nuclei of the cranial nerve, 
which corresponds to poliomyelitis of the spinal cord, and bears the name of 
acute inflammatory bulbar paralysis, unlike disease of the spinal cord, is 
rare and is seen only in adults. In this bulbar poliomyelitis, as in the medul- 
lary form, we find the same affection of the vessels, the same cell prolifera- 
tion, and the same destruction of nerve-cells. Perhaps the alteration of the 
motor neuron (which alone may be clinically prominent) is more conspicu- 
ous because the sensory and other tracts as well as the motor nuclei are 
situated in its immediate vicinity, and this is regarded by some as a proof 
that we are here dealing with a disease of the first motor neuron of the brain. 
However, the above objection may also be urged against this view as well as 
the fact that only the nuclei in the region of the fourth ventricle are diseased, 
while those of the mid-brain, the origin of the oculomotor and trochlear, 
are not implicated. 

Clinically, therefore, this rare affection may be readily confounded with 
affections which run a similar course but are histologically quite different. 
I shall mention here only acute peripheral neuritis of the cranial nerves, 
bulbar apoplexy (apoplectic bulbar paralysis), thrombosis of the basilar artery, 
asthenic bulbar paralysis (Strumpell) (pseudo-bulbar paralysis), and Lan- 
dry's paralysis, without discussing their histological peculiarities which do not 
come within the scope of this article. 

D. CHRONIC MYELITIS AND ENCEPHALITIS 

The affection designated as chronic myelitis and encephalitis varies con- 
siderably from the preceding picture of acute myelitis and encephalitis. As 
is well known, this disease often arises from acute processes, and much more 
rarely develops spontaneously. It is also well known that chronic processes 
terminate in so-called sclerosis. So long, however, as this stage is not reached, 
the histologic picture varies much more than in the acute form. This is 
due to the numerous and gradual transitional changes from early to later 
stages of development, and as long as sclerosis is not yet complete this may 
always be recognized. I have already described acute recent changes, and to 
this I will add only those which occur when the process is older, or which 
exist from the onset provided the disease has a chronic beginning. 

The more protracted the disease, the more complete the disappearance 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 119 

of the destroyed nervous substance, the granular cells, and the hemorrhages, 
and the more distributed and thickened the glia tissue and the walls of the 
vessels. At first cells of the adventitia (mononuclear structures deficient in 
protoplasm) as well as glia cells (usually containing only a narrow proto- 
plasm body) (triaeid stain) are greatly increased in numbers and these, at 
the same time, fill the spaces caused by the absence of nerve tissue. Granular 
cells are quite scant. Finally sclerosis occurs, not infrequently in a focus in 
the other parts of which quite recent changes are visible. 

Sclerosis of the gray substance may be recognized by the destruction of 
nerve-cells and those fine fibers which pass through the normal gray sub- 
stance in so dense an arrangement (with Weigerfs stain). In another area 
there is a thick network of glia with increased cells and thickened vascular 
walls, the adventitia of which is conspicuously broad and very rich in nuclei. 
The capillary wall is also thicker than the normal. In the white substance 
sclerosis is readily recognized. Upon transverse section no round cells char- 
acteristic of the nerve fibers are to be seen, their places being filled by glia 
tissue developed from proliferation of the tissues of the normal glia meshes. 
In this stage a marked proliferation of the glia cells with especially numer- 
ous processes is particularly distinct, and we also note those cells which have 
been designated neuroglia cells (spider cells). In addition there are great 
numbers of peculiar structures formed somewhat differently and about the 
size of a leukocyte which are called corpora amylacea. They resemble starch 
granules, are concentrically layered, but do not give the reaction for starch, 
and have not yet been fully .analyzed. Furthermore, a dense film work of 
fibers forms in the white substance, this being at first rich in nuclei which 
it subsequently loses and then resembles connective tissue, its structure being 
recognizable only by the use of Weigert's glia -tain. Where septa of the pia 
resembling connective tissue penetrate into the sclerotic focus, they also take 
part in the proliferation and are distributed still deeper. Occasionally the 
nuclear proliferation of glia tissue changed in this manner remains for a 
long time (particularly in the gray substance) in old well developed sclerotic 
foci, and finally reappears. The triaeid stain enables us distinctly to recog- 
nize these conditions. The vascular wall of the white >ul»tance is also mark- 
edly involved in the sclerosis; it becomes thickened, as was previously stated, 
especially in the region of the adventitia. 

E. CHRONIC POLIOMYELITIS 

As acute poliomyelitis represents a particular form of acute myelitis, so 
chronic myelitis is typical of another special form, chronic poliomyelitis. 

There have been but few thorough investigations of this very rare disease 
(Oppenheim. Xonne and others), and its curability accounts for the very 
exceptional occurrence of autopsies. The paralyzed muscles again resume 
their functions although a few remain weak ; we are not fully cognizant of 
the histologic conditions. But the histologic findings obtained up to the 
present time very closely resemble those of the acute cases, in which the 
implication of the medulla oblongata is prominent. 

Chronic poliomyelitis is. therefore, not limited to the spinal cord, and 
the name poliomyelo-encephalitis would be much more applicable. 
10 



120 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 




Fig. 116.- 



-Sclerotic Foci in the Spinal Cord, 
Stain. (After Oppenheim.) 



Weigert's 



F. MULTIPLE SCLEROSIS 

Multiple sclerosis is a peculiar form of chronic myelitis with typical his- 
tologic changes. It usually implicates the entire spinal cord as far as the 
medulla oblongata ; isolated foci are not infrequently found also in the middle 
and inter-brain. From the onset the disease is chronic and focal. The situ- 
ation of the foci is entirely independent of the course of the fibers and the 

structure of the nerve 
substance, sometimes 
being only in the gray 
or in the white sub- 
stance ; occasionally 
they occur in both, pass- 
ing from the white to 
the gray substance. 
They are distinctly 
separated from the nor- 
mal surroundings, 
show no sign of acute 
destruction, of hemor- 
rhage, or of granular cells., and are in that stage of sclerosis which still shows 
numerous cells: Glia proliferation, vascular thickening, increase of nuclei. 
The spinal cord in the medulla is permeated by such foci. With Weigert's 
glia stain they are perhaps even 
more prominent than with the me- 
dullary sheath stain; for we then 
often recognize numerous, micro- 
scopically small foci which would 
otherwise not be visible. Much 
larger foci are seen in the shape 
of round or angular circumscribed 
spots irregularly disseminated like 
defects in the nervous substance, 
and these appear as if they had 
been pierced with a stiletto. The 
chronic myelitic process of this dis- 
ease is very conspicuous and does not 
especially attach the axis cylinder. 

This is retained to a great extent, and precludes those secondary changes which 
we invariably see when the nerve fibers are completely destroyed (Figs. 116, 117). 
Those authors are probably correct who designate the disease as gliosis. 
Its origin from the nervous substance may be excluded, but the implication 
of the vessels is secondary, the seat of the foci by no means corresponding 
with their course and distribution. 




Fig. 117. — Multiple Sclerosis. 



G. SECONDARY DEGENERATIONS 

In pathologic processes of any organ certain distant effects are produced, 
so that the surroundings of parts not directly affected may show certain 
inflammatory or degenerative alterations. In the central nervous system, 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 



121 



however, other damage may be explained by the intimate anatomical relation 
of the neuron constituents. What Waller once designated as roots of the 
spinal cord, the anterior of which, corresponding to their course, are centrif- 
ugal, and the posterior centripetal, is true of every tract of the brain and 
spinal cord. Section and interruption of the conduction tract and the conse- 
quent separation from the nourishing cell invariably destroy the peripheral 
portion of the neuron. This degeneration is intense and irreparable provided 
communication is not restored. Moreover, central disturbances occur, but 
these, as I have several times stated, are less intense, they can be estimated 
only by the finer methods, and become distinct only on continued absence 
of function of the neuron. 

The knowledge of the path of secondary degenerations, i. e.. the destruc- 
tion of the peripheral ends of the nervous conduction tracts continuing from 




^^^ 









f 





© 





Fig. 118. — Transvers Myelitis with Ascending and Descending Degeneration. 
a, Staining according to Weigert; /3, triacid stain. The dark areas indicate degeneration. 

the point of interruption, is important in many respects; first, because in 
determining the localization and extent of a disease of the central nervous 
organs it is important that we should know what portions of the neuron 
are implicated, and what deleterious effects the primary focus may have upon 
remote regions of the neuron system. Very often circumscribed disease of 
the nervous system, particularly in regions where nerve centers or tracts are 
closely adjacent (medulla oblongata), causes extensive and serious degenera- 
tion of entire tracts by damaging many of the neurons situated within the 
region of the focus. 

A knowledge of the course of secondary degeneration is important, too, 
because not infrequently it enables us to determine the seat of the secondary 
focus which would otherwise be very difficult. 

Finally, these degenerations furnish a fruitful field for research. This 



122 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



unexplored region in the course of the tracts has been persistently studied, 
and if this investigation is continued with finer methods many other neuron 
systems, previously unknown, will doubtless be discovered. 

Every foci developed in the spinal cord, provided it is sufficiently exten- 
sive, leads to ascending and descending degeneration. This is most distinct 
and intense in foci which affect the entire transverse section. 

Transverse myelitis (Fig. 118) furnishes the clearest types of these degen- 
erations, and its course will therefore best illustrate other less extensive foci 
and slighter degenerations. 

(1) SECONDARY" DESCENDING DEGENERATION OF THE 

SPINAL CORD 

It is evident that descending degeneration follows in the main the course 
of the motor tracts in the spinal cord, since, so far as we know, all the motor 
tracts in the structure of the spinal cord run in a descending direction, i. e., 
caudally. 

True, we also know of a few sensory centrifugal tracts; it will be remem- 
bered that all of the fibers which enter the spinal cord (spinal ganglia) from 

the primary sensory centers divide into 
two branches, a descending branch which, 
as a rule, is short, and an ascending one 
which is often much longer. There- 
fore, in myelitic foci those descending 





Fig. 119. — Descending Degeneration of a Pyramidal Tract of the Spinal Cord. 



branches whose course is through the region of the focus and thereby discon- 
nected from the center must degenerate. But these fibers are found only in 

the posterior columns, and even there 
they form so small a part that they 
often cause no visible degeneration 
but sometimes only a complete dis- 
appearance of fibers; this we occa- 
sionally find described as Schultze's 
comma-like degeneration of the pos- 
terior columns, because the degener- 
together in the posterior column in 





Fig. 120. — Bilateral Descending Degen- 
eration of the Pyramidal Tract. 



ated bundles usually (not always !) lie 
the shape of a comma on transsection. 

The principal mass of the descending degenerative fibers is situated in 
the lateral and anterior columns. In the lateral column this region is desig- 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 



123 



nated as the pyramidal tract (Fig. 118, e and /, Figs. 119-121). It is usu- 
ally situated in the posterior internal portion of the lateral column, but does 
not extend to its border zone nor to the gray substance. 

We have also seen that there are two other descending tracts in the lateral 




Fig. 121. — a, Diagram of descending degeneration of a pyramidal tract (after Edinger); b, 
transverse section of a degenerated pyramidal tract (on the right side) and of the pons. 



124 HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 

columns which are subject to transverse lesions. One of these is Monalcow's 
bundle which descends from the middle brain in the lateral columns, partly 
between the pyramidal fibers but somewhat anterior and external to them, 
and the second is LbwentliaVs bundle which is situated quite ventrally in the 
lateral columns and at their margin. 

In the anterior columns at either side of the anterior longitudinal space 
is the remainder of the uncrossed pyramidal tracts, called Turk's bundle, 
which also occasionally shows descending degeneration. 

Degenerations of fibers is simultaneously observed, usually in the anterior 
(white) commissure; these implicate continuations of the fibers of Turk's 
bundle which have not yet crossed. 

In this region descending degeneration attacks all fibers which are sepa- 
rated from their cells below the transverse disease, and it extends to their 
own and their collateral terminal arborization. 

Besides the tracts in the neighborhood of the foci which have a long 
course many short tracts are interrupted. In recent affections these degen- 
erations are best recognized by the Marchi method. This shows immediately 
below the foci (as well as immediately above it), the degeneration of enor- 
mous numbers of nerve fibers (Fig. 118, c, d) in those areas of the white 
substance which are immediately adjacent to the gray substance, particularly 
in the lateral columns, and in the anterior columns ; it is somewhat less marked 
in the posterior columns. In the more external areas of the spinal cord, 
particularly in the region of the anterior, columnar, basic bundle, we observe 
an intense black staining in transverse sections immediately below the foci. 
These limited secondary degenerations must be referred to short tracts which 
run in segments from section to section within the spinal cord and which 
are designated quite correctly as association systems of the spinal cord. 

In conclusion it must be stated that in the inflammatory focus even the 
motor anterior root fibers arising from the anterior horn cells which have 
been destroyed invariably degenerate and produce simultaneous paralysis and 
atrophy of the roots belonging thereto. 

(2) ASCENDING DEGENERATION IN THE SPINAL CORD 

The centripetal tracts degenerate above the focus. Many of them belong 
to the posterior root regions, and we know that some of the branches of 
these do not enter the gray substance but mount upward in the posterior 






Fig. 122. — Secondary Ascending Degeneration after Disease of the Conus Medtjllaris. 

column. These tracts pass to the medulla in Burdach's and GolPs columns, 
the former invariably carrying the newly added posterior root fibers, and the 
latter the fibers which entered lower down. Immediately above the focus in 
both posterior columns marked degeneration is found; the higher Burdach's 



OTHER DISEASES OF THE CENTRAL NERVOUS SYSTEM 125 

columns ascend the more free they become ; finally, if the focus is low enough, 
Goll's columns only will be implicated (Fig. 118, a, b, Fig. 122, c, d, I). We 
have no knowledge of long tracts in the posterior columns other than those 
of the posterior root fibers. 

The cerebellar lateral column tracts and Gowers' bundle (Fig. 122, A, 
Fig. 123) also degenerate in an upward direction. These columns have already 
been described. They are situated at the border of the lateral columns, the 
former behind the latter ; but Gowers' bundle penetrates somewhat more deeply 






Fig. 122 A. — Ascending Degeneration (Right-sided Fig. 123. — Secondary As- 

Myelitic Focus). cending Degeneration. 

into the interior of the lateral columns. The fibers of the cerebellar lateral 
column tract originate from the cells of Clarke's columns. Those fibers of the 
lateral columiix must perish which originate from sensory cells situated on 
the opposite side in the gnu/ substance of (he posterior horns, e.g., the fibers 
of the second sensory neuron which in the medulla forms the median portion 
of the lemniscus and does not cross. These fibers, however, are united into 
small bundles in the lateral columns, therefore in a transverse interruption 
are not very prominent. But above '.he lemniscus layer of the medulla, in 
the pons and higher, they may be demonstrated as degenerated fibers. 

Immediately above the transverse interruption as well as beneath it many 
short tracts are found which have degenerated. These also must be regarded 
as association tracts. 

(3) SECONDARY DEGENERATION IX THE BRAIN 

The law of secondary degeneration in the brain is exceedingly difficult to 
understand. Much study has been expended upon it, and some remarkable 
facts have come to light which have been utilized in histology and structure. 
But the conditions are still too obscure and too complicated to be accurately 
described. The pyramidal tract has been more thoroughly investigated than 
others. Here I shall merely present a diagrammatic figure (Fig. 121). 



H. SYPHILIS OF THE NERVOUS SYSTEM 

Many diseases of the central nervous s}'Stem are attributed to syphilis. 
In some of these affections, for example, in progressive paralysis, a causal 
connection can only be determined by statistics, while changes of a syphilitic 
nature are never revealed in the histologic picture. It is well known that 
tabes shows histologically the changes already indicated; no signs pointing 
jx> syphilitic change have ever been determined. 

There are, however, many nervous diseases in which the syphilitic cause 



126 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 




SSBL 

Fig. 124. — Syphilitic Endarteritis and 
Gummatous Infiltration of the Sur- 
rounding Area. 




can be demonstrated. The deleterious influence of tertiary syphilis upon the 
central nervous organs has frequently been revealed anatomically, and in the 

last decades this has been repeatedly 
explained and described. 

In such cases a syphilitic endar- 
teritis most often develops (Figs. 124, 
125) as well as gummatous disease of 
the membranes of the brain. For in- 
stance, we find in the spinal cord a 
gummatous arachnoiditis, in the brain 
a gummatous infiltration of the mem- 
branes, especially at the base, which 
thence distributes itself to the nerve 
sheaths and to the choroidal plexus. 
These conditions usually develop slow- 
ly and chronically, and produce sec- 
ondary disturbances in the course of 
the nervous substance itself; particu- 
larly in those areas surrounded or cov- 
ered by the infiltrated membranes. It 
is therefore clear that gummatous in- 
filtration of the membranes of the 
spinal cord, provided it reaches a cer- 
tain extent, may produce secondary degeneration by compression. Often we 
find that degeneration (Fig. 126) of the lateral columns has also attacked 
the pyramidal tracts or the posterior column, in consequence of which muscu- 
lar contractions, disturbances in sensation, and coordination develop (syphil- 
itic spinal paralysis, after 

Erb). Microscopically we <\ Z* 7 ~-^A ,n 

see scleroses in the lateral ", „r^ -^-'' *"__ * -UT^ 

and posterior columns due 
to compression of the roots 
passing in and out, and 
these show atrophy of their 
fibers while the connective 
tissue of the membranes 
of the brain is enormously 
thickened and is perme- 
ated by small-celled infil- 
tration. 

This concludes our de- 
scription of the normal 
histology of the central 
nervous system and its 
microscopic changes. In 
what has been detailed, it 
has been impossible to give 

more than a partial picture, and it was presupposed that the macroscopic and 
gross anatomical relations of the central nervous system were already familiar 





Fig. 



125. — The Normal and the Syphilitic ally Dis- 
eased Anterior Spinal Artery. 










Fig. 126a. — Syphilitic Spinal, Paralysis. 



127 



128 



HISTOLOGY OF THE CENTRAL NERVOUS SYSTEM 



to the reader. It is, however, at once apparent that the incompleteness of my 
description is in great part due to want of microscopic knowledge of the struc- 
ture of the organs of the central nervous system. 

Notwithstanding this, in reviewing the ground gone over, Ave find much 
valuable data and abundant stimulation for future researches. The new century 
advances, and must promote our knowledge of neurology to the same extent 
as did the preceding century. We hope that scientific research may prove as 
successful in the future as it has been in the past, and that it may be of four- 





Fig. 126&. — Syphilitic Spixal Paralysis. 



fold nature, as follows : ( 1 ) Careful microscopic investigation of the diseased 
parts according to the latest reliable methods; (2) the various methods of 
embrj/ologic investigation; (3) the comparative anatomical method, especially 
utilized by Edinger; (4) experimental investigation, which is constantly fur- 
nishing such excellent results (section and division). Perhaps in the course 
of the next hundred years neurologic investigations based upon these methods 
will unveil in its entirety the complete structure of the central nervous system, 
of the brain, the organ of all organs. 



GENERAL NEUROLOGICAL DIAGNOSIS 

INCLUDING THE EXAMINATION OF PATIENTS WITH DISEASES 
OF THE NERVOUS SYSTEM 

By P. SCHUSTER, Berlin 

INTRODUCTION 

The office patients who consult the specialist for diseases of the nervous 
system all show a certain peculiarity which distinguishes them from other 
patients, namely, the ease with which they may be mentally influenced. This 
fact is generally known, even by the laity, of patients suffering from functional 
diseases of the nervous system, such as hysteria, neurasthenia, hypochondriasis, 
etc. — and much more fully by the physician. Somewhat less keenly appre- 
ciated by physicians is the fact that this same influence of suggestion is exerted 
upon patients suffering from organic nervous diseases. It is true, as a rule, 
that we have less power to control a patient with organic disease of the nervous 
system than the neurotic, for in the latter the possibility of suggestion is 
entirely endogenous, and arises from the internal nature of the disease; in 
the other patients the possibility of suggestion — provided the organic path- 
ologic picture is not obscured by neuroses — is of a more exogenous character, 
and is generated and strengthened above all else by the consciousness of the 
patient that he suffers from disease of the nervous system, by the layman's 
very peculiar conceptions of a nervous disease, and by the consequent action 
and manner of those about him. 

What has just been pointed out as peculiar to all nervous patients, the 
psychical factor common to them all, perhaps accounts for the fact that the 
layman more closely associates mental and nervous diseases than does the 
physician, and that even he commonly combines these diseases. 

The increased possibility of psychically influencing the nervous patient, 
just outlined, is not only of the greatest significance in the general pathologic 
investigation, but especially so in treatment. For in the psychical peculiarity 
of our patient there is a mighty therapeutic force, the wise application of 
which may relieve many of the patient's complaints and difficulties, and the 
improper use or neglect of which may just as greatly injure him. 

This being the case, the physician must endeavor, even at the first exam- 
ination of the patient, to gain an influence over him, and at the same time 
to become psychically en rapport with him. The physician must remember 
that with many patients with nervous disease even the examination represents 
a part of the treatment, and that a single incautious question or a careless 
remark may endanger or frustrate all later therapeutic endeavors. 

129 



130 GENERAL NEUROLOGICAL DIAGNOSIS 

The careful and patient taking of the history, and a thorough examination 
of the entire body which should consume as much time as is necessary, often 
surprisingly smooths the way to treatment. Here we are assisted by the fact 
that most nervous patients desire very frequent and careful examinations, and 
rarely give the physician any trouble on account of inconvenience or by their 
prudery. 

If the foregoing is borne in mind we may readily understand that the 
following description will be merely a diagram or framework representing 
only the technic and the mode of employing instruments in an examination. 
And although a hint will occasionally be given as to the motive for under- 
taking special investigations in the patient, or as to the caution with which 
certain questions are to be asked, nevertheless nearly all must be left to per- 
sonal and professional tact, to the human delicacy as well as the personal 
diplomacy of the physician, so that the barren structure of the technic of 
examination shall become a living process. 

In this description we shall refer only to what is practically most impor- 
tant, and, in order to keep within the scope of this article, shall not drift 
into details. 

HISTORY (ANAMNESIS) 

(The extent of the history, the manner of eliciting it, family predisposition, psy- 
choses am. nervous affections appearing in the family, previous diseases of the patient, 
childhood, education, occupation, emotional tendencies, service in the army, develop- 
ment of the last affection, its prior course, trauma, alcohol, syphilis, sexual relations.) 

The history is of predominant importance in neurologic examination be- 
cause in many cases the diagnosis cannot be made from the results of examina- 
tion but only on the basis of the history. Therefore a certain knowledge of 
diseases of the nervous system is indispensable if the history is to be at all 
reliable. The value and utility of the history by no means depends upon its 
length; on the contrary, useless and secondary details detract from its value. 
One of the chief dangers in taking a history is that we are liable to wander 
from the main road into byways and thus we fail to take a comprehensive 
survey or at least waste much time. 

Another source of error consists in believing too implicitly the reports 
of the patient or his friends, thus forming false conceptions in regard to 
causal and periodic conditions. 

It would, however, be a mistake and most unwise to stop the patient at 
once in his uncritical report, and insist upon another mode of description. 
This engenders in the patient a feeling that he is suppressed, that we are not 
allowing him sufficient time, and that without his report he can form an 
opinion as to his condition. 

LTnder such circumstances, I advise the following method : The patient 
should be asked to state his chief symptoms and his reasons for seeking aid, 
and while doing this we should note his personal peculiarities. After the 
patient has related what, in his judgment, is most important, the physician 
should take the reins in hand, for he has already obtained a guide to the 
special questions which he wishes to ask. 

We first endeavor to ascertain what diseases, particularly nervous or mental 



HISTORY 131 

affections, have prevailed among the ascendants, descendants, or the collateral 
members of his family. Of non-nervous affections, we must make particular 
inquiries concerning gout, because (aside from other reasons) the success of 
suitable treatment often favors the fact that the gouty diathesis plays a certain 
role in many functional nervous diseases, especially in migraine. In these 
patients a history of tuberculosis in the family is not very important; much 
more so is the question of syphilis in the parents. But only in exceptional 
cases, especially when the patients are children accompanied by their parents, 
will we obtain to this question a definite answer, either affirmative or negative. 

According to recent investigations (Peiper) the blood relationship of 
parents appears to be of less significance in the history of nervous diseases 
than was formerly assumed. 

Inquiries concerning alcoholism in the parents had best be reserved until 
we ask the same questions concerning the patient. 

We must enter somewhat more minutely into the question of the possible 
occurrence of mental or nervous diseases in the family of the patient, of pe- 
culiarities, idiosyncrasies, etc. Here we generally learn but little, for either 
the patients are ignorant and have not noticed these things in their relatives 
or they conceal their knowledge from the physician — especially of psychoses — 
from the fear that he may regard them also as mentally unsound. Perhaps, 
too, they may in this respect unconsciously deceive themselves. 

For instance, the pathologic importance of a case of suicide in the family 
is rarely recognized, or if recognized the occurrence is almost invariably 
concealed. Severe and persistent hysteria in a member of the family is always 
referred to as " a chronic weakness," epilepsy of near relatives is stubbornly 
denied, and psychopathic conditions, especially depression, are represented 
as the normal expression of "much sorrow" or "much care 1 and trouble" 
and the like. In all examinations of this kind we must therefore attempt 
to secure accurate reports, because, as I have already stated, questions in 
regard to the health of the family, no matter how urgently pressed, are an- 
swered in the negative; hence, as a rule, it will not require much time to 
complete the first part of the history. 

Subsequently, in the personal history of the patient, we keep the reins 
in our own hands, and I advise the following method : Eequest the patient 
to answer precisely the following questions: (1) Is the disease for which 
you consult me the first that you hare had. or have you ever before been 
seriously ill? If the second part of the question is answered in the affirm- 
ative we go on : (2) Of what nature were the diseases from which you suffered? 
Have you ever suffered from a disease resembling the present one? 

The answers to these two questions, which should always be asked first, 
form two fundamental props in the structure of our history. The interme- 
diate framework can be readily filled in subsequently as we must now consider 
how to put the special questions we desire to ask, and how much time we 
have left in which to complete the history. 

We should now seek for missing links in a certain chronological order, 
i. e., ask first whether the birth of the patient was normal, whether he had 
infantile diseases and particularly so-called teething spasms, whether the 
patient suffered from enuresis for any considerable time, whether grinding 
of the teeth in sleep and terrifying dreams had been observed during childhood, 



132 GENERAL NEUROLOGICAL DIAGNOSIS 

when puberty began, and what occurred during the years of puberty. In 
women it will frequently be found that the first menstrual period was accom- 
panied by transitory nervous symptoms, which not rarely form a miniature 
picture of the psychosis or neuropsychosis which developed in later years. 

The period of time following early youth when a definite occupation is 
chosen, or at least is prepared for, will indicate the mental development of the 
patient, beginning with his school years. But in the history the development 
of the intellectual side of the mind is even more important than that of the 
emotional side and of the psychical sphere; for these the school years afford 
a very fine reagent. Neurasthenics and hypochondriacs often tell us that, 
although they were always well in school, they " always kept to themselves " 
and did not make friends ; epileptics state that when in school they were often 
" very confused " (petit mal) or that they were remarkably stubborn and 
obtuse. The relatives of hysterical girls sometimes tell us that these girls in 
their school years often had fantastic ideas, that they were deceitful (pseudo- 
logia phantastica), and that even during these early years erotic and sexual 
manifestations, etc., became prominent. All of these factors must be consid- 
ered in intricate cases. 

Next we take up the occupation and its possible deleterious effects. Work- 
ers in poisons or metals (lead, brass), workmen who are continuously exposed 
to great heat, and those who perform extraordinarily severe bodily labor must 
be carefully examined. We must take note of those occupations which are 
combined with great responsibility, as well as those in which a very strict 
discipline is enforced (policemen), also that of teachers (music teachers), and 
all occupations relating to the Bourse (brokers), etc. In all of these, the 
injurious consequences are of psychical nature. Even the layman knows that 
these deleterious effects, especially if continuous or accumulative, are exceed- 
ingly important etiologically and in the history. Of all psychical emotions 
care and sorrow are most frequently noted. 

Supplementary knowledge of the period of adolescence in the male is 
sometimes afforded by his service in the military. Frequent punishments 
during this time, misbehavior in an active or passive sense, lead us to con- 
sider the existence of certain forms of imbecility as well as other psychical 
affections. 

These questions and their answers give us some idea of the patient's health 
up to the time when the disease appeared for which he consults the physician. 
Now the patient can no longer be restrained from a minute report of his 
present condition, which he will give, as a rule, in a haphazard way, and 
overload it with minor details. Even in this case he should be allowed to 
make his report, and should be interrupted only to suppress etiologic con- 
siderations, etc., which he is apt to include. 

The patient should then be asked the following questions : 

(a) Whether there have been prodromes; (b) whether the disease appeared 
gradually or suddenly; (c) whether the condition has remained the same from 
the onset of the affection, or not. If, either from lack of intelligence and 
education, or because of mental depression, excitement, or fear, or for any 
other reason, the patient does not comprehend these questions, the physician 
himself had better take the matter in hand, and put a few concise questions 
to the patient which can be answered briefly and to the point. 



HISTORY 133 

I recommend this method — unpleasant as it often is for some patients — 
for many reasons. Almost always it quickly gives us an idea of the develop- 
ment and the previous course of the disease. 

Before proceeding to specialize the symptoms of the patient, which we 
have recognized from his report, and to inquire into his present condition 
(status prcesens), three exceedingly important points must be investigated 
in the history of every patient with nervous disease: Trauma, alcohol, and 
the sexual relations, including a possible syphilitic infection. 

Of these three points the patient will usually — at least when this is positive 
— mention trauma voluntarily; for trauma is often of exaggerated importance 
in the opinion of the layman. I do not mean to state that it is unimportant 
for the physician to know that such a thing has occurred; on the contrary, 
we know that many neuroses, particularly hysteria, neurasthenia, and hypo- 
chondriasis, may follow injuries or even mere psychical trauma. In organic 
nervous affections trauma is of Less significance, although according to recent 
experience this condition appeal's to have been under-estimated. Hence we 
should never fail to inquire into trauma, even though other etiologic factors 
may be reported by the patient. On the contrary, when, besides trauma, 
another cause, such as chronic metallic poisoning, hereditary predisposition, 
alcoholism, or the like i- present, trauma pathogcnetieally plays an especially 
important role. 

A second point always to be investigated is alcoholism. If the patient who 
consults us is a travelling salesman for a wine house, a bartender, a waiter, 
a driver, or follows any other occupation in which, according to experience, 
the habit of drinking is almosl unavoidable, or if the patient's face reveals 
the well-known signs of chronic alcoholism, the question of his daily con- 
sumption of alcohol will soon be settled. 

We often obtain a history from our patients of the better class without 
effort. It is sometimes very difficult to induce the working classes, especially 
women, to acknowledge the abuse of alcohol, but here the difficulty may be 
bridged over, especially for the workman, if the physician remarks that, with 
such arduous labor he probably has to refresh himself often by a draught, or 
he is often forced by association with his fellow- to drink in the course of 
his occupation. If we show that we realize the conditions, we can often arrive 
at the truth. 

Women often regard any question concerning the use of alcohol as an 
insult. Therefore this subject must be led up to by tactful questions, such 
as asking whether the patient uses much tea or coffee, and then, perhaps as a 
supplement, whether wines or spirits are used in addition, and finally the 
question concerning alcohol may be very delicately put. The symptoms of 
many women patients whom we suspect of alcoholism — pain, attacks of weak- 
ness, fainting and the like — pave the way for us to ask whether they resort 
to some form of alcohol to relieve these conditions, and as soon as alcohol 
has been mentioned we may make more minute inquiries. 

After settling these questions, we must consider specific infection and the 
sexual relations. In men this usually causes little embarrassment, but we 
should not shrink from putting our questions in detail, i. e., we should not 
simply ask whether a patient has had syphilis, but whether he has had a chancre, 
eruptions upon the skin, etc. If we merely inquire about " syphilis," many 



134 GENERAL NEUROLOGICAL DIAGNOSIS 

patients who have suffered from simple gonorrhea will answer the question 
in the affirmative. With married women we may inquire concerning abortions, 
and on questioning the possible syphilis of the husband, we may obtain a clue 
to specific infection. Sensible women do not object to the direct question, 
whether or not they have been infected by their husbands. It is naturally a 
very delicate matter to make inquiries of a young girl concerning syphilitic 
infection, and unless we have powerful reasons for suspecting this infection, 
it is wiser not to allude to it. Of course, if unmarried women directly or 
indirectly admit sexual intercourse, we do not hesitate to inquire into this 
subject. 

Just as in the case of syphilis, we must individualize in questioning patients 
as to important sexual relations. In men we may inquire openly as to mastur- 
bation ; in girls and women whom we suspect of onanism, it is well delicately 
to veil this question. For example, we may inquire whether there is not some- 
times an itching of the body, particularly of the genitalia, which becomes so 
distressing that it leads to scratching and rubbing. Patients are often willing 
to acknowledge the habit when contracted under a legitimate motive. Some- 
times, however, we meet with very sensible and intelligent patients — and a 
case of this kind shows how much more easily a physician can manage educated 
patients than others — who spontaneously report, either negatively or affirma- 
tively, in regard to masturbation. It is singular, although readily understood, 
that male masturbators very often are more likely to conceal this evil habit 
from the physician than females. 

Masturbation in many cases is of less etiologic than symptomatologic 
importance ; for onanism is frequently not a cause — as the layman is inclined 
to believe — but a symptom of disease. For this reason it is of little signifi- 
cance if — as often occurs — we elicit no history of masturbation. In those 
cases in which it is of practical or therapeutic importance for the physician 
to know whether the patient is addicted to masturbation or not, he generally 
obtains this knowledge during the course of the treatment from the patient's 
spontaneous answers to his questions. When the patient fails to divulge this 
habit, although it is of scientific interest yet it is usually of little practical 
importance, particularly in the treatment. For, in this case, the patient does 
not appear to occupy himself — his negative condition shows this — in a path- 
ologico-hypochondriacal manner with the sexual sphere. Were this otherwise, 
conventional considerations and the restrictions consequent upon admitting 
onanism would soon be overcome by his psychical depression. 

Before leaving this subject I must add that, in discussing these conditions 
with the patient, we must be careful not to moralize or use expressions which 
indicate a wish to exercise censorship. Therefore, such expressions as " evil," 
"bad habit," "pollution," etc., are not to be employed, but we should appar- 
ently consider the habit as a light matter, treating the condition en bagatelle. 
This applies also to other sexual conditions, sexual abstinence, sexual abuse, 
etc., which must be discussed with the patient. To me there appears to be 
no doubt that sexual abstinence in patients sexually competent, particularly 
if not voluntary but enforced, may have a deleterious effect (perhaps chiefly 
psychical). On the other hand, too frequent cohabitation and the so-called 
congressus interruptus must be considered to have a somatic, injurious effect. 
All of the sexual conditions which here come under consideration are probably 



THE PRESENT CONDITION 135 

functional diseases, usually of neurasthenic or hypochondriacal nature. I have 
with reason discussed the details of these sexual conditions which the history 
discloses, for they are of decisive importance, and demand our deepest scientific 
interest in the complete understanding of numerous pathologic conditions. 

My chief reason for devoting so much time to the sexual relations is the 
perhaps greatly exaggerated importance which many nervous patients them- 
selves attach to these conditions, and on consulting their physicians they are 
likely to detail these. Many nervous patients do not regard themselves as 
correctly appreciated by the physician unless satisfied that he has investigated 
and correctly estimated everything connected with their disease, and has thor- 
oughly discussed with them their sexual relations and sexual cares. 

THE PRESENT CONDITION (STATUS PRjESENS) 

GENERAL CONSIDERATIONS 

(Complaints, general impressions, various types, stigmata of degeneration^ conspicu- 
ous family resemblance of features in neuropathic patients, facial expression, psychical 
condition. ) 

In determining the status prcesens we again permit the patient to detail 
his symptoms and complaint*, but here also we must individualize. If he 
complains of pain we ask whether this is persistent or appears in paroxysms, 
whether it is lightning-like, lancinating, or boring, whether there are free 
intervals (root pain), whether it always implicates the same area, etc. Minute 
questioning of this kind is especially necessary in headache. Insomnia and 
similar symptoms must also be carefully investigated, for instance, whether 
the patient soon falls asleep, whether sleep is frequently interrupted during 
the night, whether it takes a long time for the patient to get to sleep, and 
whether his sleep is sound. Often it is only as the examination progresses 
that we are able definitely to understand the complaints of the patient and 
to form a true conception of his condition. 

In gauging the actual objective condition, we must first consider in a 
psychic and somatic respect the totality of the impression which the patient 
gives us. This total impression naturally embraces many individual points. 
The most important of these are the nutrition, the muscles and the bony 
structure, the carriage, the facial expression, the color of the face, the nature 
of the patient's movements, the character of the speech, the mental condition, 
and the intellection, etc. When we combine these individual points, we have 
a certain general impression of the patient. But only to a certain degree; for 
the general impression is conveyed not so much by the total of the previously 
mentioned observations, but by faint characteristics difficult to apprehend and 
very difficult to define. This will be readily understood after we have exam- 
ined a considerable number of nervous patients. TVe often have a general 
impression of the patient who consults us, but we cannot consider him as 
representing a definite group of patients. This total impression, in itself, 
need convey no special diagnostic indication. Thus, in the course of time, a 
series of typical cases, usually of functional nervous disease, impresses itself 
upon the mind of the physician, so that at the first glance he readily recognizes 
the hypochondriac who, with " scientific " exactness and aided by written 



136 GENERAL NEUROLOGICAL DIAGNOSIS 

reports, rears the structure of his condition stone upon stone so that it fur- 
nishes a flawless mosaic of his disease ; we recognize the overworked, care-worn, 
unsteady neurasthenic, and in contrast to him the often stout, ruddy-cheeked, 
and plethoric angio-neurotic, then the " spiritual," pale neuropathic who al- 
ways appears to be suffering. The bony, thick-set epileptic with his stupid 
look and clumsy movements, the tall, thin, pale tabetic with his slight urinifer- 
ous odor, the helpless paralytic who at once awakens our sympathy, the pale, 
choreic child with its fine rococo face, the quarrelsome and disagreeable victim 
of accident who seeks redress, these are a few of the types from the clientele 
of the neurologist which give characteristic " total impressions." The general 
impression immediately conveyed to us by these types does not relieve us from 
the obligation to investigate for certain individual points often found in 
nervous patients, and known as stigmata of degeneration. Among these stig- 
mata are the abrupt, narrow, and high hard palate, the irregular conformation 
of the teeth, the thick lips, the adherent lobes of the ear, the poor develop- 
ment and slight individuality of these lobes, the retreating forehead, and 
other irregularities of the face or skull; we must also bear in mind the exist- 
ence of embryonal anomalies of development, such as hare-lip, superfluous 
fingers and toes, malformations of the genitalia, anomalies of the skin, such 
as ichthyosis, abnormal pigmentation and many other things. I desire to 
call attention to two peculiarities that I have relatively often found in neuro- 
pathies, namely, medullary nerve fibers in the eye-ground and a striking 
similarity in the formation of the face of blood relatives. 

I have so frequently noticed among neuropathies a resemblance between 
mother and child that I am inclined to regard this as more than a mere 
coincidence. 

The neurologist, more than any other specialist, must direct his attention 
to the face and its expression. 

The contraction of certain muscles of the face, above all, of the frontalis, 
as well as the implication or non-implication of the muscles of the face in 
active conversation, or their appearance during emotion, is conspicuous. In 
some diseases the expression of the face is so typical that we can from this at 
once make a diagnosis ; for example, in paralysis agitans, in brain tumor, and 
in Graves' disease, etc. 

From what has been stated, the physician will have had sufficient oppor- 
tunity during the examination of the patient to form an opinion as to his 
mental and psychical condition, his manner of speech, whether he speaks 
spontaneously or only after questioning, whether conversation is ready and 
loud or whether he speaks in a low, depressed tone, whether he is quiet or 
excited, whether he at once understands and correctly answers — these fact? 
in combination will enable us to form some opinion in regard to the psychical 
and intellectual functions. Should we still be in doubt as to the intellectual 
integrity of the patient, we may give him easy problems in arithmetic, or ask 
him to relate some details of his occupation, may test his memory for remote 
and recent events, etc. If we suspect hallucinations and similar conditions 
these must be investigated. The preceding will by no means acquaint us with 
the complete psychical status, but it exceeds the limits of pure neurological 
diagnosis as well as the limits of our theme. 



THE PRESENT CONDITION 137 



THE SKULL AND THE VERTEBRAL COLUMN 

(Form and size of the head, cicatrices upon the skin, osseous cicatrices, depressions 
of bone, elevations of bone of periostitic nature, thickening and gelatinous composition 
of the scalp in tumors, etc., bruit de pot fele, murmurs, sensitiveness to pressure of the 
nerve trunks, position of the spinous processes, movability of the vertebral column, 
manner of testing, examination and suspension, sensitiveness of the vertebrae to palpa- 
tion, Rontgen photography, lumbar puncture, cytodiagnosis. ) 

On account of the importance of the nervous central organ to the entire 
organism special attention must be given to it in examination. 

Marked deviations of the head from the normal in size or shape are at 
once obvious. A diminutive skull and a conspicuously large one are often 
found in idiocy, imbecility, and congenital nervous anomalies. Rickets and 
brain tumors often produce great enlargement of the skull. 

In the diagnosis as well as treatment it should be borne in mind that an 
abnormally large skull sometimes indicates hydrocephalus during infancy. 
Quincke lias proven that enlargement of the skull occurs in this way in some 
persons, occasionally also from trauma, or in consequence of some infectious 
febrile disease, and may indicate the renewed development of meningitis serosa. 

An abnormally long, or flat, or abnormally round skull often has no path- 
ologic significance. But, in contrast with this, the so-called bird-skull develops 
only in those who are psychically below par. 

On inspecting and palpating the skull the first point to be noted is whether 
there are cicatrices on the scalp. The patients often have forgotten the exist- 
ence of these, nevertheless, they are occasionally — for example, in epilepsy — 
of the greatest importance. 

We must differentiate sharply between physiologic depressions in the skull 
and the protrusion of the bones, between pathologic traumatic depressions and 
thickening of the bones. 

A physiologic thickening of the bone which is often misunderstood is found 
at the upper insertion of the tabular part of the occipital bone. Certain 
thickenings of the cranial bones, often insignificant or but slightly prominent, 
which may sometimes be pressed in by the firm finger, are of great pathologic- 
importance. They are chiefly situated in the region of the parietal tuberosity 
and are extremely painful. These thickenings are due to syphilitic periostitis 
which causes the acute headache from which these patients suffer. The swell- 
ings are often overlooked; therefore, in all cases of intense headache the 
physician must search for a periostitis. In rare cases this periostitis to which 
the headache is due is so slight externally as to produce no palpable swelling. 
One or several circumscribed areas intensely sensitive to pressure render the 
diagnosis of specific periostitis certain. 

A more diffuse, instead of circumscribed, area of the skull, much less 
intensely painful upon pressure, is occasionally observed in rheumatic affections 
of the galea, in brain tumor, and in some cases of hysteria. 

In palpating the skull of patients with brain tumor I have often observed 
a symptom which I will now describe, a quite noticeable thickening of the 
scalp, especially of the anterior portions. While the scalp of the normal person 
is thin, tense, elastic, and almost adherent to the bone, in these patients on 
palpation it seems as though a serous infiltration, a loose and soft pliable 



138 GENERAL NEUROLOGICAL DIAGNOSIS 

mass, were under the fingers. The surface of the bone can, therefore, no 
longer be felt by the palpating finger. 

In patients with tumor there is another symptom, which was first de- 
scribed by English authors. Upon lightly tapping the skull in some cases of 
brain tumor or serous meningitis with the fingers or with the metallic side 
of a percussion hammer, a peculiar succussion sound is heard, such as is 
evoked by tapping a window-pane which is a little loose or upon tapping a 
cracked pot (bruit de pot fele). This sound may also be elicited under normal 
conditions in early childhood, and is then of no diagnostic value. 

An attempt has been made to auscultate the skull, but with little success. 
In neoplasms and other organic diseases within the skull, we sometimes hear 
murmurs with the stethoscope. The differentiation of pathologic murmurs 
in the head from normal ones requires practice. 

Among the symptoms still to be considered in examining the skull is sensi- 
tiveness to pressure over the trunks of the fifth and the occipital nerves. 

The branches of the fifth nerve are susceptible to the pressure of the finger 
at the supraorbital notch, at the infraorbital foramen, and the mandibular 
foramen; the occipital nerve responds at a point between the mastoid process 
and the processes of the vertebrae. The points enumerated by no means repre- 
sent the only pressure points of these nerves in neuralgia, nor are they always 
sensitive to pressure in actual neuralgia. But the extreme tenderness of these 
points which causes the patient to cry aloud, in contrast to the painlessness 
of the surrounding tissues upon pressure, is to be regarded as true pressure 
pain of the nerve. Anemics, hysterics and neurasthenics will often tell us 
that a nerve trunk is sensitive to pressure, but actual tenderness on pressure 
can be assumed only when the previously mentioned condition is observed. 

The examination of the vertebral column which encloses the spinal central 
organ is less difficult than that of the skull. Here we must observe the follow- 
ing points : The normal position of the spinous processes and the vertebra? 
belonging to them, the normal S -shaped curvature of the entire column, and 
its normal passive movaMHty. The lateral or posterior deviation of one or 
several spinous processes is not readily overlooked. This, however, is the 
case in the absence of lumbar lordosis which sometimes occurs — apparently 
from reflex causes — in tumors of the vertebra? and spinal cord, and even in 
hysterical conditions. Its possibility must also be borne in mind in the various 
forms of arthritic disease of the vertebral column and in ankylosis of the 
vertebral column. 

We test the movability of the vertebral column by having the patient bend 
forward, backward and laterally as far as possible, and also turn the trunk 
laterally while the feet are close together. Next we ascertain whether sudden 
and brisk movements produce pain in the vertebral column. To demonstrate 
this we strike the patient a light blow upon the parietal region — protected by 
the hand — or the patient may be caused to bend laterally by a slight blow 
against the shoulder, or he is merely shaken. If he is sitting, the thorax 
may be tightly grasped, and the thoracic vertebra? be moved laterally against 
the remaining parts of the vertebral column. Finally, in doubtful cases, 
suspension may be practised, or, while the patient is in the dorsal decubitus, 
his legs may be pulled, or while fixed at the hip-joint they may be suddenly 
moved. I detail these manipulations because, when there are complaints of 



THE PRESENT CONDITION 139 

pain in the back, it is often of the greatest assistance in the treatment to know 
whether a small pathologic focus, a tumor or the like, is hidden in the vertebral 
column. In such cases we should never be content with a purely neurologic 
diagnosis. 

The spinous processes show a tenderness upon pressure and palpation which 
is generally of little diagnostic importance. 

This is found in neurasthenics and hysterics (spinal irritation) often in 
varying intensity and with a varying localization as to height. It usually 
does not reach a high degree. If, however, one or two of the spinous processes 
are invariably painful on pressure and palpation, there is probably a vertebral 
affection. Here, as in all other doubtful cases, a Bontgen photograph is 
decisive. 

As a supplement to examination of the vertebral column we must briefly 
describe lumbar puncture and cytodiagnosis. Lumbar puncture, introduced 
by Quincke, 1 enables us to determine the composition of the cerebrospinal 
fluid. Cytodiagnosis, a process originated by French investigators, particu- 
larly by Eaymond's pupils, represents merely the special examination of the 
cerebrospinal fluid, namely, the determination of its cellular contents. 

Lumbar puncture may be performed in the sitting posture, but, according 
to the inventor of the method, is best done in the left lateral position. In 
this position we introduce a slender trocar in the middle line, or somewhat lat- 
erally, between the spinous processes of the third and fourth or the fourth and 
fifth lumbar vertebrae If, following the first attempt, there is no lessening of 
the resistance of the soft parts to show thai we have penetrated the dural sac, we 
may probe with the tip of the needle and move it about laterally until it enters 
the dural sac. Then the mandrin of the trocar is withdrawn, a piece is added 
which connects it with a rubber tube, the other end being attached to the 
cannula, and, elevating the tube, we determine the pressure of the fluid, that 
is, its height in the tube. After this has been done a few cubic centimeters 
of the fluid are allowed to exude. The amount of fluid withdrawn must not 
be large (upon the average from 20 to 30 c.c). for the pressure of the cerebro- 
spinal fluid should not be too rapidly nor too greatly reduced. After evacu- 
ating the fluid, the needle is rapidly withdrawn and the small cutaneous 
wound closed with cotton and collodion. To diminish the pain from the 
insertion of the needle, the skin should be anesthetized with ether spray or 
ethyl chlorid. For the technical and other details of this slight operation 
the reader is referred to Quincke's article on lumbar puncture. 

Different instruments have been suggested for lumbar puncture. The 
small instrument advised by Quincke has always given me good results. The 
operation in itself is generally quite insignificant, and only particularly 
unfavorable circumstances can lead to disagreeable results. On the other 
hand, I cannot deny that the performance of lumbar puncture in nervous 
patients, who are often hyperesthetic and excitable, particularly in private 
practice, is not always such a simple matter as it is represented to be by 
most authors who report instances from their clinics and large hospitals. The 
most important data concerning the cerebrospinal fluid are as follows: 

The specific gravity of the normal fluid varies between 1.006 and 1.007. 

i See this volume. 



140 GENERAL NEUROLOGICAL DIAGNOSIS 

According to Quincke, the pressure within the clural cavity amounts to 40 
mm. and 130 mm. water. The pressure may fall with a decrease in blood 
pressure, and (this is more often the case) it may rise with any increase 
of brain pressure (tumor, hemorrhage, etc.). The normal cerebrospinal fluid 
has the transparency of water, is colorless, contains a little albumin (0.2-0.5 
per 1,000), and sometimes a substance showing reducing properties with a 
test for sugar. 

A distinct amount of albumin, one per 1,000 or more, as well as a turbid 
composition of the fluid, indicate recent inflammatory processes in the brain 
and spinal cord; but a slight amount of albumin and a colorless, clear fluid 
favor chronic hydrocephalus. Changes in the specific gravity have a similar 
diagnostic significance. Blood is normally present only in slight amounts, 
provided it originates from the wound made by the introduction of the needle. 
In hemorrhages of the brain or spinal cord there is often a marked admix- 
ture of blood. 

Among the cellular constituents of the normal fluid obtained by puncture 
are some scant lymphocytes (upon an enlargement of from 400 to 500 about 
3 to 4 in the field). The examination and diagnostic utilization of these 
cellular constituents of the cerebrospinal fluid forms the so-called cytodiag- 
nosis. 

For cytodiagnosis we require only 3 or 4 c.c. of the cerebrospinal fluid. 
Puncture, therefore, if only for the cytodiagnosis, may be performed with a 
very fine needle. According to French authors the puncture fluid should 
first be centrifugated for 20 minutes with a rapidity of 3,000 revolutions per 
minute. On removing the fluid, the sediment is microscopically examined. 
As previously stated, there are normally but a few small mononuclear lympho- 
cytes in the fluid. The presence of polynuclear and large mononuclear leuko- 
cytes favors acute inflammatory processes in the region of the brain or spinal 
cord, or in their membranes. 

If the number of small lymphocytes exceeds the minimal of 3 or 4 to the 
field, their presence is of much greater practical and diagnostic significance 
than the presence of polynuclear and large mononuclear leukocytes in the 
residue. For, according to Raymond's adherents, which other authors con- 
firm, in all syphilitic and metasyphilitic affections of the central nervous 
system, therefore, in cerebrospinal syphilis, specific hemiplegia, specific mye- 
litis, progressive paralysis, tabes dorsalis, etc., there is a conspicuous lympho- 
cytosis of the liquor cerebrospinalis so that 20 or more cells appear in a 
single field of the microscope. Of course, the appearance of lymphocytes in 
the cerebrospinal fluid is not absolute proof of the previously mentioned con- 
ditions, for in addition to syphilitic and metasyphilitic affections of the 
central nervous system they have also been found in quite unlike pathologic 
processes, in multiple sclerosis, in herpes zoster, in recent hemiplegia, etc. 
And, what unfortunately almost vitiates this sign, lymphocytosis of the cere- 
brospinal fluid has also been found in secondary syphilis without any impli- 
cation of the nervous system. In spite of these disadvantages, lumbar punc- 
ture, according to our present knowledge, is a most valuable diagnostic aid. 



THE PRESENT CONDITION 141 



VASOMOTOR AND TROPHIC DISTURBANCES 

Of the nature and occurrence of most trophic and vasomotor symptoms 
we still know very little. They occur in organic affections of the brain, the 
spinal cord and the nerves, as well as in various functional diseases, and 
may implicate all the tissues of the body. 

TROPHIC DISTURBANCES OF THE SKIN AXD CUTANEOUS 

STRUCTURES 

(Color of the skin, temperature of the skin, "blushing," flushing of the face, urti- 
caria faetitia. edema, hemorrhage, disturbances in the secretion of saliva and of sweat, 
myxedema, scleroderma, glossy skin, ichthyosis, loss of pigment of the skin, herpes, 
neurofibromata, cutaneous ulcerations, perforating ulcer of the foot (mal perforant), 
changes in the hair, changes in the nails, changes in the teeth.) 

We are most familiar with the vasomotor and trophic disturbances of the 
skin, for these may be recognized by mere inspection, palpation being rarely 
necessary. 

The temperature and color of the skin, its thickness, its greater or less 
infiltration, and its reaction to mechanical irritation, are observed when in- 
vestigating for vasomotor disturbances. Under certain circumstances (hys- 
teria and neurasthenia) the skin of almost the entire body assumes a bluish, 
marbled appearance. In certain peripheral areas, such as the region of the knee, 
the hand or the foot, a distinctly bluish discoloration is observable, the area 
is often cool and sometimes moist. In prolonged paralyses — no matter of 
what origin — this cyanosis with coolness is observed in some of the extrem- 
ities, or in parts of them. Here circulatory disturbances due to the absence 
of normal muscle and tissue movements is apparently the cause. In other 
cases (Raynaud's disease) certain portions of the body, usually the fingers 
or toes, are paroxysmally very pale and cool, and contain a minimal amount 
of blood; or, on the contrary, they are exceedingly red, swollen and painful 
(erythromelalgia) . Under still other circumstances (paralysis agitans, the 
climacterium), peculiar fluxions appear with sudden, profuse, arterial disten- 
tion and flushing of the larger vascular areas of the head or trunk, and disap- 
pear in one or two minutes. 

Erythrophobia is usually found in congenitally neurotic individuals and 
the like with compulsory ideas (idee fixe). It is a reddening of the face on 
the slightest possible irritation (usually psvehieal), of which ordinarily no 
account is made, and simultaneously with this (on account of the pathologic 
psychical disposition) there is a distressing fear of blushing. Periodic or 
permanent unilateral flushing of the face indicates a unilateral disease of 
the sympathetic nerve. The sudden reddening or pallor of epileptics, with or 
after attacks, and the unilateral flushing or pallor of the fact in migraine, 
need only passing mention. 

The physician, while examining a hysteric or neurasthenic and while the 
patient is undressing, is often astonished to see. directly under his eyes, flam- 
ing red flakes develop which are confluent, large or small in size, but not ele- 
vated. These are usually situated upon the neck or chest (vasomotor collar) 
and disappear just as rapidly as they appear, usually after the patient has 



142 GENERAL NEUROLOGICAL DIAGNOSIS 

become composed. Moreover, we find that in these patients copious perspira- 
tion is very readily induced, and also the appearance of an urticaria-like erup- 
tion after the ingestion of certain foods (such as strawberries, lobsters, etc.), 
as well as the so-called urticaria factitia {dermographia). The latter symp- 
tom may be evoked by drawing lines upon the skin with a hard, pointed object. 
After about a minute these lines appear as red or whitish bands elevated above 
the level of the skin, and they may persist for hours. In urticaria factitia 
we are apparently dealing with small serous exudations from the vessels into 
the tissue, such as occur in inflammations. 

True edema will also be found, sometimes with cyanosis and extremely 
cold skin — that due to nervous disturbance. The first form occurs in some 
varieties of neuritis, in cerebral hemiplegia (particularly of the middle brain), 
and in similar affections. Hysterical edema, that attributable to an organic 
nervous cause, is sometimes accompanied by other symptoms of stasis; some- 
times, however, it exists as a simple, or hard, or doughy serous exudation 
under the normally colored and normally warm skin (the so-called flying 
edema of hysteria). In addition to the purely nervous edema, stasis edema 
due to purely mechanical causes also occurs, for instance, edema of the eye- 
lids in tumors of the brain, etc. 

Vasomotor disturbances combined with hemorrhages into the tissue are 
exceedingly rare. Relatively the most common of these are mucous membrane 
hemorrhages from the genital canal or the respiratory tract in hysterical 
women. The small hemorrhages into the conjunctivae or the skin of the 
face observed in epileptics after an attack are purely mechanical hemorrhages 
produced by venous stasis during the seizure. 

Irregularities in the secretion of saliva and sweat belong to the vasomotor 
disturbances which occur in the structures of the skin. Of course, the drib- 
bling of saliva in paralysis of the facial nerve, in bulbar paralysis, and sim- 
ilar clinical conditions is primarily due to an inhibition of the normal closure 
of the lips or the impossibility of swallowing the saliva. In certain spasmodic 
conditions of epileptic or organic nature, the production of saliva appears to 
be actually increased. 

Disturbances in the secretion of sweat will usually be found in the form 
of hyperhidrosis, that is, hypersecretion. Too slight secretion of sweat and 
also of the sebaceous glands sometimes appears, the skin being remarkably 
dry, scaly, and fissured (occasionally in neuritis and in disease of the gray 
substance of the cord). The diminution of the secretion of sweat is often 
noticed by the patient ; for example, the tabetic refers to the absence of sweat- 
ing as an important etiologic factor in the development of his disease. 

Hyperhidrosis may be distributed over the entire body, especially in func- 
tional affections, hysteria, neurasthenia, Graves' disease, etc., and is frequently 
accompanied by dermographia or nervous and cardiac conditions, feelings of 
anxiety, etc. Limited to certain parts of the body or to members, hyper- 
hidrosis is also seen in neuritis, in spinal, and in cerebral processes. It is an 
interesting fact that sweating may occasionally be abnormally induced by 
certain sensory irritations. For instance, I recall a patient with syringo- 
myelia who, a few minutes after eating a spiced pickled herring, showed 
active unilateral sweating of the face. 

Trophic disturbances of the shin occur partly in connection with the 



THE PRESENT CONDITION 143 

previously described vasomotor symptoms, but also without these. They are 
much rarer in purely functional diseases than in the organic. 

The normal thickness and texture, the amount of fatty tissue, etc., of 
the skin, is frequently altered by the influence of trophic disturbances. Under 
certain circumstances, fat increases to an enormous extent throughout the skin 
of the entire body, and pain and other general symptoms appear (Dercum's 
disease, adiposis dolorosa), or the skin of the whole body becomes peculiarly 
thickened and swollen and resembles edema, but does not pit in the manner 
characteristic of edema (myxedema) . In patients suffering from brain pres- 
sure with chronic serous meningitis I have several times seen a skin condi- 
tion of the face, the trunk, and especially of the hands, which closely resem- 
bled myxedema, but I am unable to explain these changes in the skin (Neurol. 
Centralbl, 1900, p. 548). 

Another change which also implicates wide areas of the skin is found in 
scleroderma. Here extensive areas of the face, the arms, the back, etc., have 
a peculiarly tense, glistening, and usually very red appearance, and almost 
convey the impression of being firm, smooth, surface cicatrices. Here the 
skin cannot be picked up from the underlying structures; on the contrary 
it appears to adhere to them. If we succeed in pinching up a small fold of 
skin, it feels between the fingers like a coarse, tough, and inelastic piece of 
leather. 

A similar condition — but limited to much smaller areas of the skin — is 
the so-called dermatomyositis, an extraordinarily rare affection. 

If, in the previously mentioned conditions, the skin increases in thickness 
and toughness, the opposite, atrophy of the skin and of all its structures in 
consequence of nervous disturbances, occurs under the following circumstances. 
The best known form of atrophy of the skin is the so-called " glossy skin." 
In this the skin becomes so thin that by lightly passing the finger over it 
numerous small folds are formed; it also glistens, is usually of a yellowish 
color, and easily desquamates. This pathologic glossy skin closely resembles 
the physiologic atrophic skin of the aged. Glossy skin occurs in neuritis 
— more rarely in spinal processes. 

An abnormal and intense dryness and desquamation of the skin is seen in 
ichthyosis. In neuropathic subjects this disease is frequently seen upon the 
skin of the leg, particularly in the region of the tendo Achillis, hence this 
symptom may perhaps be regarded as one of the so-called stigmata. Some- 
times the scaliness and desquamation are not great enough to warrant its 
being called ichthyosis, but, nevertheless, it is sufficient to attract the atten- 
tion of the examiner and to be regarded as pathologic. The patient usually 
observes this condition in himself or in other members of his family before his 
attention is called to it by the physician. 

In old cerebral paralyses, especially those of the upper extremity, another 
peculiar change in the skin is often observed. The pigment in the skin of 
the hand and fingers is decreased. The skin has a rosy, delicate, and child- 
like appearance, and is smoother and softer than on the normal side. 

This cutaneous change is often combined with general atrophy of the 
bones of the finger and a peculiar tapering of the fingers. A decrease in the 
pigmentation of the skin is also seen in neuritic processes. 

I must still mention a few trophic disturbances of the skin which pro- 
11 



144 GENERAL NEUROLOGICAL DIAGNOSIS 

duce conspicuous changes, namely, an eruption of herpetic vesicles, tumor- 
like neoplasms of the skin (neurofibromata), and ulceration of the skin. 
The eruption of herpes accompanied or preceded by severe pain, and the 
vesicles which are usually arranged in rows and which at first contain serous 
fluid (subsequently pus if in the area of a peripheral nerve) form such a 
characteristic phenomenon that it cannot fail to be recognized. The previously 
mentioned circumstances and the occasional occurrence of hypesthesia and 
hyperesthesia in the area of the affected nerves favor the presence of neuritic 
processes. As a rule, herpes occurs in the course of the intercostal nerves (a 
possible disease of the vertebra should then be considered!) but it also occurs 
in the area of other peripheral nerves. 

[It is now pretty definitely proven that true herpes zoster is invariably 
associated with an inflammatory and hemorrhagic process in the posterior 
root ganglion. Irritation of the posterior roots or the posterior ganglia in 
spondylitis, or involvement of the intercostal nerve, may be attended by an 
herpetiform eruption, which, in the case of the peripheral nerve, is limited 
to the corresponding cutaneous distribution of the nerve involved. — Ed.] 

N euro fibromata of the skin are small nodules under and in the skin which 
are sensitive to pressure, usually very movable and only discovered when the 
patient consults the physician for other nervous disturbances than the condi- 
tion of the skin. Very often, simultaneously with these relatively harmless 
tumors of the skin, neurofibromata of the same nature form on the anterior 
and posterior roots of the spinal cord, and by destruction of the root fibers 
produce severe pathologic conditions. If, on the other hand, we first discover 
the neurofibromata of the skin, we must be exceedingly careful to investigate 
the other nervous symptoms of the patient. 

The most conspicuous trophic alteration of the skin is caused by 
ulcers. 

If no other reason for cutaneous ulceration can be found, trophic nervous 
disturbance must be thought of, particularly that due to disease of the spinal 
cord, to tabes dorsalis (mal perforant), or to syringomyelia. In the last 
named disease the condition becomes complicated, for, besides genuine ulcers 
not due to trauma, others appear in consequence of a traumatic defect in the 
skin and sensory disturbance, and the defective tendency of the tissue to heal 
produces true ulcerations. 

Similar to the ulcerations of syringomyelia is a rare variety of ulcers seen 
in so-called Raynaud's disease, in diabetes mellitus, and in similar affections. 
Here there is a local tissue necrosis due to the spasmodic closure of vessels, 
and a succeeding ulceration at the point of necrosis. 

Almost as common as trophic disturbances of the skin is an implication 
of tissues adjacent to the skin, the hair, the nails, etc. Falling out of the 
hair, alopecia, sometimes depends upon functional nervous disturbances; in 
myxedema there is almost invariably a marked alopecia of all of the hairy 
parts of the body including the eyebrows. A local falling out of the hair 
occurs in neuritis, although an abnormal growth of hair in the diseased area 
is more common. That alopecia is often limited to the areas of peripheral 
nerves is shown by the most familiar form of baldness. In the baldness of a 
person who, fortunately, cannot be regarded as a nervous patient, only the 
areas of the fifth nerve are usually bald, the hair remaining in the region 



THE PRESENT CONDITION 145 

of the occipital nerve. General hirsuties (hypertrichosis) over the entire 
body is looked upon as a stigma of degeneration. 

Grayness of the hair — either general or partial — in neuralgia or other 
severe psychical conditions is well known to the layman. It is less well known 
that, in mental diseases and some other nervous disturbances, similar changes 
may occasionally take place in the color of the hair and its entire composi- 
tion. Cases are reported in which blond, straight hair became red and curly 
or dark hair turned white. The texture of the individual hairs is changed 
only in the last mentioned case. In myxedema, also, the hair which remains 
upon the head becomes stiff, ropy and completely loses its normal luster. In 
neuritic hirsuties, the hairs upon the surface of the body are abnormally long 
and thick. 

The symptoms presented by the nails of the fingers and toes after trophic 
disturbances are quite uniform : Abnormally rapid growth, the normal form 
of the nail changing to a marked curve, the appearance of longitudinal and 
transverse ridges, a friable condition, and the gradual loss of the entire nail. 
These changes are usually attributed to the peripheral neuron, and preemi- 
nently to the spinal processes (tabes, syringomyelia). They are also occa- 
sionally found in cerebral hemiplegia. Some of these changes in the nail 
occur in rare cases of functional nervous diseases, such as prolonged paralysis 
or contractures of the individual members. 

A symptom seldom observed is formed by the numerous small white spots 
which in neurotics often cover the entire nail from above downward, and 
which may appear and disappear periodically. A smaller number of these 
spots may be observed in almost any person. 

In examining the teeth we must look for well known defects in growth 
and development which occur in rachitis and congenital syphilis. In neuro- 
logic diagnosis the finding of the characteristic notched semilunar and exca- 
vated free border with the peculiar oblique end makes us suspect hereditary 
syphilis (Hutchinson's teeth). Regularity or irregularity of the teeth is also 
to be observed. Superfluous teeth projecting from the surface of the jaw are 
not rarely found in nervous patients and neurotics. In trophic disturbances 
in the region of the fifth nerve, for example in tabes dorsalis, the teeth some- 
times fall out singly or several at a time, without much pain. On the other 
hand, in neuralgia of the fifth nerve, there is neither trophic nor other recog- 
nizable change in the teeth, hence I must issue a warning against unnecessary 
extraction of teeth. 

TROPHIC DISTURBANCES OF THE MUSCLES 

(Hypertrophy, atrophy, atrophy in the course of the cranial and the spinal nerves, 
hypertrophy, pseudo-hypertrophy, occurrence of pseudo-hypertrophy, atrophy, simple 
atrophy, degenerative atrophy.) 

Trophic disturbances of the muscles appear as hypertrophy and atrophy, 
and are recognizable by inspection, by palpation, and by mensuration. The 
practised eye soon notices — particularly on comparison with the normal side — 
any abnormal depression or prominence of the skin from atrophy or hyper- 
trophy of the muscular tissue beneath. 

The results of mere inspection are confirmed by palpation and mensura- 



146 GENERAL NEUROLOGICAL DIAGNOSIS 

tion. With a marked development of fat, inspection will not suffice, and 
the latter methods become necessary. It is quite comprehensible that trophic 
changes of the deeply situated muscles cannot be recognized at all, and in 
other muscles can only be determined with difficulty. 

The muscular areas most likely to be involved in trophic changes are, 
as a rule, the following : Along the course of the cranial nerves, the face, the 
tongue, and the lips. Atrophy of the temporal muscles is recognizable by the 
marked depression in the temporal region; that of the masseter muscle on 
palpation of the sunken cheek ; atrophy of the muscles on one side of the face 
(facial hemiatrophy) by the singular appearance of the diseased side of the 
face which sometimes almost resembles the bare skull. 

Only an extreme atrophy of the lips is noticeable at the first glance. 
Usually we must take the lips between the thumb and index finger in order 
to detect this condition. But in old persons the lips are physiologically very 
thin. 

Atrophy of the tongue is readily determined, whether it be unilateral or 
bilateral. The tongue appears withered, contracted, and dry; its surface is 
fissured. The extremely small size of the organ, especially upon protrusion, 
is pathologic and unmistakable, and in peculiar contrast with the normally 
opened mouth. 

In the neck and nape of the neck atrophy of the sternocleidomastoid and 
of the upper trapezius is most easily recognized. These muscles are readily 
palpated, and they are so necessary for the proper contour of the neck that 
their atrophied condition at once attracts attention. Moreover, they are 
among the muscles which not rarely undergo true hypertrophy (for exam- 
ple, in patients with tic). In such cases, the sternocleidomastoid, even during 
rest, stands out prominently as a massive rope under the skin. 

Hypertrophy of the upper portion of the trapezius, recognizable by its 
enormous prominence, which, even under normal conditions, may be distinct, 
should only be diagnosticated when the shoulder on the involved side is not 
higher during rest, for only thus can a permanent contraction of the trapezius 
(which also causes the muscle to appear more massive) be excluded. 

The depression of the supraspinatus fossa is readily determined, but it 
is not so easy to decide whether atrophy of the trapezius or the supraspinatus 
is the cause. For this purpose a test of the function and an electric exami- 
nation are necessary. 

In atrophy of the deltoid, the shoulder viewed anteriorly has an angular 
appearance (from the prominence of the acromion process and the external 
end of the clavicle). Posteriorly the stiff and angular external contour of the 
upper arm is conspicuous. Turning to the muscles of the trunk, the deter- 
mination of atrophy of the middle and lower portion of the trapezius will 
occasion no perplexity. Atrophy of the teres major and the rhomb oideus 
is much more difficult to perceive; to determine it in the first mentioned 
muscle we must when palpating compare the normal with the affected side; 
an atrophy of the rhomboideus while the trapezius which covers it remains 
intact, also of the latissimus dorsi, can be assumed only when the palpating 
finger feels the ribs more distinctly than usual under the superficial muscle 
masses. 

Atrophy or aplasia of the pectoralis major on the anterior side of the 



THE PRESENT CONDITION 147 

trunk is revealed by the presence of an abnormal and ugly groove below the 
clavicle. Atrophy of the serratus anticus is readily shown by the absence of 
the characteristic zigzag of the muscle in the anterior axillary line. The 
intercostal muscles, on the contrary, lie so deep that atrophy cannot be de- 
tected. In very thin persons only is it possible to recognize atrophy of the 
abdominal muscles. 

Atrophy of the muscles of the extremities is readily perceptible because 
these muscles, even during rest, are more susceptible to inspection and palpa- 
tion than the musculature of the trunk, and, moreover, the many changes 
in the position of the extremities give us more opportunities thoroughly to 
examine a definite muscle. 

The scope of this article does not permit us to investigate all of the mus- 
cles of the extremities in regard to atrophy. In those cases in which mere 
palpation and inspection are insufficient to enable us to decide whether or 
not the muscle is atrophic, a knowledge of the special physiology of the 
muscles will make the diagnosis positive. Many common forms of muscular 
atrophy may be recognized at the first glance. Among these is atrophy of the 
biceps, of the extensors of the hand and fingers, of the interossei and the 
muscles of the ball of the hand, of the quadriceps, the tibialis anticus y the 
muscles of the calf, etc. In examining for muscular atrophy in the extrem- 
ities it should be borne in mind that the left upper arm and forearm are 
usually 1 cm. less in circumference than the right [provided the person is 
right-handed. — J. L. S.]. 

Besides atrophy of the muscles I have spoken of hypertrophy of the mus- 
cles as also a trophic disturbance. True hypertrophy of the contractile mus- 
cular substance is rare. It is occasionally observed in the affected members 
in long standing cases of clonic spasm, for example, post-hemiplegic chorea 
and the like. Pseudo-hypertrophy of the muscle, in which the entire vol- 
ume of the muscle appears enlarged from the fact that fat has been depos- 
ited between the individual muscle fibers, is much more frequent, and appears 
chiefly as dystrophia muscularis progressiva. Here the vulnerable points for 
pseudo-hypertrophy are the triceps, the infraspinatus, the muscles of the 
calf, the quadriceps and the gluteal muscles. If these muscles appear to 
be abnormally large, pseudo-hypertrophy is very probable. This becomes 
certain if, on palpating the muscle when at rest as well as during exten- 
sion, we feel a soft and easily movable mass instead of a firm and tense 
tissue. 

While pseudo-hypertrophy of the muscles occurs, as a rule, in only one 
disease, progressive muscular dystrophy, atrophy of the muscle is noted in 
various pathologic processes. Disease of any part of the great cortico- 
muscular tract may be accompanied by muscular atrophy. Two special vari- 
eties of atrophy produced by the arrest of function or by the intense effect of 
irritants upon the muscle are so-called inactivity and pressure atrophy. Inac- 
tivity atrophy appears to be closely related to reflex atrophy. In this condi- 
tion the extensor muscles in the neighborhood of a diseased joint undergo 
wasting — the quadriceps in disease of the knee-joint, the triceps in affections 
of the elbow — probably because the centripetal irritation of the sensory nerves 
and reflex collaterals no longer takes place normally but is pathologically con- 
veyed into the anterior horn cells. Our diagnosis primarily depends less 



148 GENERAL NEUROLOGICAL DIAGNOSIS 

upon classifying the atrophy than upon deciding whether it is " simple " or 
" degenerative." 

The division of atrophy into these two groups is pathologico-anatomical. 
In simple atrophy there is only a quantitative diminution of the muscular 
mass, in the degenerative form the muscular substance has undergone chem- 
ical change, " degeneration." Mere inspection and palpation will never reveal 
with certainty which form is present. This can only be ascertained by electric 
examination. In simple atrophy there is either no, or merely a quantitative, 
change in contractility; in degenerative atrophy there is a qualitative change 
in contractility which manifests itself by the sluggishness of the contraction, 
and by the sluggish arrest of the electrical contraction of the muscles caused 
by direct galvanic muscular irritation. Normally, all electrical muscular 
contractions take place with lightning-like rapidity. 

The diagnostic significance of the character of the atrophy can be appre- 
ciated when we consider that degenerative atrophy is produced only by in- 
juries or disease of the motor nerves or the anterior horns of the gray matter 
of the spinal cord. When we differentiate degenerative atrophy we have a 
valuable aid for the topical diagnosis. In the cases in which, for any reason, 
an electrical examination is impossible, mechanical muscular irritation is 
advisable (by means of a sharp blow of the pleximeter upon the muscle mass) ; 
with degenerative atrophy this will produce a sluggish contraction. In my 
experience mechanical irritation is a very poor substitute for electrical irri- 
tation, and frequently leaves us in doubt, because in some muscles, even nor- 
mally, neither the contraction nor its cessation is so rapid as the contraction 
produced by electricity. 

Of the relation of atrophy of the muscles to paralysis I shall say merely 
a few words. In certain cases, atrophy of a muscle too deep to be susceptible 
to examination may be concluded from the existence of paralysis even when 
it cannot be decided by palpation or inspection. 

TROPHIC DISTURBANCES OF THE BONES AND JOINTS 

(Hypoplasia of the bones, osseous atrophy in neuritis and after injury, changes in 
the bones and joints in spinal diseases, partial giant growth, acromegalia, intermittent 
dropsy, hysterical affections of a joint.) 

The growth of bone depends upon nervous influences. In infantile spinal 
paralysis the growth of the bones in the affected extremity does not keep pace 
with that of the normal side. This is often the case in the cerebral paralyses 
of children, although it is not so conspicuous as in poliomyelitis. Some- 
times only a unilateral, extremely small shoulder-blade reveals that the affected 
person has had infantile cerebral paralysis. In this case we are not dealing 
with atrophy of the bone, but with hypoplasia, a retardation of the growth 
of bone. True atrophy of the bone is usually observed in diseases of the 
peripheral nerves, with or without preceding injury. For instance, in poly- 
neuritis the peculiarly thin and tapering phalanges of the fingers are re- 
markable; but atrophy of the bones of the extremities is more frequently ob- 
served in patients who have suffered from accidents. The latter phenomenon 
also indicates long existing hemiplegia or similar processes. Other trophic 



THE PRESENT CONDITION 149 

disturbances of the bones which are often combined with similar disturbances 
of the joints are less common than atrophy. I refer chiefly to a peculiar 
change in the consistence of the bony tissue in which the normal firmness 
is lost and a friability of the bones appears. The joints show a similar vul- 
nerability as well as a tendency to synovial thickening, deposits, and enlarge- 
ment. Usually after slight trauma — unnoticed because of the coexisting 
analgesia — or even without a recognizable lesion, in these cases (syringo- 
myelia, tabes dorsalis or similar processes) fractures of the bone, deposits, and 
thickening of the joints, subluxations and similar conditions appear. 

Hypertrophic, as well as atrophic, disturbances take place in the bones. 
So-called partial giantism, in which a foot, a hand, etc., is abnormally large 
at birth, is formed during infancy or continues to develop, belongs to this 
category. The abnormal processes of growth which take place in acromegalia 
within a relatively short time (within months) and which affect particularly 
the hands, the feet, and the lower jaw, are generally more important. The 
soft parts also are involved in this abnormal growth of the bone. After seeing 
and investigating one typical case of this kind, a diagnosis can be made at 
the first glance. 

Before dismissing the trophic disturbances of the joints I must call atten- 
tion to an exceedingly rare affection, namely, so-called intermittent dropsy 
of the joint. This usually attacks the knee-joint without recognizable cause, 
there is neither pain nor reddening of the skin, it persists a few days and then 
disappears, to recur after the lapse of some time. The nature of this disease 
is absolutely unknown ; it is presumed to be due to an affection of the nervous 
system. 

I advise the greatest caution in making a diagnosis of hysterical swelling 
of the joints, an affection which has been described by some authors. 

MOTILITY 

(Muscle tonus, significance of the anterior horn cells of the spinal cord as the motor 
power station, increase and decrease of muscular tonus, the effect of other areas of the 
central nervous system upon the power station, the muscles of the body in a position 
of rest, the muscles innervated by the cranial nerves in a position of rest, the muscles 
of the face and eyes in rest, the muscles of the upper and lower extremity in rest, 
position of the pelvis, shoulder-blade, and muscles of the back- Passive movements, 
spastic conditions, recognition of slight spastic muscle resistance, active and passive 
contractures, clinical difference between active and passive contractures, influence of 
the psychical condition upon muscular tension, exclusion of this influence during exam- 
ination, the value in diagnosis of determining the spastic character of a paralysis, 
relation of spastic to flaccid paralysis, occurrence of spastic conditions and contrac- 
tures, Kernig's sign, contractures of paralysis agitans. Hypotonia, cause of hypotonia, 
clinical symptoms of hypotonia. Active movements, the test of active movements in 
the region of the cranial nerves, nerves of the forehead, and of the facial branches 
of the mouth, voluntary and emotional movements of the nerves of the face, paralysis, 
contracture, and atrophy of the tongue, paralysis in the bulbar sphere, in the muscles 
of mastication and of the larynx, dysarthritic speech disturbances, stuttering, hyster- 
ical stuttering, other hysterical speech disturbances, scanning speech, syllable stum- 
bling, muscles of the nape of the neck and of the back, movements of the arm and 
hand, adynamia, myasthenic conditions, movements of the leg and of the foot, psychical 
and resistance movements, psychical and hysterical paralysis, abasia, weakness of the 
grasp and flexure of the hip in neurasthenia, occurrence of organic paresis and 
>is.) 



150 GENERAL NEUROLOGICAL DIAGNOSIS 

The investigation of the motor sphere is of course not limited to the 
examination of voluntary movements. But, turning to these, we must first 
consider the tension of the muscle and combined with this the rest of indi- 
vidual portions of the body as well as their passive movability. Then only 
will we able to test voluntary movement, the coarse development of power 
and the faculty of muscular coordination. 

Subsequently we shall consider involuntary movements and the principal 
types of spasm. 

In the normal individual while awake the muscles of the body are not 
fully relaxed but are in a certain state of contraction. This slight degree of 
contraction is called the tonus of the muscle. Clinical researches make it ap- 
pear highly probable that the normal tonus is not an autochthonous muscular 
phenomenon, but is produced from the spinal cord by centripetal sensory irri- 
tation, and is, therefore, a reflex condition. To understand this perfectly it 
is necessary for us to analyze — although in a diagrammatic manner — the 
mechanism of the motor central organs which is revealed as a clinical postulate. 

Here, as the first and most important requisite, we must adhere to the 
fact that the sole and actual source of energy for muscular contractions, i. e., 
the motor power station, is situated in the large anterior horn cells of the 
spinal cord or in analogous cells of the medulla oblongata and the mid-brain. 
The physiological intactness of these cells is an absolute prerequisite for the 
normal function of the entire motor apparatus. Clinical facts lead us to 
the conclusion that the anterior horn cells form merely a power station, or 
a power station with potential energy, i. e., with the ability to discharge 
power; that, therefore, some causative factor is necessary to change the ability 
to furnish force into an actual discharge of force. Under normal con- 
ditions there is always — even in rest — such an active cause; namely, the 
thousands of irritations ceaselessly passing from the periphery to the 
spinal cord, and produced by the circulation of the blood, by the chemical 
processes in the tissues, by the action of the tissues upon each other, by the 
effect of air and clothing, or by the subcutaneous tissues, etc., upon the skin. 
All of these irritations transmitted to the spinal cord by way of the reflex 
collaterals through the posterior roots — to say nothing of the fact that they 
are transmitted also as conscious sensations by way of the long spinal cord 
tracts to the cortex. of the cerebrum — are conveyed to the gray substance of 
the spinal cord. Here — reaching the terminal arborizations of the reflex 
collaterals — they surround the motor anterior horn cells and cause (like all 
sensory irritations) a motor correlation ; namely, a continuous slight dis- 
charge of motor energy. We can, with great probability, assume the muscle 
tonus to be the effect of this continuous output of power from the power 
station of the anterior horn cells. The motor power station in the spinal cord 
is like a fire which is kept aglow by the constant addition of fuel from the 
periphery, and only flames up brightly when it is caused to do so — as we 
shall soon see — by other agency. 

The intimate relation of the muscular tonus to sensory stimulation is 
shown, among other ways, by the fact that a pathological increase of tonus is 
diminished by lessening the peripheral irritation (absolute rest, warm bath, 
and the like) and, inversely, it may be increased by intensifying these irri- 
tations (cold and pain). 



THE PRESENT CONDITION 151 

The reason for assuming a muscular tonus to be the explanation of patho- 
logically increased or decreased muscular tension will subsequently be apparent. 

Sensory impressions of the periphery are not the only agents by means 
of which the anterior horn cells are caused to give off force. On the con- 
trary, the most distant areas of the nervous system may produce an effect 
upon the motor power station and a consequent discharge of force: For in- 
stance, the cerebellum, the basal ganglia, the trunk of the brain, and, last 
but not least, the cortex of the cerebrum. The action of the cortex of the 
cerebrum, the central convolutions, upon the motor power station of the 
spinal cord is by far the most important; it represents what has been called 
the conscious movement of the will. This simple relation between the brain 
and spinal cord in regard to the motor function must be borne in mind for 
it forms the base of our later descriptions. Therefore, in the anterior horn 
cells is located the true, active, and absolutely essential power station; the 
central convolutions act upon this power station only in that, at a given 
moment, the source of power sends an impulse of contraction into certain 
muscles. This cerebral effect upon the anterior horn cells is chiefly observed 
in what is known as the pyramidal tract, perhaps to a slight extent also in 
other tracts. 

The relations of the cerebrum to the motor power station are not con- 
fined alone to the function just described. On the contrary, physiologic and 
clinical facts make it obvious that the cerebrum (perhaps also other parts of 
the brain) has a second action, opposed to the prior one, which prevents the 
too great development of power in the anterior horn cells. This inhibitive 
regulation of the continuous discharge of power, which according to our 
view is developed and promoted by an afflux of sensory irritations, must be 
assumed for muscular tonus from the obvious fact that in pathologic cases 
in which the power station, in itself completely intact, is disconnected from 
the brain, there is a decided increase of muscular tonus. We know less of 
the nature of the negative " inhibitive " influence of the cerebrum upon the 
anterior horn cells than of the positive effect of the cerebrum upon the spinal 
cord, but it is not impossible that the apparent inhibitive action is caused 
by other powerful influences. 

After this deviation we return to the examination of the patient, and, 
in observing the symptoms of motility, the previously sketched brief diagram 
must be kept in mind. 

The first test of motility is the position of the muscles of the face when 
at rest. Here we examine the wrinkles of the forehead, the position of the 
eyebrows and upper eyelid, the width of the apertures, the frequency with 
which the lids close, the position of the bulbi, the angle of the mouth, the 
nasolabial fold, and the width of the nasal opening. In these tests we must 
always compare the right with the left side. The majority of deviations 
from the normal in the position of these structures depend upon disturbances 
in the innervation of the facial nerve. Besides this nerve we must also con- 
sider the nerves of various muscles: Particularly the oculomotor \for the 
position of the eyes [strabismus] and for the position of the upper lid in rest 
[ptosis]) as well as the sympathetic nerve. The latter controls the position 
of the eye in the frontal plane by means of the smooth muscle of Mtiller which 
extends through the orbit, and at the same time regulates the normal opening 
12 



152 GENERAL NEUROLOGICAL DIAGNOSIS 

of the lids. Protrusion of the eyeball is most prominent in Graves' disease, 
and is here attributed to a disturbance in the innervation of the sympathetic. 
Decrease of the palpebral fissure, a sign of paresis of certain branches of the 
sympathetic, is found in many spinal processes, particularly in syringomyelia, 
as well as in diseases of the brachial plexus, and is sometimes congenital. 
Mere gaping of the palpebral fissure without protrusion of the ej^eball indi- 
cates paresis of the orbicularis oculi supplied by the facial nerve. An ab- 
normal dilatation of the palpebral fissure (usually hysterical contracture) 
is in rare cases produced by contraction of the facial muscles or of the 
levator palpebrse. We must observe whether the pupils are circular and of 
equal size. All of their other relations will be described under the reflex 
symptoms. 

We will now turn to the examination of the extremities in rest. 

In the upper extremities we should observe whether the head of the 
humerus is sufficiently firm in the socket (supraspinatus), and whether the 
arms, relaxed and drooping, are in the correct position midway between pro- 
nation and supination. When the objective findings are scant, a slight devia- 
tion from this normal middle position is often the only distinct sign revealed 
to the practised eye, and warns us to test minutely the rotators of the arm. 
We should also note whether the basal phalanges of the fingers are normal 
during rest, i. e., slightly flexed, or whether there is a tendency to extension 
and hyperextension (weakness of the interossei). Of course a conspicuous 
change of this kind will no more be overlooked than a conspicuous flexion 
of the hand in radial paralysis. But a slight degree of flexion of the hand 
may readily escape the eye. 

The examination of the lower extremities during rest must be made with 
the patient in the horizontal position; here also a slight outward or inward 
rotation of the leg may elude observation. 

On the other hand, a slight drooping of the tip of the foot, such as occurs 
in paresis of the extensors of the foot and in kypotonia of the muscles of the 
lower leg, will be very noticeable. The heavy mass of the quadriceps upon 
the upper thigh will sometimes appear as if it had slid off externally, thus 
making the outline of the femur more prominent than normal, or we may 
note that the entire posterior surface of the leg — even the popliteal space — 
seems to be adherent to the underlying bone. These are simultaneously indi- 
cations of flaccid ity of the muscles, or hypotonia. 

Another symptom the observation of which is of decided significance, is 
the elasticity of certain muscles, for instance, a permanent hyperextension 
of the great toe. This is found with especial frequency in some forms of 
ataxia and in spastic conditions. 

The examination of the erect trunk during rest gives us opportunity to 
investigate further what was observed in the legs when in a horizontal posi- 
tion, particularly to estimate the condition of the gluteal muscles. The posi- 
tion and course of the so-called gluteal fold at either side forms a criterion 
for the examination of the gluteal muscles. 

In the trunk we should observe any lateral deviation, any curvature of the 
vertebral column, any inclination of the pelvis and possible asymmetry of the 
iliac crests. In some cases of paresis with pain in one leg, the diseased side 
of the pelvis is always held higher, even during rest. An abnormal position 



THE PRESENT CONDITION 153 

of the pelvis — too great an inclination in the horizontal plane — is most likely 
to be found in persons suffering from muscular dystrophy. 

The position of the shoulder-blades, their distance from the median line, 
the normal, nearly vertical, lines of their internal borders, and their normal 
position upon the thorax, should be noted in every case. 

Abnormal tension of the muscles which connect the pelvis with the verte- 
bral column is usually of a reflex nature. This is most distinctly pointed out 
by the unilateral or bilateral prominence of the rigid, contracted erector trunci. 

After observing the condition of the members and the trunk during rest, 
we must proceed a step further, and investigate the passive motility, i. e., the 
possibility of excursions of the different parts of the body toward each other 
— for this test the patient must entirely relax. Normally, passive movement 
of the extremities is not perfectly free. On the contrary, when an arm or a 
leg of a normal person whose attention has been diverted is flexed or extended, 
we note a certain resistance — I might say a smooth and gliding one — like that 
felt on moving an oiled piston to and fro in a syringe. This physiologic 
muscle resistance is due to the tonus of the muscles, the nature of which 
I have attempted to explain. If, therefore, the tonicity of the muscles is 
increased, passive movement is the more difficult. If the tonus is decreased, 
passive movement is facilitated. Of course, we presuppose that the joints 
are perfectly free. Increase of muscular tonus, according to its degree, is 
designated as muscular spasm, hypertonia, or a spastic condition of the mus- 
culature. Decrease of muscular tonus is called hypotonia. 

A moderate spastic condition is present if, on attempting passive move- 
ments — sometimes in flexion, more often in extension — we meet with a resist- 
ance which must be overcome. 

If the spasm is slight, we should not continue the passive movements 
slowly and cautiously, but, on the contrary, Ave should grasp the extremity, 
either the hand or the foot, hold it for a moment motionless, and then sud- 
denly and completely flex or extend the entire extremity. I think it impor- 
tant that the member to be tested should be grasped distally from its most 
peripheral chief joint; for we thus test all the muscles simultaneously, and 
all possible muscular spasms are sure to be evoked. The suddenness of passive 
movements, or, what is also advisable, the sudden repetition of a movement 
interrupted in about one-half of its excursion, stimulates the sensory muscle 
and cutaneous nerves, increases the tonus of the anterior horn cells, hence 
increases the possible slight muscular spasm, for all influences and irritations 
which increase or diminish the normal tonus have the same conspicuous action 
upon spastic muscles. 

Extreme degrees of muscular spasm are characterized by the flexion of 
extremities or portions of the body during the most severe muscular 
spasms. 

These extreme degrees of spasticity of the muscles are true contractures, 
i. e., active, muscular contractures, either because the anterior horn cells in 
the spinal cord are in a state of pathologically increased irritability and activ- 
ity, or because the sensory irritations conveyed to the anterior horn cells are 
abnormally intense and continuous (reflex trismus in neuralgia of the fifth 
nerve), or because there is no central inhibition from the cerebral cortex 
(contracture in cerebral hemiplegia), or because power is constantly sent 



154 GENERAL NEUROLOGICAL DIAGNOSIS 

from the cerebrum to the power station (hysterical contracture), or because, 
for any other reason, there is an immoderate discharge of power from the 
power station in the spinal cord, and this is so strong that the muscle con- 
stantly contracted produces a distinctly visible motor effect, i. e., is actively 
contracted. The genesis of contracture and the accentuation of its activity 
are therefore important, because the same coarse mechanical effect produced 
by active contracture may also be attained by other processes. 

For instance, a muscle may gradually be so shortened by cicatricial con- 
traction, by inflammatory processes, by the merely secondary contraction and 
retraction of its connective tissue, that a coarse motor effect is produced. 
Such a shortening, i. e., from retraction and contraction of the connective 
tissue contained therein, occurs in every muscle when the points of insertion 
are brought closer together, for example, during enforced rest of a joint or in 
consequence of disease of the joint. 

Such a contraction develops in all muscles which are permanently in a 
hypertonic spastic condition. Hence, in addition to the originally purely 
active contracture there is, after a certain time, also a passive contracture 
which gradually controls the former until at last the originally active con- 
tracture is no longer apparent clinically, and only the passive contracture can 
be demonstrated. Similar to the passive contracture produced in permanently 
spastic muscles, which has been described, there is another passive contracture. 
This develops in the antagonists of these paralyzed muscles when, in any area 
of the body, only one definite muscle group, or an important locomotor mus- 
cle, is paralyzed or arrested in function. As, owing to the absence of oppos- 
ing muscles, a certain hypertonia apparently exists in these antagonists, this 
passive contracture which develops closely resembles the one previously men- 
tioned. 

I have attempted to define clearly the nature of the spastic condition and 
contracture. I have already stated how a muscular spasm is clinically re- 
vealed. Now we must discuss the important and often difficult problem of 
the differentiation of active and passive contracture. 

The first and chief difference between these forms is that, on attempting 
passive movements, active contracture shows itself by an elastic, but not quite 
uniform, resistance, while passive contracture is revealed by a uniform, con- 
stant and rigid resistance which resembles that noted on attempting to bend 
a piece of wire or lead pipe. 

That a sudden, energetic attempt at passive movement will increase a 
spasm and the resulting active contracture, I have already stated. Passive 
contracture, on the other hand, is the same whether we begin passive move- 
ment cautiously and gradually or suddenly and energetically. Spasm, active 
contracture, shows great variations : Early in the morning after a quiet sleep 
and when the patient is otherwise rested, during and after a warm bath, when 
there is a general corporeal and mental feeling of well-being and contentment, 
active contracture diminishes in intensity. In the evening, however, and if 
the patient is tired, is cold, if he must make any mental or bodily exertion, 
if there is pain, active contracture becomes more marked. Passive contracture 
is in no way affected by these circumstances. 

A capricious condition, rapidly changing, is therefore significant of active, 
the absolutely unchangeable and rigid one, of passive contracture. 



THE PRESENT CONDITION 155 

Besides passive movement, we have other aids to the recognition of the 
nature of a contracture; first, the condition of the tendon reflexes. 

The importance of testing the reflexes we shall later more minutely dis- 
cuss. According to the scheme which I proposed for the examination of the 
muscle tonus we may readily comprehend that when there is a pathologically 
increased muscle tonus, or spasm, the tendon reflexes are increased. If, in 
a subsequent examination, we systematically test the reflexes, and find a very 
active tendon reflex or even clonus in an extremity which has resisted passive 
movement, this unquestionably indicates active muscular contracture. 

It is also to be noted that an increase of reflexes actually present may be 
difficult to demonstrate because of old, long-standing active contractures. The 
secondary passive contractures which gradually result in these conditions fix 
the joints and the affected muscles in such a manner that reflex mechanism 
— at least in its motor portion — is hindered. 

Like the tendon reflexes, the cutaneous reflexes serve to indicate the 
nature of the spasm. The cutaneous and periosteal reflexes are usually in- 
creased in active contracture and, as a rule, the toe reflex is pathologically 
changed. 

In the discussion of spasm and contracture we have so far omitted a point 
of vital importance; namely, the influence of the attention of the patient 
in producing passive movement by muscular tension. When undertaking 
passive movements we usually tell the patient " to relax entirely " or " to let 
the member hang loose." 

We rarely succeed with this request. As a rule — although unconsciously 
— the patient does exactly the contrary ; the muscles to which his attention 
is attracted are only made more tense the more he attempts to relax them, 
and this may make a physician not skilled in these maneuvers believe that 
muscular spasms are present. 

Another source of error springs from the fact that as soon as the physician 
attempts passive movements in an extremity the patient — again unconsciously 
— actively performs the same movements, and in spite of all urging seems 
unable to refrain from conjoint action without, on the other hand, relapsing 
into a state of muscular tension. 

In all such cases we can only succeed by an actual deflection or diversion 
of his attention into other channels. Hence it is wiser to say nothing to the 
patient about relaxing and the like, to say nothing of the extremity that is to 
be tested; the patient should be engaged in interesting conversation which 
requires no effort on his part, no allusion being made to his disease. In the 
meantime, uniform passive movements should be attempted and, in order 
to further distract the attention of the patient, should be practised in all of 
the extremities. It is advisable to avoid a definite rhythm since a change of 
this rhythm is likely to attract the patient's attention. 

If no satisfactory conclusions can be arrived at, we should undertake very 
thorough and powerful active movements, so as to set all parts of the ex- 
tremity simultaneously into activity, and should then note whether the pa- 
tient's efforts meet with resistance, whether this motion becomes suddenly 
slower or is even arrested, or — and this is a finer reaction — whether he com- 
plains of stiffness and a feeling of tension in any one joint. 

If, notwithstanding these maneuvers, we reach no definite opinion — and 



156 GENERAL NEUROLOGICAL DIAGNOSIS 

this is by no means rare — nothing remains but to reexamine the patient in 
the manner previously described. 

When we have positively demonstrated active contracture, we have an 
important aid for all subsequent diagnostic conclusions. In all those cases 
of paresis or paralysis of the extremity in which spasm or contracture is simul- 
taneously present so that the condition is designated spastic paralysis, the 
pathologic process is located above, i. e., centrally from the motor power sta- 
tion, and in some area of the brain or spinal cord the pyramidal tract must be 
damaged or disturbed. As we have seen, it sometimes happens that the power 
station is no longer regulated by the brain, but responds to the irritations 
brought to it from the periphery with a constant production of power. 

When, however, the pathologic process is situated in the power station 
itself and destroys this, spastic paralysis becomes flaccid paralysis, since all 
the sources of power are destroyed. 

There is, however, as is evident from the scheme proposed, one possibility 
in which, in spite of disease of the power station in the anterior horns, spastic 
paresis develops. This condition arises, in the first place, if the motor anterior 
horn cells are only in part destroyed, and, secondly, if the pyramidal tract 
above this area is simultaneously damaged. In this condition — which we see 
in amyotrophic lateral sclerosis — the damage to the pyramidal tract as well 
as atrophy of the muscles in which a number of anterior horn cells are de- 
stroyed, produces paresis. The simultaneous spasm is due to the fact that, 
owing to the absence of the " inhibitive fibers " of the damaged pyramidal 
tract, the cells still existing in the power station continuously produce force 
which is transmitted to the muscles. 

Spasms and active contractures of the extremities will be found in tumors, 
hemorrhages, sclerosis, etc., in which the processes directly or indirectly 
act upon the motor zone of the brain or the intracerebral course of the pyram- 
idal tract. In these cases the monoplegic or hemiplegic type will predomi- 
nate. In damage of the pyramidal tract within its spinal course, the paraplegic 
spasm will predominate on account of the small anatomical area in- 
volved. It is not surprising that actual spasms and contractures should occur 
in all functional diseases in which we assume a pathologic action of the cere- 
bral cortex — therefore in hysteria and certain psychoses. 

Those active spasms and contractures which appear as persistent, abnormal, 
peripheral sensations and pains in the implicated region or in the region of 
the paresthesia have been touched upon previously in discussing trismus, which 
sometimes accompanies neuralgia of the fifth nerve. In the same group of 
active contractures belong the spasm of the lids in affections of the conjunc- 
tivae, the contracture of the erector trunci in certain cases of pain in the 
back, the contracture of certain muscles of the back in sciatica, probably also 
the so-called Ehret's contracture the distinguishing feature of which is that 
while walking there is constant pain in the foot, and this gradually produces 
a contracture of the tibialis anticus. 

Trismus in neuralgia of the fifth nerve has been attributed to a constant 
discharge of force from the motor power station (in this case the motor nucleus 
of the fifth nerve) and is produced by the excessive irritation of the severe 
pain in the sensory branch of the fifth nerve. For some reasons, however, 
it seems likely — this is particularly true of the similar spasms just mentioned 






THE PRESENT CONDITION 157 

— that it may be due to other influences on the part of the cerebral cortex 
which also produce force (unconscious voluntary innervation). 

Spasms in tetanus must be regarded as active, and most likely due to 
direct toxic influences upon the anterior horn cells. 

Contractures in the flexors of the leg, frequently seen in meningitis 
(Kernig's sign), are peculiar in that they appear only while the patient is in 
the sitting posture, and disappear when in the recumbent position. These con- 
tractions also are active, but we are still ignorant of their origin. 

We note a special and peculiar form of active muscular contraction in 
paralysis agitans. In this disease the muscles of the nape of the neck, of the 
arms, and of the hands are always abnormally hard and tense. 

Although the tendon reflexes — not the cutaneous — are frequently increased 
in paralysis agitans, the tension of the muscles is unquestionably of very dif- 
ferent nature, being less elastic, and more moderate than in spastic hypertonia. 

Hypotonia of the muscles, the opposite of hypertonia, is less difficult to 
differentiate. 

Hypotonia, as the name indicates, consists in a decrease of the normal 
muscle tonus. A smaller current of power is discharged from the spinal 
power station than under normal circumstances. From a purely theoretic 
consideration, a decrease of normal tonus may be produced in as many vari- 
ous ways as increase of tonus. In the genesis of hypotonia, the conditions 
now to be named frequently come under consideration: Absence or defective 
nature of the sensory irritations transmitted by the posterior roots to the 
spinal cord (tabes, other processes in the posterior roots) ; diseases of the 
power station itself and defective supply to the motor nerves (certain spinal 
and neuritic muscular atrophies and "flaccid" paralysis), as well as shock 
of the spinal cord, and diseases of the cerebrum and cerebellum (some forms 
of porencephalia and other extensive cortical and cerebellar diseases). In 
passive movements hypotonia is revealed by the fact that the joints permit a 
great latitude of abnormal movements ; for example, the forearm may be 
passively over-extended, the legs may be drawn apart almost to an angle of 
180°, in flexion of the lower limb the heel may be placed against the tuberosity 
of the ischium, the knee may be over-extended, and the lower extremity ex- 
tended at the knee may be easily and painlessly flexed to a position beyond the 
vertical. Before investigating the abnormal excursions of the joints, it is 
sometimes well to remember that on attempting passive movements an absence 
of resistance will be conspicuous, and while in the normal person we must 
suggest relaxation, in the patient with hypotonia the individual members — 
apparently only subject to weight and without organic connection with the 
whole — readily become flaccid. 

In the legs hypotonia may be easily recognized, so that the condition of 
"the tendon reflexes — these are absent or greatly diminished — is no longer 
necessary for the diagnosis. 

Only after we have exactly informed ourselves as to the passive movability 
should active movements be tested. First, we should ascertain the gross 
power which the individual muscles and muscle groups are able to exert. For 
this purpose we should naturally be more or less familiar with the special 
physiology of the muscles, and here the classic work of Duchenne is still an 
authority, 



158 GENERAL NEUROLOGICAL DIAGNOSIS 

The practical investigations of the chief muscles of the body is best made 
by beginning with the cranial nerves and the head. 

The patient is asked to rotate the eyes upward and downward, to the left 
and to the right. The movements of the eyes must be synergistic. The 
abducens muscle should bring the border of the iris to the external angle of 
the eye. But if the bilateral movement of the abducens should appear to 
be insufficient, we must be cautious in assuming therefore a bilateral paresis 
of the abducens, since fretful and stupid patients, or sometimes those with 
a general neurosis, are from mental weakness incapable of rotating their eyes 
outwardly to the physiologic extreme. 

Slight paresis of the muscles of the eye, especially of both oblique muscles, 
readily escapes detection by this method of examination, and is more readily 
recognized by examining for double vision. 

We must, of course, strictly differentiate between a strabismus due to 
paralysis of the muscles of the eye and a non-paralytic one, so-called concomi- 
tant strabismus. Strabismus may arise, too, from active spasm of individual 
muscles of the eye (meningitic processes, hysteria). 

It must be observed that an incomplete movement of the eye upward is 
sometimes only a seeming one, and is due to a slight drooping of the upper 
eyelid (ptosis). 

The movements of the lids may be studied while examining the muscles 
of the eye. On raising and lowering the plane, the upper eyelid must simul- 
taneously and uniformly follow the movement of the eye so that no white 
streak of sclera is ever visible between the iris and the border of the lid. If 
the upper lid does not follow uniformly but spasmodically, we have the so- 
called " Graefe's sign " which is due to a disturbance of the sympathetic 
innervation of the smooth muscles of the orbit, and occurs in Graves' disease 
without being absolutely pathognomonic of this condition. Drooping of the 
upper lid (ptosis) and inability to raise it sufficiently depends upon disturb- 
ance of the levator palpebral which is supplied by the oculomotor nerve. Par- 
tial paralysis of the levator palpebral is functionally corrected by the contrac- 
tion of the frontal muscle. Hence, in doubtful cases in testing the muscle 
which raises the lid the action of the frontalis must be excluded by fixing the 
skin of the forehead with the finger. 

The impossibility of closing the e}^elids or of lessening their abnormal 
gaping is a sign of paralysis of the upper branches of the facial nerve. Spas- 
modic conditions in the muscles of the lids, as in the muscles of the eye, 
are rare and occur chiefly in hysteria. Tremor and convulsive movements 
of the lower eyelid on attempting to close the lids is usually seen in neuras- 
thenia or other neuroses. In the normal person the eyelids may be so tightly 
closed by the orbicularis muscle which is supplied by the facial nerve that the 
closure can hardly be overcome by the investigator. 

The other muscles supplied by the facial nerve should now be tested; 
the patient is asked to wrinkle the forehead (often this is only possible by 
a synergistic movement in looking upward), to move the nose, to open the 
mouth wide, to close it firmly, to pucker the lips, to move the angle of the 
mouth to the right and to the left. 

To ascertain if there is weakness of the lower branches of the facial nerve, 
those supplying the mouth, we closely examine the branch supplying the 



THE PRESENT CONDITION 159 

forehead, for the recognition of paresis of the lower facial branches often leads 
us to a diagnosis which would otherwise remain obscure. This depends chiefly 
upon the fact that in various cerebral and intracranial processes there is much 
more frequently a paresis of the lower branches of the facial nerve than a 
paresis' of the upper branch. The latter is less likely to be functionally in- 
volved, because, unlike the branch supplying the mouth, it is innervated from 
both hemispheres of the brain. 

Inability to retract the angle of the mouth, due to facial paralysis, often 
appears more distinctly in certain conjoined movements than in merely 
opening the mouth; for instance, when the patient makes firm pressure 
with the hand, or shows the tongue, etc. Care must be exercised to exclude 
asymmetry of the mouth — which is often congenital or may be due to 
defective teeth — so that this be not ascribed to a disturbance of inner- 
vation. 

The angle of the mouth and both sides of the face should be observed 
during lively speech, and especially during laughing. The laughing must be 
spontaneous; if the patient is told — as is often unwisely done — to laugh, the 
movement is in most cases purely voluntary and a mere imitation of emo- 
tional movement. The observation of voluntary emotional (affective) move- 
ments in laughing and crying is therefore important because, in the first place, 
indistinct and scarcely noticeable facial paresis with varied localization be- 
comes most distinct upon affective movements. Here the weakness of one 
facial nerve often shows itself not so much by the total absence or the quan- 
titative diminution of its motor effect as by the fact that the contraction 
of the paretic side appears after a preliminary stage of rest, and after there 
has been a fully developed contraction in the normal state at the conclusion 
or at the height of the expression of emotion, that, therefore, when both sides 
of the face contract, the contraction of the diseased side occurs later (nor- 
mally it should occur at the same time). 

The testing of affective movements is also important because certain con- 
clusions as to localization may be drawn from the irregular action of the mouth 
in voluntary and in emotional movements. The motor innervation of affective 
movement is most likely conveyed from the thalamus region or from the 
basal ganglia by a path independent of the tract for voluntary motion. This 
explains why in some cases voluntary innervation of the angle of the mouth 
may be preserved and the affective movement be absent or only slight (foci 
in the region of the thalamus), and, inversely, why there are cases in which 
voluntary movements are absent but the emotional are retained (certain cases 
of cerebral hemiplegia, infantile pseudo-bulbar paralysis). 

In an examination of the movements dependent upon the facial nerve, 
that of the tongue and palate must be included. 

The tongue must lie still in the mouth, and when at rest must not be ele- 
vated nor deviate to either side. When the tongue is protruded this should 
be done quickly and without tremor, and it should be extended so far that 
the tip will reach at least to the anterior border of the lips, while the raphe 
of the tongue corresponds to the median line of the chin. The middle of the 
chin must be marked with the finger while the tongue is protruded. Not- 
withstanding the absence of actual paresis, some patients, especially hysterics, 
do not protrude the tongue beyond the edge of the teeth, which condition is 



160 GENERAL NEUROLOGICAL DIAGNOSIS 

due to psychical influences, and analogous to the imperfect lateral movements 
of the eyes. 

The patient should be able to move the tongue rapidly to and fro in the 
mouth, to insert it in the spaces between the alveolar processes and the mucous 
membrane, to turn the tip of the tongue upward and downward, and to roll 
it laterally. 

In unilateral paresis of the hypoglossal nerve the tongue during rest devi- 
ates to the side of the non-paralyzed muscle, as the weight of this muscle 
overbalances the weight of the paralyzed one. On protruding it, however, the 
unilaterally paralyzed tongue deviates to the side of the paralysis, because the 
normal muscle, which overbalances, mechanically turns to the side where 
there is least resistance, therefore toward the flaccid paretic half of the 
tongue — and thereby draws the entire anterior half of the tongue with it. 
The mechanical conditions in contracture of one-half of the tongue are 
are in the main the same. Here the normal half of the contracted organ 
is opposite the side mechanically least resistant, therefore the tongue 
when protruded deviates to the non-contracted side. With simple paresis of 
the hypoglossal nerve — no matter of what character — the deviation of the 
tongue may be differentiated from the deviation in the last mentioned hemi- 
spasm of the tongue : First, because the entire tongue never deviates in paral- 
ysis of the hypoglossal nerve but only the tip, and, moreover, it is never so 
pronounced and extreme as in hemispasm. In the latter condition we are 
reminded of the voluntary oblique protrusion of the tongue in grimaces; for 
in this exaggerated way the tongue is often protruded. 

The tongue which protrudes and deviates in consequence of paresis may 
be readily returned to a normal median position; a spastic deviation, how- 
ever, cannot be corrected, but shows great elastic resistance to the finger. 
Touching the protruded tongue, which we are often tempted to do, is apt to 
leave us in doubt; for it is sometimes very difficult to determine from the 
protruded tongue whether that half which has deviated has the normal tonus 
or is abnormally flaccid. The decision of this question is greatly facilitated 
if atrophic conditions are simultaneously present. If both halves of the 
tongue are atrophic, the entire organ appears shrunken, often as if dried up, 
the impression of the teeth is seen on the edges, also small but deep fissures 
and longitudinal grooves. If the atrophy is unilateral, only one-half of the 
tongue has this appearance. The fissures due to atrophy may readily be dif- 
ferentiated from the cicatrices found on the edges of the tongue in epileptics. 
These cicatrices should be carefully looked for in all patients with spasms. 
If found on only one-half of the tongue, they may indicate the unilateral 
nature of the spasms. 

Unilateral paralysis of the tongue does not interfere with the patient's 
eating, and less with his speech than might be supposed — much less than 
unilateral peripheral facial paralysis. Of course, there is always a purely 
subjective limitation in unilateral paresis of the tongue. Bilateral paralysis 
of the tongue naturally causes great disturbance. 

The soft palate should be inspected simultaneously with the tongue. Nor- 
mally both arches of the palate are equally curved, are equally elevated during 
rest, and are uniform. The position of the uvula is of comparatively little 
importance. As a rule, the palate cannot be moved voluntarily. In an exami- 



THE PRESENT CONDITION 161 

nation of patients, therefore, we must cause them to make a movement which 
synergistically raises the arches of the palate. Phonation is suitable for this 
purpose, for example, they may utter the vowel a. If in doing so one side 
of the palate moves less than the other or remains completely immotile, or 
if the palate moves obliquely to one side instead of medially and vertically, 
there is unilateral paresis of the palate — provided no cicatrices or adhesions 
can be found. Unilateral contractures of the palate are extremely rare. In 
bilateral paralysis the entire structure of the soft palate during phonation 
remains completely immotile, or does not extend to the posterior wall of the 
pharynx. 

The movability of the soft palate is also tested by producing the so-called 
palate reflex. This mode of examination for various reasons is not adapted 
to motor testing, as I shall point out later when describing the reflexes. A 
voluntary act of deglutition on the part of the patient is better; normally, 
during this act, the pharyngeal cavity is completely closed by a close approxi- 
mation of the soft palate to the posterior wall of the pharynx. Regurgitation 
of fluid through the nose on voluntary swallowing is proof of the inadequacy 
of this closure and of paresis of the soft palate. 

As several nerves are concerned in the innervation of the soft palate, 
namely, the pneumogastric, the sympathetic, the glosso-pharyngeal and the 
facial (the last by a branch given off in the geniculate ganglion which passes 
into the spheno-palatine ganglion and terminates in the palatine nerve), 
the explanation of paresis of the palate is not always easy. The difficulty is 
enhanced when, owing to the implication of individual nerves which supply 
the velum of the palate, considerable individual variations appear. 

Paralysis and paresis of the tongue and soft palate, like paralysis in the 
course of any other cerebral nerve, may be due to disturbances in any point 
of the motor tract between the cortex of the cerebrum and the muscle. From 
the many possibilities and clinical pictures which result, I should like to 
emphasize one especially typical in which the lips, the pharynx, and the tongue 
are simultaneously and bilaterally paralyzed or paretic. This is the picture 
of so-called bulbar paralysis or paralysis glosso-pharyngo-labialis. In this 
symptom-complex the pathologic focus is situated in the motor power station 
itself, i. e., in the nuclear structures of the bulbus, which are analogous to the 
anterior horn cells. The seat of the focus in this nuclear region therefore 
produces, what I have previously described in the anterior horn cells, a degen- 
erative atrophy of the muscles of the lips and tongue, but this characteristic 
condition is even intensified in so-called bulbar paralysis. 

After investigating the movability of the palate, we turn to the muscles 
of mastication. The muscles which here come into action, the temporal, mas- 
seter and both pterygoid muscles, are supplied by the motor branch of the 
fifth nerve, and are tested by telling the patient to press the lower jaw tightly 
against the upper while the physician simultaneously attempts to prevent 
this movement. Normally the jaws lock together extremely tight, and cannot 
be moved by the investigator either to the right or left. With a slighter 
development of power, while the jaws are firmly closed, we may determine 
by palpation of the masseter muscle between the external angle of the eye 
and angle of the jaw and also of the temporal muscle in the temporal groove 
whether one alone or both muscles are paretic. The chief function of the 



162 GENERAL NEUROLOGICAL DIAGNOSIS 

pterygoid muscles is to move the lower jaw laterally and this function can be 
readily tested by a lateral movement against resistance. As a rule, the muscles 
of mastication play no great role in pathology. 

In the examination of patients with organic nervous diseases the investi- 
gation of the muscles of the larynx is more important. Voluntary phonation 
and inspiration, the closure and opening of the glottis, are observed with the 
laryngoscope. If this is always done in suspected cases, a decrease or entire 
absence of motility in one vocal cord may be demonstrated in cases where such 
a condition has been unsuspected. We may find either a median position 
(as the expression of paralysis of the posterior cricoarytenoid muscle, i. e., the 
initial stages of a paralysis of the recurrent laryngeal either of central or 
peripheral origin) or a cadaveric position of the vocal cord (the expression 
of total paralysis of the recurrent laryngeal nerve). The foregoing enables 
us to understand why paralysis of the posterior cricoarytenoid muscle should 
occur relatively often at the onset of many organic nervous diseases. 

We now come to the act of speech. Disturbances of speech are extraordi- 
narily important, and occupy a prominent place in neurologic symptomatology. 
The most significant features of central speech disturbance have been described 
in a special article in this volume by the most eminent authority upon speech 
disturbances, Wernicke. His description indicates the signs by which we 
determine the existence of an internal central disturbance of speech, and 
also the particular form of the aphasia. I shall, therefore, here limit myself 
to a few suggestions regarding the differentiation of the most important 
" speech disturbances " of non-central nature. Speech disturbances due to 
anatomical lesions or to functional impairment of the muscles, nerves and 
nerve nuclei of the medulla oblongata, which are called into action by speech, 
are included under the designation " dysarthria." Most of these disturbances 
which are now to be sketched will become obvious in an examination of the 
patient without an especial test. 

If, because the pharyngeal space is imperfectly closed in phonation (paral- 
ysis of the soft palate), some of the expiratory air passes out through the 
nose, the speech has a peculiar nasal twang. If there is paralysis of the 
muscles of the tongue, the lips, and the cheeks as well as of the soft palate, 
the speech will.be nasal and very indistinct (bulbar paralysis). In peripheral 
disturbances of the organs of speech the nasal factor may not be so prominent, 
but the speech is often thick, heavy, indistinct, and slow because the produc- 
tion of correct sounds has made the numerous fine distinctions in the contrac- 
tions of the muscle difficult or impossible. 

In all dysarthritic speech disturbances, especial attention must be given 
to faulty formation of the voice and a faulty mode of speaking. Often on 
attempting to speak the voice is uncertain, or on persisting it becomes irregu- 
lar, vibrating and trembling. To determine the nature of dysarthritic speech 
disturbances, it is well to have the patient intonate clear vowel sounds like 
a and e and to prolong them for a short time. 

If the disordered speech is not due to the difficulty of enunciating sounds 
(stammering), but because the union of syllables (which is normally quiet 
and uniform) is interrupted by spasms in the muscles and organs of speech, 
we are dealing with stuttering. Stuttering is merely a functional disturbance 
of speech, its cause probably originating in the cerebrum. In stuttering all 



THE PRESENT CONDITION 163 

of the muscles of the face may be contracted simultaneously with the mus- 
cles of the tongue and the mouth, particularly when the patient makes an 
effort to speak. If he is alone or does not raise his voice but merely whis- 
pers, stuttering will often cease. A disorder of speech which resembles 
stuttering manifests itself by an interruption in the sequence of syllables, so 
that the patient in beginning a word or phrase always halts at a certain 
letter or syllable, and repeating this several times, holds on to it until he 
is able to pronounce the following syllable. This kind of speech often appears 
to be due to affectation or design, and is noted in hysteria, particularly that 
form of traumatic origin. Among the disturbances of speech common in 
hysteria the most important are aphonia, egophony and the falsetto voice. 
All three are disturbances in the production of voice. In aphonia, during 
voluntary speech the glottis cannot be closed; the patient can only whisper. 
During paroxysms of cough in these patients, the rima glottidis closes and 
an audible sound arises; in the same way hysterical patients with aphonia 
sometimes speak naturally when excited, or when they speak unconsciously 
and involuntarily. Egophony (the bleating of the goat) is the production 
of a peculiar hoarse, bleating and suppressed kind of voice; the peculiarities 
of the falsetto voice are generally known and need no description. (See arti- 
cle, "Disturbances of Speech" by H. Gutzmann, in this volume.) 

Among the most common disturbances of speech is the so-called scanning 
speech which is observed in multiple sclerosis. In this form of dysarthria 
the patient divides his words into syllables, but dwells upon one syllable until 
he can produce another. As the power of modulation generally suffers simul- 
taneously, and the patient speaks very slowly, speech becomes somewhat 
monotonous and labored. 

Before leaving disorders of speech I must call attention to a very charac- 
teristic disturbance which, in the main, belongs to the disturbances of internal 
speech. As, however, in every case of disturbed speech, there are usually also 
dysarthritic affections, I must state that this speech disturbance, " syllable 
stumbling," is characteristic of progressive paralysis. We test this by having 
the patient repeat a long word which is difficult to articulate, for example, 
" Konstantinopolitanisch." If this affection exists the patient will repeat it 
about as follows : Konstantino — Konstanto — poli — tanisch — konstanti — nopel 
— politsch — tanisch, or the like. If we listen closely, in the faulty repetitions 
of this dyslogia and dysphagia we will note dysarthritic difficulties also in 
the production and formation of individual sounds and letters. Among simi- 
lar examples for practice are the following : " Around the rugged rock the 
ragged rascal ran " ; " Peter Piper picked a peck of pickled peppers " ; "A 
big black bug bit a big black bear." In paralysis the speech generally becomes 
more distinct the more frequently the word is repeated. 

Another cranial nerve, the spinal accessory, and, to a small extent, the 
cervical nerves, innervate the trapezius and sternocleidomastoid muscles, the 
most important movers of the head and the cervical vertebral column. Lesser 
movements, particularly in the joint between the occipital bone and the atlas, 
are produced by the small muscles of the neck. Eesistance is generally lim- 
ited to the forward and backward movements of the head and cervical verte- 
bral column and to lateral movements and turning of the head. Normally 
any of these movements may be performed so vigorously by an adult that the 



164 GENERAL NEUROLOGICAL DIAGNOSIS 

physician cannot prevent them. The same is perhaps true to an even greater 
extent of raising the shoulder, which is also done by the action of the trapezius 
muscle. 

In testing the function of the muscles of the bach the most important 
movements are the following : Adduction of the shoulder-blades to the median 
line (action of the rhomboideus and of certain portions of the trapezius), 
retraction of the shoulder (latissimus dorsi), as well as flexion and extension 
of the back and of the vertebral column at various heights (abdominal mus- 
cles, ilio-psoas, erector trunci, quadratus lumborum and others), and turning 
of the vertebral column (semispinalis dorsi and the small muscles of the 
back). 

After these functions have been tested, the general motility must be inves- 
tigated, particularly the coarse power of the extremities. We begin with the 
upper extremity. First, with equal rapidity, the arms should be raised per- 
pendicularly, and we should note whether both arms simultaneously reach the 
vertical plane, whether in this movement the slight flexure of the forearm 
toward the upper arm is equally marked on both sides, which is normal, 
and whether in raising the arms to the vertical position the extension of the 
hand and fingers is bilaterally uniform. For detecting slight debility in the 
musculature of the arm which is not revealed by coarse tests owing to the 
resistance which will be described later, we have a fine reagent in the pre- 
viously mentioned test : The slightly paretic arm on rapid raising reaches the 
vertical plane later, the forearm is flexed less tensely upon the upper arm, 
and the hand and fingers are less strongly extended against the forearm, than 
is the case upon the normal side. 

The chief resistant movements are the following : We should attempt forci- 
bly to lower the extended arm of the patient. Next, when the arm is extended 
laterally, we should try to draw it toward the middle and, vice versa, when the 
arms are stretched posteriorly and downward we make an effort to move 
them from this position. Finally, the arms which are extended laterally from 
the thorax at an angle of about 45° are to be forcibly lowered while at the 
same time the physician attempts with his hands to resist this movement. 

As already indicated, slight weakness, particularly on movements of the 
shoulder-joint, may escape this test by resistance ; probably because the amount 
of power developed in the shoulder-joint is extraordinarily great. 

The movements of the elbow- joint are examined to see with what strength 
the forearm can be flexed and extended, the physician trying to prevent the 
movement. In conditions of debility, in flexion of the forearm the supinator 
longus may be tested alone by having the pronated forearm flexed, and the 
biceps alone may be tested by having the supinated forearm flexed. Prona- 
tion and supination of the forearm may very easily be examined by producing 
the corresponding resistant movements. 

The flexors and extensors of the hand and fingers and the muscles of 
the hand are tested by having the patient squeeze the hand of the examiner 
as tightly as possible. Squeezing the hand is at the same time a good test 
of the strength of the muscles of the upper arm and for some of those of 
the shoulder and back; on tightly closing the hand all of these muscles 
become tense when the arm is fixed. 

Of the action of the extensors of the hand, we can easily convince ourselves 



THE PRESENT CONDITION 165 

by making a fist. In making a fist each extension of the root of the hand 
results in stretching as far remote from one another as possible the points of 
insertion of all the muscles which flex the hand and the fingers. It is easy 
to see that this increases the dynamic action of the flexor muscles. In radial 
paralysis pathology furnishes a proof of the correctness of these statements, 
for, in this condition, the fist cannot be tightly closed. 

A number of instruments have been invented (the best representative 
being the spring dynamometer) to test the grasp of the hand. As a rule, 
all of these instruments may be dispensed with. 

Special examination of the muscles of the hand is only necessary when 
symptoms point to the hand, or when we suspect certain diseases of the spinal 
cord in which experience proves the muscles of the hand to be usually impli- 
cated. 

The power to separate the fingers (the interossei, extensor digitorum com- 
munis) and then to restore them to the normal position (interossei) is tested 
by placing the volar surface of the patient's hand and entire forearm upon a 
flat table after which the investigator places his hand with slight pressure 
upon the back of the patient's hand. Normally, in spite of the pressure of 
the overlying hand, the separation and adduction of the fingers will be quite 
free and rapid, and will be done by a single movement. The adduction and 
opposition of the thumb will normally be so great that the investigator can 
scarcely overcome it. The completeness of opposition can be tested in another 
way, the patient being told to touch the tips of all the other fingers with the 
tip of the thumb extended in all of its joints. In this test the distal joints of 
the fingers that are to be touched must be extended, only the metacarpal pha- 
langeal joints being flexed. 

This movement can only be performed in normal breadth and with the 
normal power, which can hardly be overcome by the investigator, when the 
opposition muscles as well as the interossei functionate normally. 

If we suspect slight debility, the function of the muscles of the forearm 
and hand may be tested by having the patient rapidly and alternately flex and 
extend the fingers as, for instance, in piano playing. Even slight differences 
between the normal and diseased extremity become noticeable. Notwith- 
standing the fact that the right arm is normally somewhat stronger than the 
left, these movements as in piano playing are normally made with the same 
power by both the right and left hands. 

An irregular tremor of the forearm which appears upon powerfully grasp- 
ing with the hand suggests a slight degree of debility which is not detected 
by resisting movements. 

Sometimes it is advisable to extend both arms either perpendicularly or 
horizontally or at the side, and to observe whether the arms simultaneously 
become weak, or whether this may vary. 

In some diseases it is necessary to note whether the strength gives out. 
Thus in many functional states of debility, so-called adynamia, the first put- 
ting forth of a strength in movement may be very good, but it soon gives out, 
and the terminal mechanical effect is very slight. A similar condition will 
occasionally be observed in so-called myasthenic diseases. Here the original 
and marked muscular power, which is sufficient for the performance of the 
first movements, becomes slighter on each repetition. 



166 GENERAL NEUROLOGICAL DIAGNOSIS 

If, in these cases, the arms of the patient are vertically raised, several 
times in rapid succession without permitting a pause, the arm can the first 
time be raised to the vertical, the second to a somewhat lower position, and 
after a few repetitions — perhaps the tenth — can be moved only very slightly 
from the wall of the thorax. 

This phenomenon, however, can only be designated " myasthenic " pro- 
vided there are other signs (electrical) of the myasthenic pathologic picture, 
for similar but less conspicuous diminution of strength is occasionally seen 
in neurasthenia and hysteria. 

The last stage in testing gross muscular power is the examination of the 
legs. Here it is best to have the patient flat upon his back. At first the leg, 
extended at the knee, is to be raised with all the might, while the physician 
makes strong pressure downward at about the middle of the thigh. The leg 
is then powerfully abducted and adducted, the physician each time making 
powerful resistance. The extension of the thigh against the pelvis can best 
be tested in the lateral position. The power of the chief flexor and extensor 
of the pelvis upon the thigh (the ilio-psoas and the gluteus magnus) may 
also be tested inversely with the leg fixed; for example, while the patient is 
standing erect or in the recumbent posture with the leg fixed, the pelvis is 
bent forward and extended, or the patient is asked to rise from the dorsal 
decubitus in spite of resistance and then to lie down. 

Flexion and extension of the knee-joint may be tested in the recumbent 
posture or, still better, while the patient is lying upon his abdomen. The 
power of flexion and extension of the foot is tested by telling the patient to 
move his feet dorsally or in the plantar plane with all his might. It is 
advisable for the physician to illustrate these movements with his own foot 
or to perform them passively with the patient's foot. When the foot is flexed 
dorsally, the physician uses his whole strength and attempts to draw down the 
patient's foot. If the flexors of the foot have normal power this can always 
be done. The plantar muscles of the foot (as well as all the other muscles 
of a normally vigorous lower extremity) are remarkably strong. The only 
movement in the lower extremity of the normal adult which the physician 
can counteract is the flexion of the knee. 

As a rule, such an examination as sketched above is sufficient for the mus- 
cles of the lower extremity. For special examination, the necessary points 
are indicated by the physiology of the muscles. 

It is well to bear in mind that when the ilio-psoas flexes the thigh against 
the pelvis it at the same time rotates the leg somewhat outwardly while the 
tensor fasciae latse simultaneously turns it a little inward; that the tibialis 
anticus bends the foot dorsally, and at the same time elevates the internal 
border of the foot, while the extensor digitorum communis simultaneously 
with the dorsal flexion of the foot raises the external border of the foot. In 
a minute examination of the muscles of the leg we must always remember 
that the muscles upon the posterior surface of the thigh pass over two joints 
and, like some of the muscles running from the pelvis upon the extensor 
surface of the limb — passing through the thigh — extend to the leg. 

The movements of the toes, as a rule, can only be tested by a simultaneous 
and voluntary innervation of all the toes. In hysterics I have occasionally 
found the curious faculty of moving the toes singly and separately from each 



THE PRESENT CONDITION 167 

other, like the fingers. There is normally no difference between the power 
of the right and left legs. 

In the legs, just as in the arms, we can sometimes determine a slight 
degree of .weakness with resistant movements from the facts that when certain 
motions, such as flexion and extension of the knee-joint, are rapidly repeated, 
or both legs are alternately extended, tremor may appear, or dropping of the 
leg is observed prematurely ; when the patient is directed to put first one and 
then the other leg upon a chair without aiding himself with his hands, if one 
leg is slightly paretic the patient is obliged to support it in putting it upon 
the chair, or perhaps some other uncertainty or a difference between the legs 
may become apparent. The gait often reveals a slight weakness in one or 
both legs. 

Examination by means of resistant movements has the disadvantage that 
it is dependent upon the cooperation or, rather, upon the psychical condition 
of the patient. If, in the examination, a motor condition of debility appears 
to depend upon the psychical element, this circumstance, in all probability, 
contraindicates the organic nature of the paralysis. If a voluntary weakness 
of function is brought about by the patient, i. e., by simulation, the debility 
is of hysterical, hypochondriacal, or neurasthenic nature. As the most com- 
mon variety of this paralysis I must mention hysterical astasia-ahasia and 
general, hypochondriacal, neurasthenic adynamia. The psychical nature of 
these affections is especially revealed by the fact that the muscles intended for 
certain movements which the patient deems important (for instance, walking, 
pressure of the hand, etc.), are paralyzed, or at least paretic, yet are able 
to perform their functions as soon as the final resulting mechanical or loco- 
motor effect is different from that which the patient has inseparably associated 
with the actual function of the muscles affected. This will likewise explain 
the fact that the shoulder and elbow movements are often quite strong in 
neurasthenics yet the pressure of the hands is very feeble, and, vice versa, 
that in the lower extremities the knee and hip movements may be weak while 
flexion and extension of the foot are very energetic : Therefore, the movement 
which the patient believes to be the most important is always the weakest. 

Pareses and paralyses of organic nature are much less dependent upon 
the influence of the psychical condition, although not entirely independent 
of this. Debility due to organic defects follows interruptions to and damage 
of the motor tract from the cerebral cortex to the muscle. A detailed descrip- 
tion of the many peculiarities of organic paresis is not within the scope of 
this article. I shall only mention that hemiplegic organic paresis or paralysis 
signifies a pathologic seat in the brain. Paraplegia (paralysis of both arms 
or of both legs or of both arms and legs) contraindicates that the seat of the 
disease is in the brain, and favors its situation in the spinal cord or in the 
peripheral nerves. A few of the fundamental laws for the topical differential 
diagnosis of organic paralysis were mentioned in the description of spastic 
muscular conditions. Here I must reiterate that organic as well as functional 
paralysis may be of spastic or flaccid type. 



168 GENERAL NEUROLOGICAL DIAGNOSIS 

THE EXAMINATION OF THE COORDINATION OF 
MUSCULAR MOVEMENTS 

(Coordination of muscular movements, ataxia, static ataxia, test of ataxia in the 
arms and legs, the erect posture, various degrees of swaying with closed eyes and with 
feet in juxtaposition, swaying in organic ataxia and in functional affections, swaying 
in amaurosis with closed eyes, swaying of patients with genu varum, observation of 
the gait, inclination of the pelvis, pelvic movements, paretic gait, equine (steppage) 
gait, spastic gait, spastic-paretic gait, ataxic varieties of gait, determination of these, 
waddling gait, the gait in multiple sclerosis, cerebellar gait, dragging of a leg, inter- 
mittent claudication, arrhythmic and " sweeping " gait in hysteria, influence on the 
gait of using a cane.) 

Even though we have determined by resistant movements that the indi- 
vidual muscles are of normal power, nevertheless voluntary motions may be 
markedly impeded, because in all voluntary movements the action of several 
muscles is necessary. It is evident that in all muscles which perform a definite 
movement by their simultaneous contraction the various degrees of contrac- 
tion must bear a certain relation to each other. If one of the muscles whose 
action is necessary for a certain movement contracts at the wrong time, or too 
much or too little, i. e., too early or too late, too violently or too feebly, the 
simultaneous contraction of all the other muscles will not produce the desired 
result, for the general activity depends upon the normal function of all mus- 
cles of the group. A lack of harmony in a common functioning muscle group 
may be due to the fact that one muscle of the group is paretic or paralytic, 
or that spastic conditions exist in one or several muscles, etc. This purely 
mechanical disturbance of coordination we shall not now discuss further. On 
the contrary, we turn to a form of incoordination — called ataxia — due to a 
faulty or insufficient activity and disposition of each muscle of the group 
which has a function to perform, as well as to a faulty control and regula- 
tion of the necessary degree of contraction in the muscle group. Here the 
existing disturbance — diagrammatically presented — arises because the relations 
and processes in the periphery (ataxia in many affections of the spinal cord, 
particularly in tabes dorsalis) are imperfectly transmitted to the subordinate, 
so-called coordination, centers in the brain, the trunk of the brain, and the 
medulla; or it may arise because the coordination centers themselves are dis- 
eased (diseases of the cerebellum, certain affections of the motor cortical re- 
gion) ;, or, finally, because the motor nerves are not intact (certain forms of 
neuritis [so-called ataxic neuritis without demonstrable sensory disturbance] 
in diphtheria, etc.). 

The limits of our theme do not permit me to give a diagrammatic sketch 
of ataxia, particularly as the most important questions concerning it have 
not yet been cleared up. But one particular form of ataxia, static ataxia 
(in contrast to movement ataxia), I must mention. By static ataxia I mean 
a disturbance in the coordination of those contracted muscle groups, chiefly 
tonic, which, for instance, are particularly active in standing erect, in sitting, 
in maintaining the equilibrium of the body, in raising or extending a mem- 
ber, etc. 

From the foregoing it is evident that in the examination of our patients 
— although ataxia may occur in almost any muscle groups — much depends on 



COORDINATION OF MUSCULAR MOVEMENTS 169 

whether the arms or legs are ataxic, and whether the equilibrium of the body 
can be preserved in all positions. 

In testing ataxia of the extremities the most suitable movements are those 
which require the conjoint action of the greatest number of individual muscles 
in order to perform a minimal movement. The finer and more complicated 
the movement, and the greater the number of muscles called into action, the 
more sensitive is the reaction. 

Therefore the patient should be directed to touch with the tip of his 
index finger certain parts of his face (the tip of the nose, the lobe of the 
ear at either side, the eyes, certain teeth, etc.) ; or he is asked to point rap- 
idly with a lead-pencil to certain spots upon a paper, to thread a needle, and 
the like; or food and drink are to be carried to the mouth, or he is asked 
to button and unbutton a garment, to write, to play the piano, to pick up 
with his fingertips a needle which lies on a smooth surface — the ataxic will 
use the volar surface of the end of the finger or even the middle joint — etc. 
A method less fine which is in common use, is to bring together the tips of 
the two index fingers; sometimes it is advisable to flex the individual joints 
of the arms and fingers slowly and uniformly, and then to extend them. If 
these movements are not performed evenly and harmoniously, if they are 
clumsy, paroxysmal and ununiform, or are waddling, ataxia is present — pro- 
vided there is no paresis or spasm. Sometimes ataxia is most obvious when 
the patient performs the desired movement rapidly, sometimes when it is very 
slowly done. If we are in doubt concerning the existence of ataxia — as will 
often be the case — or if we have special cause to suspect it, the patient should 
not be allowed to control the movements by the use of his eyes. 

Ataxia in the upper extremity is markedly increased on closing the eyes. 
The slight ataxia at first present may show a diminution on subsequent 
attempts. This is especially true in slightly complicated movements, for ex- 
ample, in bringing together both the index fingers and the legs, and still 
more so in the tests for ataxia of the legs which will later be described. 

Better than any of these methods, at all events less complicated and 
tedious, is the observation of the trivial actions and movements of the patient. 
The way in which he takes off his coat, puts his fingertips on the inside and 
his thumb on the outside of the front of his coat, loosens the collar and buttons 
it, how he unfastens his waistcoat, whether on donning the coat he tries to 
adjust his cuffs or the sleeves of his shirt, etc. These observations, if accu- 
rate, will tell us, even before beginning the actual investigation, whether or 
not the patient has ataxia of the hands. 

In the lower extremity it is best to test for ataxia with the patient in the 
horizontal position. One of the best known tests is to have the patient place 
one heel upon the knee of the other leg or upon the great toe of the other 
foot ; he should then be asked to touch with the tip of his foot anything that 
is held out toward him, to describe in the air a certain figure, an 8 or the 
like, or to raise slowly and uniformly the leg extended at the knee-joint, and 
in like manner to let it fall. If there is an ataxic disturbance of the leg, 
in the horizontal position this becomes evident as soon as the patient closes 
his eyes. 

Simple as these tests appear to be, they will rarely leave us in doubt as 
to whether or not actual ataxia is present. This is partly due to the fact that 



170 GENERAL NEUROLOGICAL DIAGNOSIS 

stupid and clumsy patients are incapable of understanding or accurately per- 
forming complicated movements. On the other hand, in slight ataxia — pro- 
vided the patient is intelligent — the repetition of a simple tactile test which 
has been practised only a few times will often cause ataxia of the legs, for 
the moment at least, seemingly to disappear, particularly that produced by 
one special movement. 

As a rule, we need not be particular to test the legs, but may rest content 
with the test of the heels and toes provided this has been correctly carried 
out two or three times. Naturally, other and easier movements will be cor- 
rect. One of the most difficult tests is the simple, slow, but uniform eleva- 
tion and lowering of the leg when extended at the knee-joint. 

A test of the coordination when standing and walking leads us to the 
description of both of these functions. Not only must the coordinative faculty 
be investigated, but passive motion and the development of coarse power is 
complemented by observation while standing erect, particularly on walking. 
Hence we now follow the same method of examination with complicated 
movements while walking and standing, which can only be normal when 
passive motion, power and coordination are also normal. 

The investigations of physiologists have taught us that on standing erect 
the position of the legs is not fixed by gravity but by muscular tension. If, 
therefore, the muscles are decidedly debilitated or incoordination exists, nor- 
mal standing is impossible during their uniform contraction — static ataxia. 

A good test of the patient's ability to preserve the equilibrium of the 
body while motionless and standing erect is to have him place both feet close 
together at the heels and toes, and then shut his eyes. This lessens to a 
minimum the base of support for the feet, while by the simultaneous closing 
of the eyes any slight disturbance of the sensory conduction, indispensable 
for normal coordination, becomes increasingly noticeable. Therefore by this 
or a similar test (for example, having the patient stand on one leg) minimal 
disturbances of coordination are sometimes revealed which would not be 
apparent in the horizontal position. 

While the normal person — perhaps after a slight uncertainty at the start 
— is able to stand firmly in the position just mentioned and only occasion- 
ally bends the trunk slightly, in the ataxic great uncertainty at once appears. 
The upper part of the body sways, the feet which were at first in close juxta- 
position separate and move to and fro, the extensor tendons on the dorsum 
of the foot begin to twitch in the effort to restore the disturbed equilibrium, 
the patient attempts to balance himself with the arms, and finally opens his 
eyes in order to support himself. This is designated " Romberg's symptom," 
and in the form described is only found in true ataxia of the trunk or legs. 
We usually succeed in determining the ataxia on thorough examination in 
the recumbent posture. There is, however, another kind of swaying on clos- 
ing the eyes and with the feet in juxtaposition in which ataxia will not be 
revealed by examination in the horizontal position. Here the swaying itself 
is different, and only when most extreme can it be confounded with the 
true Romberg sign. The patients do not attempt to overcome their uncer- 
tainty by movements of the trunk or the feet, or by correction of the extensors 
of the foot. On the contrary, these are impossible because of the coarse and 
violent movements of the body which at once appear upon closure of the eyes. 



COORDINATION OF MUSCULAR MOVEMENTS 171 

A true swaying appears when the patient grasps at objects for support, makes 
a few steps forward or backward, or seems about to fall. On opening his 
eyes, all uncertainty quickly disappears, much more rapidly than in true ataxia. 

To differentiate the kind of swaying which follows closure of the eyes, 
we must bear in mind that a true ataxic upon closing his eyes will sway most 
severely, and is unsteady on standing still even with open eyes, while in the 
non-ataxic, in spite of marked swaying with closed eyes, this is not the case. 
The latter form of swaying is observed in certain cases of neurasthenia, hypo- 
chondriasis, hysteria, and other neuroses. It is due to a psychical cause, 1 and 
may be decreased or caused to disappear by various measures. It is advis- 
able for the physician to examine the reaction of the pupils after the patient 
has just placed his feet into juxtaposition. As the physician converses with 
the patient, he tells him to cover one eye with his hand, and the physician for 
an instant covers the other eye with his own hand, thus encouraging the 
patient and distracting his attention. In such cases we may of course attain 
the same end and cause the swaying to disappear by other means if only 
we can distract the patient's attention from the test about to be undertaken; 
this will prevent restlessness, anxiety, or any other similar hypochondriacal 
effect. Patients of this kind often spontaneously complain of vertigo. 

Scarcely to be distinguished from the behavior of this group of neuras- 
thenics are the actions of another class of patients who also complain of 
vertigo when this test is made. These are patients with cerebral arteriosclero- 
sis and with certain focal diseases of the brain. In these cases the swaying 
upon closure of the eyes cannot be stopped by suggestion, like the swaying 
due to psychical conditions, or by distracting their attention. On the con- 
trary swaying often begins when the eyes are open, like the swaying in severe 
ataxia. 

The enumeration of the various forms of " Romberg's symptom " by no 
means exhausts the varieties of swaying. Another characteristic form of 
swaying is found in disease of the cerebellum, and is designated cerebellar 
ataxia. In the horizontal position, as a rule, no ataxia can be perceived. 
But on standing, and standing with the eyes open, unsteadiness and sway- 
ing similar to that of a person under the influence of alcohol becomes notice- 
able. This swaying is peculiar from the fact that closing the eyes increases 
the uncertainty much less than might be supposed, and as is the case in 
other forms of swaying. 

I must call attention to a point always to be borne in mind in testing for 
Romberg's sign. It sometimes happens that those muscles which unite the 
trunk with the pelvis are either partially paretic, and therefore make stand- 
ing impossible, or, like the muscles of the extremity, they are in a state of 
incoordination, i. e., are actually ataxic. 

In a case of this kind, as may be readily understood, examination of the 
muscles of the leg in the horizontal position will reveal perfectly normal 
coordination. Here the impossibility of standing quiet with the feet in juxta- 
position has nothing to do with the lower extremity, but is due to inability 
to fix and balance the upper part of the body and the vertebral column. 

i In this connection the fact is interesting that many cases of amaurosis sway when 
their feet are in juxtaposition as soon as the eyes are closed. 



172 GENERAL NEUROLOGICAL DIAGNOSIS 

Before turning to the consideration of the gait I must point to a source 
of error, frequently overlooked, which sometimes produces Romberg's symptom 
when no ataxia is present. If the patient is bow-legged or has any other 
deformity of the legs it is often difficult for him to balance his body with 
the feet in juxtaposition and the eyes closed, hence the seeming " Romberg's 
sign." 

The observation and judgment of the gait forms one of the most important 
yet difficult processes in the examination of our patients. A normal gait 
implies the putting forth of sufficient coarse power, and that there is normal 
(neither increased nor diminished) muscle tonus, intact coordination, undis- 
turbed cerebral innervation, and free movability of the joints. Moreover, in 
disturbances of the gait there are abnormal sensations and pain in the leg, 
therefore centripetal processes are active. All of these points may be con- 
sidered when we examine the gait. 

In order that his gait may be observed, the patient should remove his 
lower clothing and his shoes and stockings. Slight disorders of gait, espe- 
cially if ataxic, are more readily perceptible in the naked feet than other- 
wise. If possible, the pelvis and the lower part of the trunk up to< the navel, 
as well as the legs, should be inspected during walking. While the patient 
walks forward and backward in a straight line on the smooth floor of a large 
room, the physician should note whether there is a normal tendency of the 
pelvis to a horizontal plane (forward inclination of the pelvis and lumbar 
lordosis are seen in muscular dystrophy), and whether the crests of the 
ilium are equally high and remain in this position during the act of walking. 
In certain pathologic conditions, particularly in paralysis of the flexor mus- 
cles of the thigh, when the patient raises his leg on attempting to walk the 
entire side of the pelvis is immediately elevated, and the trunk is slightly 
inclined backward and to the opposite side. In other cases (with paralysis 
of the gluteus medius and gluteus minimus, muscular dystrophy), just as in 
congenital dislocations of the hip-joint, the moment the feet are placed upon 
the floor and one leg is freely moved the crest of the ilium on the moving 
side is distinctly seen to descend, while on the motionless side an upward 
movement is noticed. This " waddling " gait is due to the fact that the 
two gluteal muscles lack the power to keep the pelvis firmly attached to the 
trochanter. Hence, on walking, the trochanter at the side of the leg is re- 
moved from the crest of the ilium, i. e., it rises or, more correctly, it is drawn 
upward so that by gravity the unsupported surface of the pelvis sinks down- 
ward. 

More points are to be noted in the legs than in the pelvis: First of all, 
whether the insertion of the leg into the hip- joint is normal, i. e., whether 
the tip of the foot appears to turn out or inward more than normal, and 
whether all the necessary movements of the joint in walking are performed 
in their normal excursus. If the leg is not sufficiently flexed or extended 
at the hip or at the knee-joint, or if the foot is not sufficiently flexed dorsally 
and the tip is therefore not raised from the floor, there is — provided no passive 
hindrance exists — simply a paretic disturbance of the gait. Such a disturb- 
ance of gait is observed in all flaccid paralvses of the legs, for instance, in 
anterior poliomyelitis, in neuritis, etc. The characteristic steppage gait belongs 
to the simple paretic forms of gait. In this gait, which depends upon paral- 



COORDINATION OF MUSCULAR MOVEMENTS 173 

ysis of the muscle group supplied by the peroneal nerve, the tip of the foot 
hangs flaecidly upon the floor. The leg must be hyperflexed at the hip and 
knee in order to lift the foot from the floor and to make any forward move- 
ment. This causes a gait which resembles the walk of a horse (equine 
gait). 

If the movement of the leg from the floor and toward the floor is springy 
(similar to the spring of the blade of a pen-knife in opening and closing), 
and instead of the normal and gradual removal of the foot from the floor the 
sole of the foot pressed against the floor makes a prolonged, lingering, sliding 
or shuffling sound, we are dealing with a spastic gait. When the ear has 
become somewhat accustomed to it, this disturbance in gait will be recog- 
nized by the peculiar scraping sound due to the sliding of the sole of the spastic 
foot over the floor. If the knees at each step rub against each other, and 
thus force the feet to describe a long drawn out S-shaped figure, we know 
that the adductors of the thigh are spastically contracted. 

A patient with this spastic gait well-marked reminds one of a soldier 
practising a " slow step " ; the extended trunk appears to hurry ahead while 
the entire lower part of the body seems to be held back. 

As a rule, spastic gait is not purely spastic but is combined with paretic 
conditions. This is because spastic symptoms primarily follow an interrup- 
tion of the voluntary innervation of the pyramidal tract. 

Spastic-par etic gait is found in myelitis and in traumatic diseases of the 
cervical and thoracic portions of the spinal cord, in amyotrophic lateral scle- 
rosis, and sometimes in multiple sclerosis, in syringomyelia, and in various 
spastic cerebral paralyses. Another spastic-paretic gait is the so-called cir- 
cumduction gait, in which the leg firmly extended at the knee-joint and the 
tip of the foot pressed against the floor are moved forward in an arc by ele- 
vating the same side of the pelvis and flexing the upper part of the body 
backward to the opposite side. In passing, it may be said that a spastic gait 
may often be diagnosticated by examining the soles of the patient's shoes: 
We will find a worn appearance at the tip of the sole where the foot scrapes 
the floor. 

A third disturbance of gait, different from the two previously mentioned 
but no more rare, is the ataxic. I must emphasize that this is not a uniform 
phenomenon, as is, for instance, the spastic gait, but it appears in different 
conditions. All forms of ataxic gait have in common that on placing the 
feet upon the floor they do not reach the point aimed at, and which the 
patient himself expected to touch. 

If an ataxic patient is told to walk in a straight line at each step putting 
one foot just in front of the other, this is either impossible or just as diffi- 
cult as if he were required in walking to touch at each step one or another 
definite spot upon the floor with the tip of his foot or his heel. 

On attempting to follow instructions the uncertainty increases, and the 
physician notices that only with the greatest difficulty and by strenuous and 
compensatory efforts to balance the body, sometimes with the swaying of the 
entire body, do the feet reach the spot indicated on the floor or one along- 
side it. 

When the disturbance is slight, the previously mentioned tests of ataxia 
may be made more difficult by having the patient close his eyes. Under these 



174 GENERAL NEUROLOGICAL DIAGNOSIS 

circumstances a normal person, after a preliminary slight swaying, walks with 
steadiness. 

Among other tests to determine the ataxic gait, I advise the so-called " turn 
about face " in which the patient, as in the military, turns upon one heel 
on his axis, without changing the position of the feet which are at a right 
angle with each other. 

It is often well to interpose this rapid turning between the simple tests 
in walking, so that the command of " right about face " will be sudden and 
unexpected. On being confronted with this immediate necessity to perform 
a prescribed movement, slight degrees of ataxia visibly increase. 

I have stated that the ataxic gait is not a strict entity. One disorder 
of the gait which belongs to the ataxic group appears, at the first glance, to 
have nothing in common with those previously described. 

The disturbance to which I allude is conspicuous because the patient throws 
his legs outward at random, he stamps, or lifts his foot high up from the 
floor, the gait resembling that of a fowl. The knees appear to be over- 
extended. On close observation we notice that the patient, in spite of appar- 
ent certainty, never quite hits his mark, but either fails to reach it or throws 
his foot too far, and is finally happy that he can bring it back to earth at all. 
This gait is seen in severe ataxia associated with conspicuous hypotonia of the 
muscles of the leg; therefore in some (but by no means in all) cases of tabes. 

In other ataxic patients, in the first moment of examination the ataxic 
gait is less conspicuous than something which reminds us of the spastic gait, 
a detached appearance of the leg and a stilt-like walk. Simultaneously we 
observe a peculiar trembling of the legs and body, and at the first glance it 
is hard to say whether the uncertainty, the zigzagging, is due to pure ataxia 
or to the tremor. This gait is found in some cases of multiple sclerosis, an 
affection which, as we have seen, often presents the simple spastic gait. From 
the foregoing it is evident that the ataxic gait as well as the paretic and spastic 
elements of other gaits may be admixed. 

In speaking of static coordination we have already mentioned cerebellar 
ataxia. This reveals itself in walking just as it does in standing. The patient 
sways like an alcoholic, i. e., while the individual step and the action of the 
joints are quite normal the patient reels and sways to and fro from the fact 
that with each step he must regain his lost equilibrium, hence the constantly 
swaying, deviating direction of his gait. 

These three varieties of gait, the paretic, the spastic, and the ataxic, with 
their combinations, by no means exhaust all the difficulties in walking; one 
of the most frequent and most common disturbances of gait we have not 
yet considered, namely, mere dragging of one leg and avoidance of its use. 

If we observe that a patient drags one leg and refrains from standing 
upon it as long as upon the other, while no other spastic or ataxic symptom 
can be discovered, this usually means nothing but that the patient spares 
the leg. 

This assumption seems all the more plausible if the patient puts only 
the tip of his foot or its external border on the floor. As a rule, in these cases 
there is pain or other abnormal sensation in the leg; if this disappears the 
gait at once returns to the normal. 

The best example of this latter disturbance in gait is intermittent claudica- 



COORDINATION OF MUSCULAR MOVEMENTS 175 

tion, which always continues as long as the pain lasts, and is due to an insuf- 
ficient supply of blood to the muscles of the calf. 

The simple limping gait is conspicuous, in the first place, by its irregular 
rhythm which deviates from the normal, because only one leg is pathologically 
moved. Under similar circumstances, i. e., by dissimilar but marked motor 
impediment to the use of both legs, the paretic, spastic and ataxic gait may 
show arrhythmia, besides other symptoms which have been described. This 
also should receive due attention. 

In describing different varieties of gait I have only discussed those noted 
in organic diseases. The question arises. Do none of these varieties of gait 
occur in hysteria? In answer, it must be stated that all varieties of gait 
occur in hysteria except the ataxic waddling gait and the true circumduction 
gait of hemiplegics. 

A gait strictly characteristic of hysteria does not exist, unless we con- 
sider as such the " sweeping " gait which occurs in hysterical hemiplegia ; here 
the paralyzed leg is scarcely moved at all at the hip-joint, but is dragged 
stiffly like a broom. 

The spastic gait of hysteria closely resembles that of organic disease, and 
dysbasia and abasia, which are so common, differ but little from ataxic or 
cerebellar swaying. An aid to the differential diagnosis of the hysterical forms 
is the great changeability of mind under psychical influence. 

Before discussing other symptoms in the motor sphere I must call atten- 
tion to a valuable point in the investigation of the gait: The patient's walk 
should always be tested without permitting him the support of a cane. The 
aid of a cane greatly changes the mode of walking, particularly in the ataxic, 
and makes a differentiation extremely difficult. 

INVOLUNTARY MOTOR SYMPTOMS 

(Associated movements, tremor, tremor during rest, test of intention tremor, the 
tremor of paralysis agitans, coarse and fine tremor, nystagmus, shaking tremor, fibril- 
lary tremor, myokymia, idiomuscular contraction, athetosis, choreic movements, their 
occurrence, tic, its nature.) 

In the examination of the motor sphere we have so far concerned ourselves 
only with voluntary movements. I will now consider a number of involuntary 
movements which we observe in examination. That form of involuntary 
motion which is perhaps most closely allied to the voluntary is the so-called 
coordinated movement. Coordinated movements, i. e., the contractions devel- 
oped by involuntary movements of other muscles than those which obey the 
impulses of the will, are invariably the accompanying phenomena of motor 
activity in an individual whose motor sense is dormant. The more highly 
developed the individual's* motor sense, the more control he exercises over 
the impulses of his will, and he limits to these muscles the contractions neces- 
sary for the desired effect. Thus the child gradually learns to suppress a 
number of coordinate movements; but in most persons manv of these move- 
ments (for example, the innervation of the facial nerve when making firm 
pressure with the hand, etc.) persist. 

We occasionally meet persons in whom, without other symptoms of disease, 
these well-developed coordinated movements appear; for instance, whenever 
13 



176 GENERAL NEUROLOGICAL DIAGNOSIS 

they move the fingers of one hand they also move those of the other hand, 
or they simultaneously move the toes. In such cases, usually neurotically 
predisposed persons, these coordinated movements are often observed in other 
members of the family, and this can be regarded only as a curious anomaly. 

Under pathologic conditions, however, coordinated movements occur in 
individuals not formerly thus affected. During the course of hemiplegia, 
coordinated movements often appear upon the paretic side; if, for example, 
the patient makes forcible pressure with his hand upon the healthy side, the 
fingers of the paralyzed side react slowly and sluggishly. As this symptom 
appears almost simultaneously with contracture of the paralyzed extremity, 
it must be regarded as unfavorable. In hemiplegics we sometimes see coordi- 
nated movements in the paretic foot when the patient tries with all his might 
to innervate the paralyzed upper extremity. 

Another form of involuntary motor activity is tremor. By the term tremor 
we mean the rhythmic sequence of a number of similar involuntary move- 
ments of but slight excursion. Such a tremor will usually at once become 
noticeable upon examination: for example, the tremor in paralysis agitans, 
the tremor of the alcoholic, senile tremor, the physiologic tremor due to cold, 
etc. Other forms of tremor, however, are distinctly revealed only when an 
extremity is tonically innervated; for instance, when the hands are extended, 
and among these we find the physiologic tremor which follows excitement 
and excessive corporeal exertion, the closely related tremor of neurasthenics, 
hysterical tremor, that of Graves' disease, etc. The tremor present during 
rest increases on extending the hands. A peculiar form of tremor which 
occurs during rest or on tonic innervation, can never, or exceedingly rarely 
be observed, and only becomes distinct when the patient attempts certain move- 
ments {intention tremor). Even if no tremor is observed in the patient dur- 
ing rest, or upon extending the hands, and no trembling appears when a piece 
of paper is placed over his hand, nevertheless there may be intention tremor. 
We test for intention tremor by telling the patient to grasp the finger of the 
physician which is held in front of him, or we direct him to drink water 
from a glass filled to the brim, or to carry out some similar movements. In- 
tention tremor, which chiefly occurs in multiple sclerosis, reveals itself by 
this sign; trembling movements appear which extend perpendicularly from 
the direction of the intended movement, and these constantly become more 
rapid, distinct, and violent the more nearly the hand of the patient reaches 
its goal. These trembling movements are never of great extent, nor do they 
appear so irregularly as to obliterate the aim of the intended voluntary move- 
ment. By this fact we distinguish intention tremor from ataxia, a differen- 
tiation which occasionally is extremely difficult. Since not only intention 
tremor but most other forms of tremor increase with complicated voluntary 
movements, in the diagnosis of true intention tremor we must adhere very 
strictly to the previously mentioned characteristics. 

The tremor of paralysis agitans forms a decided contrast to intention 
tremor, being most marked during rest; with intended movements it usually 
(but not always!) ceases or decidedly lessens. The last mentioned tremor 
may be readily recognized being the most complicated variety. Usually the 
tremor of shaking palsy presents the so-called coin-counting characteristic, i. e., 
there are separate and distinct tremors of the bent fingers and thumb. Occa- 



COORDINATION OF MUSCULAR MOVEMENTS 177 

sionally we observe in the forearm the movements of pronation and supina- 
tion. We now come to the description of the different movements in tremor, 
and must differentiate between the coarse, medium, and very fine vibratory 
tremors. The finer the tremor, the more rapid does it become. To the first 
mentioned group belong the tremor of multiple sclerosis, senile tremor, and 
that of paralysis agitans. The rapid form is seen in neurasthenic tremor, in 
chronic poisoning (tobacco), and especially in Graves' disease. Alcoholic 
tremor may be rapid or slow. We can inform ourselves of the nature of the 
trimor by watching the vibration of a sheet of paper placed upon the dorsum 
of the trembling hand or by a test of the writing. If we desire an accurate 
count of the individual movements, this is readily obtained in the slow forms, 
and is 4 — 5 — 6 per minute. Xothing now remains but to determine which 
muscles or which joints are moved. 

This is usually quite easy. But it is more difficult — sometimes impossible 
— to determine which parts are primarily active and which are passive and 
only secondarily and mechanically implicated. This difficulty is enhanced by 
the fact that while, in the beginning, there is merely a passive movement of 
some part of the body, for example, the head, under the eye of the observer 
this may turn into active tremor. 

Tremor is observed in the head as well as in the extremities, but rarely 
in the trunk or eyes. Tremor of the eyes, nystagmus, cannot be pathogeneti- 
cally grouped with most of the other forms of tremor. In some diseases (mul- 
tiple sclerosis or certain congenital cerebral defects) it may be evoked by 
having the patient roll his eyes to one side or upward. If laterally a spas- 
modic horizontal, if upward a rapid vertical or rotary, tremor of the eyeball 
appears. Horizontal nystagmus may be produced in healthy persons by rap- 
idly turning the eye several times in its longitudinal axis. In multiple scle- 
rosis or other affections this reaction is naturally more prompt and more 
intense. 

A form of nystagmus is sometimes seen in hysteria; in fact, all kinds of 
tremor have been described in this affection. But one variety of tremor is 
frequently observed in the hysterical cases (especially in the traumatic), and 
very rarely in patients with organic disease ; namely, a peculiar, coarse, " shak- 
ing tremor " with severe oscillations. Sometimes this reminds us of the 
atypical tremor of paralysis agitans ; occasionally it resembles the coarse inten- 
tion tremor. 

Fibrillary muscular contractions are not to be confounded with tremor. 
They resemble the latter in that slight tremblings succeed each other in a 
certain rhythm. These movements, however, do not simultaneously affect 
the entire muscle, but merely parts of the muscles, the fibrillar; hence, they 
never produce a locomotor effect. On the contrary, these fibrillary contrac- 
tions are revealed only by the fact that the small area of skin over the con- 
tracting portion of muscle suddenly rises and falls. This phenomenon is 
usually repeated several times in rapid succession in the same or neighboring 
areas. If the fibrillary muscular contractions are very active and extensively 
distributed, we speak of "muscle waves" {myokymia). Fibrillary muscular 
contractions may occur in any muscles; in the tongue they are often errone- 
ously regarded as tremor. 

Fibrillary muscular contractions and myokymia are frequently observed in 



178 GENERAL NEUROLOGICAL DIAGNOSIS 

organic and also in functional diseases — particularly in neurasthenia. This 
symptom appears in almost all diseases of the peripheral nerves and the ante- 
rior horns; therefore in all those organic nervous affections which run their 
course with degenerative muscular atrophy. Hence, fibrillary contractions 
form a valuable diagnostic aid in the differentiation of degenerative from 
simple muscular atrophy. 

A single fibrillary muscular contraction may be evoked if we stimulate 
a superficial muscular area by a sharp tap with the percussion hammer. If, 
however, instead of a rapidly disappearing contraction in the parallel muscle 
bundles we perceive a transverse quiver at the point where the blow was given 
and this lasts several seconds, we have before us a pathologic phenomenon, the 
so-called " idio-muscular contraction." This is most often noted in general 
cachexia. Instead of the single and fleeting " fibrillary contraction " after 
stimulation by the blow of a hammer, we sometimes note, analogous to and 
accompanying the sluggish galvanic contraction, a sluggish fibrillary contrac- 
tion. This sluggish mechanical contraction, however, is not so valuable a 
sign as a sluggish electrical contraction, for in certain muscles, even under 
normal circumstances, a contraction by no means lightning-like appears upon 
tapping. 

We now turn to the more complicated forms of involuntary motion which 
appear almost solely in the extremities, namely, athetotic and choreic move- 
ments. Athetotic movements are usually confined to the fingers and toes. 
In athetosis, we note a continuous, uniform, and slow flexion, extension, abduc- 
tion and adduction of the fingers, these movements not being produced 
simultaneously in all of the fingers, but each finger performs a movement inde- 
pendently. These motions are very grotesque ; they are never observed in vol- 
untary and necessary movements, but are occasionally seen when a person 
yawns or stretches himself. The tempo of the finger movements often made 
in yawning reminds us of those of athetosis. Athetotic movements occur 
bilaterally — usually as a congenital condition — in neurotics and in families. 
Hemiathetosis is not infrequently a sequel of hemiplegia, either of children 
or of adults. At all events, athetosis always indicates a disease of the brain. 
In athetosis, naturally, the power of voluntary motion is decidedly impaired, 
and still more so when combined with the second of the previously mentioned 
disturbances, namely, chorea. Choreic movements chiefly differ from those 
of athetosis by their lightning-like tempo. The contractions of the muscles 
of the face, of the extremities, and of the trunk are very brief. The forehead 
is wrinkled, the mouth is drawn, the tongue rolls, the eyes are turned, and, 
simultaneously, the shoulder is raised and lowered, the thorax is expanded, the 
arm is pronated or supinated, the forearm is flexed, the fingers are briefly 
flexed or extended or otherwise moved. Analogous contractions are observed 
in the feet. The most unlike portions of the body may be implicated by the 
choreic movements, either simultaneously or a few minutes afterward, this 
and that part of the body being alternately attacked. 

If these choreic movements appear singly and substantively, the neurosis, 
chorea, is present. In its milder forms this affection is not serious; an ex- 
treme degree is by no means rare, and makes the unfortunate person attacked 
the helpless prey of a distressing and continuous muscular action which 
nothing will check, and the resulting exhaustion and impossibility of taking 



COORDINATION OF MUSCULAR MOVEMENTS 179 

food occasionally even cause death. Choreic movements are closely related 
to the athetotic; they appear unilaterally in cerebral foci and in hemiplegia, 
and resemble pathologic processes which have their seat near the optic thala- 
mus. In this form they are often continuous and persist for years, and 
extreme muscular atrophy appears in the affected extremity. Unlike athetotic 
movements, choreic movements are not rare in hysteria. 

If the choreic movements are infrequent and merely implicate the face 
or the fingers, the diagnosis may be difficult because there are also other dis- 
turbances of motion which — in their abortive forms — closely resemble chorea. 
The genesis and pathology of these disturbances of motion, the " tics," differ 
from those of chorea. True tic (not the tic-like clonic contractions in the 
course of certain cranial nerves which are caused by irritation of the peripheral 
nerves) invariably produces a complexity of movements which in themselves, 
as individual motor phenomena, reveal nothing pathologic, and perhaps become 
conspicuous only by the rapidity and severity of their course. For example, 
we observe that the patient spasmodically throws his head backward or to the 
side as we do, for instance, when a collar annoys us, or we notice that a child 
suddenly grasps the region of the knee, as if to draw up a stocking which has 
slipped down, or we see that the patient's advancing foot shuffles over the 
floor as is the case when a small object adheres to the sole of the shoe, etc. 
If any of these movements occur but once, they seem of little consequence. 
It is only when we note that the patient frequently moves his head, shuffles 
his foot upon the floor, or grasps the region of the knee in the manner de- 
scribed, that we perceive these movements to be abnormal. It is true that 
other tic movements, which under similar circumstances may possibly be 
regarded as accidental, may make their appearance during the examination 
and at once characteristically stamp the disease; for instance, a patient sud- 
denly claps his hands, in walking he may suddenly incline to the side or 
turn around, he may give vent to inarticulate sounds or obscene words (copro- 
lalia), etc. The exact study of tic movements and similar phenomena has 
made it absolutely certain that tic is caused by a disturbance of the psychical 
components of the motor act which represent uncontrollable voluntary move- 
ments. Hence tic is never to be regarded as the sign of an organic affection. 

EPILEPTIC AND HYSTERIC SPASMODIC ATTACKS 

(Course of an epileptic attack, nature of the muscle contractions, absolute loss of 
consciousness ; course of a hysterical spasmodic attack, nature of muscle twitchings, 
clouding of consciousness ; pupillary reaction in the epileptic attack and in the hys- 
terical spasmodic attack ; difficulty of determining the pupillary reaction ; the effect of 
external irritation upon the course of the spasmodic attack; the manner of falling in 
epileptic and in hysterical attacks; biting the tongue and other differentio-diagnostic 
factors.) 

Following what has been stated of the examination of the motor sphere, 
I must call attention to a phenomenon which neither physiologically nor 
pathologically can be regarded as an entity, and which reveals itself clinically 
in many somatic systems, most obviously in the motor sphere. This phenom- 
enon consists of spasmodic at tads. Our discussion will here be limited to 
general spasms, since localized spasms will be described in another part of this 
work. 



180 GENERAL NEUROLOGICAL DIAGNOSIS 

I shall first describe the symptoms which may be observed in epileptic 
and hysterical attacks by merely looking on, and will later discuss the points 
which are important in a differentio-diagnostic respect. Epileptic spasms 
appear suddenly like flashes of lightning from a blue sky. Only rarely is 
there a well developed, so-called aura (peculiar sensations in the cardiac 
region, vertigo, ascending heat, etc., sometimes hallucinations). The patient 
gives vent to a cry or utters a gurgling sound and then falls without noting 
his surrounding local conditions, and thereby not infrequently injures him- 
self. 

A brief tonic stage at the onset with a lateral rotation of the head and 
eyes or with general extension of the extremities may readily be overshadowed 
by the succeeding clonic contractions which form the chief feature of the 
spasmodic attack. These clonic contractions usually occur at once, with great 
severity and great power. Arms and legs of both sides are symmetrically 
and rapidly flexed and extended, or they reveal a semi-tonic flexion or exten- 
sion with gross vibratory movements consisting of alternating minimal hyper- 
flexions and hyperextensions. On inspiration the thorax becomes spasmodic 
and greatly expanded, remaining thus for some seconds, or it is elevated and 
lowered with gasps. The obstruction to respiration makes the face bluish 
red, the production of saliva is stimulated by simultaneous spasmodic attacks 
in the mouth, the saliva exudes from the lips with a hissing, rattling sound, 
and is changed into foam consisting of large bubbles. The lips are usually 
parted. The teeth of the lower jaw are firmly pressed against the teeth of 
the upper jaw. In this locking of the jaws the tongue is frequently caught 
between the teeth, is dented or lacerated, and bleeds. 

The eyelids are either open or half open; not infrequently the muscles 
of the eye are implicated in the spasm. The muscles of the trunk are not 
so markedly involved in epileptic spasms as are the muscles of the extremity. 
The spasm lasts, as a rule, but a few minutes; after a few isolated muscular 
spasms have succeeded one another at somewhat longer intervals it ceases just 
as suddenly as it began. The face rapidly regains its normal color, the respira- 
tion becomes more quiet. While the patient is absolutely unconscious during 
the attack, and reacts to nothing, he now reacts to a touch, to a call, etc., 
for he opens his eyes, moves his head, murmurs a few words, perhaps drinks 
a glass of water which is handed to him. Usually, however, he is still so 
confused and debilitated that he takes but little notice of his surroundings ; on 
the contrary, he turns upon his side and falls into a deep sleep. From this 
the patient awakens feeling very weak, and from familiar sensations he knows 
he has had an attack. Of the attack itself he generally has not the faintest 
recollection. 

Although variations in true spasmodic movements are not at all rare — 
I need only refer to epilepsia cursoria and epilepsia rotatoria (in which 
latter condition the patient during the spasm turns on his own axis) — as a 
rule all epileptic spasms conform to the type which has just been described, 
so that we may speak of a " typical " epileptic attack. 

On the contrary, in hysteric spasm it is impossible to speak of a " typical " 
attack. Just as the spasm of one epileptic attack in the main resembles that 
of another, just so surely do hysterical spasmodic attacks show manifold vari- 
ations. 



COORDINATION OF MUSCULAR MOVEMENTS 181 

A hysterical spasmodic attack never sets in with such severity and sud- 
denness as an epileptic attack. As a rule, there is some pain, some peculiar 
sensation, "nausea," "palpitation of the heart," "anxiety" and the like, to 
show that an attack is beginning, and this causes the patient to seek a place 
of safety for the possible fall. Frequently the hysterical attack follows some 
unusual mental condition or emotion. In this case, the original and at first 
physiologic expression of this emotion, crying or laughing, becomes more 
immoderate and by its duration constitutes a pathologic symptom, for with- 
out a sharp line of demarcation it passes into an actual attack. It is remark- 
able that in nearly all hysterical spasmodic attacks the muscles of respiration 
which physiologically are so closely related to the expression of emotion are 
predominantly implicated. While, as a rule, the epileptic attack begins with 
a brief tonic spasm followed by a long clonic stage, in the hysterical spasmodic 
attack tonic and clonic spasms may alternate. Tonic spasms in hysteria are 
often of long duration, even lasting several minutes, which is not the case in 
epilepsy. 

What follows the initial stage of the hysterical attack, which is often 
marked by laughing, crying, or moaning which causes a marked increase in 
respiration, varies so extraordinarily in individual cases, and often even in 
the same case, that no uniform symptoms can be described. Sometimes the 
patient merely flexes or extends the limbs as in epileptic contractions, at other 
times he stamps his feet or drums with the extremities, or upon the floor or 
in bed he turns upon his own axis, rotates his head to the right and to the 
left or forward and backward, flexes and extends the trunk, or raises and 
drops the pelvis as in vomiting; in the severest attacks we observe the well 
known " arc en cercle," a tonic spasm involving the entire musculature of the 
back and the legs, so that only the rigid head extended backward and the heels 
touch the floor or bed, and the rest of the body is arched upward (opisthot- 
onos). These movements which have been described may each occur singly 
and constitute the sole feature of the attack, or they may alternate. In some 
cases, during the course of the spasm very complicated movements appear 
which can hardly be designated as spasms. The patient bites, scratches, or 
strikes about him, he rises in bed, tears hib hair, etc. Thes3 " muscle deliria" 
form a transition stage, sometimes are even accompanying phenomena of 
actual delirium which not infrequently appears in the course of a hysterical 
spasmodic attack, and begins or terminates the spasm. Hence, it is evident 
that the mimical muscles are predominantly involved in the spasms of hys- 
teria. The eyes, as a rule, are firmly closed and the branches of the facial 
nerve supplying the mouth are often innervated. Frightful grimaces are not 
rare in this condition. 

During the hysterical spasmodic attack consciousness is never wholly lost, 
but is more or less impaired. It frequently shows a marked change in that 
the patient, during the attack, is completely lost to his surroundings, and 
believes himself to be in a strange place or position, and even loses the con- 
sciousness of his own identity. 

Recovery from a hysterical attack may be quite sudden with an immediate 
and full return to consciousness, or it may be gradual. The patient looks 
anxiously about, half astonished, helpless, not knowing what to do, rises, and 
then, as a rule, recalls, as a dream, what has happened. In the unconscious 



182 GENERAL NEUROLOGICAL DIAGNOSIS 

interval of the psychical condition, the patient has apparently been aware of 
the attack. Thus, the underlying cause of the emotion which brought about 
the attack is frequently remembered at the moment of waking. The entire 
duration of a hysterical spasmodic attack may vary from minutes to hours. 

All that I have stated of the two most important forms of spasm may 
readily be observed without special investigation and without even touching 
the patient. After a number of spasmodic attacks have been witnessed, and 
considering what has here been stated, the diagnosis of the nature of the 
attack will be very easy. If, however, this experience is lacking, if the physi- 
cian himself does not see the attack or only a part of it, or — what is most 
often the case — the attack is not typical, other factors must be considered 
before we can make a differential diagnosis between epilepsy and hysteria. 
The most important differentio-diagnostic factor is the reaction of the pupils 
to light. It should be remembered that, as a rule, there is no reaction to light 
in the epileptic attack, but this reaction is present in the hysteric attack, 
although there are exceptions to this rule; for, as rare occurrences, we note 
hysteric spasms in which the pupil, owing to a spasm of the dilator muscle, 
is continuously dilated, it does not follow the stimulation of light reflexly, 
and it cannot contract. 

While hysterical pupillary rigidity as a feature in the differential diagnosis 
is of very rare occurrence, difficulty in determining the reaction of the pupils 
is much more common. In the first place, rigidity of the pupils in the epileptic 
attach by no means continues during the entire spasm, but often only for a 
few seconds. Toward the end of the epileptic attack, which may be the only 
part of the attack that the physician sees, the pupils again react. Inversely, 
the pupil is just as often dilated in the hysteric attack as in the epileptic 
attack, and not only resembles the pupil of the epileptic, but reaction to light, 
although not absent, is extremely slow. In such cases the physician should 
permit the light to fall two or three times into the eye before he trusts his 
own observation, and even then he is usually in doubt as to whether the reac- 
tion was absent shortly before the examination and is now only slowly return- 
ing, whether the attack is epileptic, or whether he is dealing with a mydriatic 
and slowly reacting hysterical pupil. But these internal difficulties are not the 
only ones ; there are also external ones. Manifestly, it is very difficult to deter- 
mine the reaction of the pupil during a spasm. I shall only mention the diffi- 
culty of securing a good light just in front of the eye of a patient who is in 
spasm, and the possible danger to the patient's eye by carelessly holding a 
light too near it, or by permitting a particle of the light, for instance, a part 
of a match, to fall into the eye. I will merely state that it is often very 
difficult to get at the eyes or to see the pupils at all while the head of the 
patient is moving in spasm, or when the eyes are spasmodically rigid, are 
turned upward, or are moving to and fro. Fortunately, however, from the 
last mentioned condition we may often draw diagnostic conclusions as to the 
nature of the attack. If, when we attempt to open the lids and examine 
the eyes, the patient closes them more tightly, or if he violently beats his 
head about from right to left while we attempt to fix it, and thus tries to 
prevent an examination, this may be regarded as a positive proof of the non- 
epileptic character of the attack. And here we come to another point which 
may be of use in the differential diagnosis. From the fact that during the 



EXAMINATION OF THE SENSORY FUNCTIONS 183 

spasm the patient tightly closes his eyes or tries to draw his head away from 
the hands which attempt to hold it — even when calling to him, sprinkling his 
face, etc., are absolutely without result — we may be sure that loss of con- 
sciousness is not complete, but that the patient reacts to sensory impressions. In 
hysterical spasms this consciousness is often obvious on testing with Briquet's 
grasp (deep pressure in both hypogastric regions) : the patient either feels 
the pain, or in some other way becomes conscious of the pressure. By this 
grasp — as well as by many other violent or irritative maneuvers — we often 
succeed in aborting a hysterical attack or changing its course. Another fact 
is that by firmly holding the spasmodic limbs during a hysterical attack the 
intensity of the spasm is increased. 

As soon as we note that the spasm — generally speaking — is decidedly influ- 
enced in any way by external measures or conditions we may assume with 
great probability the non-epileptic nature of the attack. Two important dif- 
ferential points are that in hysterical attacks the patients, as a rule, never 
injure themselves by falling nor do they bite their tongues, while epileptics 
may severely injure themselves during a spasm, sometimes even suffocating 
or drowning. According to the degree of consciousness in these opposing forms 
of spasm, we note that during a hysterical attack the patient sometimes moans 
and cries, he speaks a few words, or moves his lips as if speaking. This does 
not occur in an epileptic attack (I do not now refer to the so-called psychical 
equivalents and dazed conditions, such as precede stupor in epilepsy 1 ). 

The muscular twitchings in hysteria are usually of altogether different 
character from those of epilepsy, and this may be attributed to the appar- 
ently profound loss of consciousness during the hysterical spasm. In epi- 
lepsy there is merely primitive flexion and extension, or rotary and similar 
movements; in the attacks of hysteria we usually see highly coordinate and 
complicated movements which, on account of their peculiarity, are in sharp 
contrast to epileptic contractions which are not characteristic of voluntary and 
intended movements. 

In concluding our differentio-diagnostic considerations, I must mention 
the significant circumstance that involuntary evacuation of urine or feces 
during a spasmodic attack makes its epileptic nature almost certain; I have 
now touched upon the most important points by the aid of which most cases 
can be diagnosticated. 



EXAMINATION OF THE SENSORY FUNCTIONS AND OF 

SENSATION 

In the examination of sensation and of the sensory functions of the body 
we must depend more upon the reports of the patient than upon the inves- 
tigation of motion. Only the statements of the patient will enable us to 
decide whether sensory irritation is normally perceptible. The observation of 
the reflexes, the movements of tendons or of the skin after stimulation are 
not positive tests; cutaneous reflexes are by no means rare, yet in spite of 
this the patient's sensation may not be normal; or, inversely, cutaneous re- 
flexes may be absent while touch is correctly appreciated. Therefore, in a 
restricted sense of the word, the objective determination of sensation or of 
14 



184 GENERAL NEUROLOGICAL DIAGNOSIS 

the sensory functions is impossible. With, these preliminaries, we shall pro- 
ceed to investigate the organs of special sense and their functions in so far 
as this is practicable in the office of the physician. 

EXAMINATION OF THE OPTIC NERVE 

(Eye-ground examination, medullary sheath of the optic nerve fibers, optic neuritis, 
resemblance to this, choked disc, occurrence of optic neuritis and choked disc, variation 
in the intensity of the latter, atrophy, optic nerve atrophy after optic neuritis, genuine 
optic nerve atrophy, the function of sight, the field of vision, testing the field of vision 
with and without the perimeter, scotoma, limitation of the field of vision, hysterical 
amaurosis, hemianopsia. ) 

After ascertaining the reaction of the pupil, we should in every case next 
endeavor to examine the eye-ground with the ophthalmoscope. Although we 
may be quite positive of the diagnosis of neurasthenia or hysteria, we should 
never omit an opportunity of directly examining by means of the ophthalmo- 
scope the only nerve which it is possible for us to examine in every patient. 

In examining the eye-ground we first give our attention to the optic nerve 
papilla. Changes in the retina — above all retinitis albuminurica — are by no 
means rare, but the changes in the optic nerve are more important. A pecu- 
liarity of the origin of the optic nerve, which is not pathologic but which, 
it appears to me, occurs often in neurotics, occasionally causes errors. I refer 
to the contents of the medullary sheath of the optic nerve, which is by no 
means rarely found. The entrance of the optic nerve in such cases resembles 
a sun-burst with protuberances upon its border: Medially from the periphery 
we see the light yellow, shining disc of the optic nerve from which bundles 
of the same light color radiate in various directions. This radiating appear- 
ance is due to the fact that at their ends these bundles in the retina proliferate 
somewhat, and diverge to a slight degree. 

This abnormal but not pathologic feature of the optic nerve disc must 
not be confounded with the pathologic cloudiness of the papilla which denotes 
optic neuritis. In this affection, the normally sharp outline of the papilla 
is no longer clearly defined; the usually distinct line of demarcation is oblit- 
erated, the color of the optic nerve, is no longer a glowing golden yellow of 
various tints, but, as a rule, is uniformly a yellowish red. Moreover, the 
vessels, particularly the veins, are not slender and slightly tortuous, but are 
filled, dilated, and markedly tortuous. 

With some practice the recognition of optic neuritis is generally easy. 
Confusion is occasionally brought about from the fact that anomalies of re- 
fraction make the optic nerve disc appear somewhat dull, as, for example, 
in hypermetrophic children. But I must issue a warning not to lay too much 
stress upon the fulness of the vessels. 

If, besides the signs of optic neuritis, we note that the papilla is very 
prominent, if the vessels, which in this case should extend from the retina 
above the higher level of the optic nerve disc, are " kinked," if there are 
hemorrhages in the surroundings of the optic nerve disc, or if the entire eye- 
ground appears swollen or infiltrated, we are dealing with choked disc (stasis 
papillae). In practice, a sharp differentiation of choked disc from simple 
optic neuritis cannot always be made. 



EXAMINATION OF THE SENSORY FUNCTIONS 185 

Optic neuritis — as well as choked disc — usually occurs bilaterally, pri- 
marily in pathologic conditions in the anterior areas of the basal cerebral 
membranes (cerebrospinal syphilis and similar processes) with inflammatory 
symptoms. Optic neuritis, however, occurs also in certain diseases of the 
spinal cord (myelitis, tabes, multiple sclerosis) without essential inflamma- 
tory processes, as well as in rare cases of multiple neuritis. Unilateral optic 
neuritis indicates a circumscribed pathologic process implicating only one 
optic nerve. 

When choked disc is recognized, we may feel assured of an intracranial 
increase of pressure. 

This increase of pressure is usually caused by a brain tumor. It may, 
however, also be due to serous meningitis, and in rare cases to an abscess 
of the brain or to hemorrhage. Primary increase of pressure in the posterior 
cranial fossa especially favors the development of choked disc because the 
latter is separated by the tentorium from the remaining parts of the intra- 
cranial space. Here I must call attention to the fact that optic neuritis and 
choked disc may vary greatly in intensity during the course of the disease. 
In a case on which I subsequently held an autopsy I determined the periodic 
and absolute disappearance of choked disc which was due to a glioma of the 
cerebellum, a finding which was confirmed by a prominent ophthalmologist. 

Another change, much more frequently found in neurologic practice than 
optic neuritis and choked disc, is atrophy of the optic nerve. This is mani- 
fest in the ophthalmoscopic picture in that the papilla has either entirely or 
in certain areas lost its beautiful golden yellow color, and is paler than nor- 
mal. The pallor varies between an abnormally light yellow and an almost 
porcelain-like color. If the pallor in atrophy is due to an optic neuritis which 
has run its course, the pallor usually implicates the entire optic nerve disc. 

If, on the other hand, we note that only a portion of the disc is pale, 
usually the nasal half of the inverted picture, genuine primary atrophy is 
indicated, and this in the course "of time may lead to atrophy of the entire 
transverse section. 

Non-neuritic atrophy of the optic nerve will be found in tabes, in pro- 
gressive paralysis, in multiple sclerosis, and in certain general intoxications 
(chronic alcoholism, lead poisoning, nicotin poisoning, etc.). 

The investigation of the function of sight is only of importance to the 
neurologist inasmuch as it is unnecessary for him to determine errors of 
refraction and to test the refractive media. 

The test of the central acuity of vision is in most of our cases of subordi- 
nate importance; it may be made by the aid of the familiar Snellen's tables. 

The examination of the field of vision is much more important in the 
diagnosis. If no perimeter is available, the investigation of the field of vision 
is very inaccurate, but with much practice results may be obtained without a 
perimeter. The patient is placed with his back against the light, one eye 
being covered with his hand. The physician seats himself opposite his patient, 
who is told to rivet his eyes upon the physician's face. Now a light tissue, 
for instance, a folded handkerchief, is waved to and fro, each time at a dif- 
ferent angle from the periphery to the center of the field of vision. When- 
ever the patient, looking into the face of the physician, sees the handkerchief 
approaching, he must say " now." In this way hemianopsia and even con- 



186 GENERAL NEUROLOGICAL DIAGNOSIS 

centric limitations of the field of vision not of too slight degree may be 
determined. 

In examination with the perimeter, a piece of paper about 1 c.c. in size 
is fastened upon a black staff as a testing object. With this we perform 
movements similar to the coarser test with the handkerchief; but, instead of 
moving the test object with the free hand, it is moved upon the firm or rotary 
meridian of the perimeter. In every investigation of the field of vision we 
must be careful to see that the head is kept perfectly motionless, and, if 
necessary, the upper eyelid must be drawn slightly upward with the fingers. 
Moreover, it must be borne in mind that the test object is longer visible 
if it be moved from the center of the field of vision to the periphery than 
when moved in the opposite direction. As a rule, the limits of the field of 
vision are attained by moving the test object from without toward the center. 
If, instead of a white test object, a colored one is used, the field of vision 
for colors may be determined, a process which is indicated when we find pallor 
of the optic nerve. 

While the external limits of the field of vision may, under some circum- 
stances, be determined without a perimeter, this is absolutely necessary for 
the recognition of a dark spot (scotoma) in the center or other region of the 
field. The test object should not be larger than 0.25 c.c. The examination 
of the field of vision necessitates considerable practice on the part of the 
physician as well as no small degree of intelligence and the cooperation of 
the patient. 

Limitations of the field of vision and scotomata are observed in all dis- 
eases combined with optic atrophy; in doubtful cases the limitation of the 
field of vision makes it certain that a doubtful pallor of the optic nerve is the 
expression of beginning atrophy. 

Concentric limitation of the field of vision is also observed in hysteria 
and other neuroses. Functional limitation of the field of vision, unlike visual 
defects in diseases of the optic nerve, are not absolutely constant. Some- 
times the limits of two fields of vision observed at the same sitting do not 
coincide. The most extreme degree of concentric limitation of the field of 
vision which is seen in nervous diseases is furnished by hysteria, and not by 
organic affections. 

Hysterical amaurosis sometimes develops gradually from an extreme visual 
limitation of this kind. 

A peculiar, but also rare, defect in the field of vision is hemianopsia, in 
which one-half of the field of vision is absent. According to the absent half 
of the field of vision in both eyes affecting symmetrical or . non-symmetrical 
halves of the retina we speak of " heteronymous " or " homonymous " hemi- 
anopsia. 

Hemianopsia may readily be diagnosticated without a perimeter. We may 
suspect its existence if the patient complains of defective vision " in one 
eye," also if his sight is poor upon the street, and he frequently " stumbles 
over things." Hemianopsia is a transitory symptom in migraine; usually it 
indicates an organic disease of the brain. 



EXAMINATION OF THE SENSORY FUNCTIONS 187 



EXAMINATION OF THE SENSE OF SMELL 

(Nasal respiration, manner of testing the sense of smell, physiologic hyposmia, 
hyperosmia, central disturbances of smell, the sense of smell in paralysis of the fifth 
and seventh nerves.) 

Examination of the senses of smell and taste is not necessary in all cases; 
as a rule, our attention is directed to these senses by the symptoms. 

Before testing the smell we should ascertain whether both sides of the nose 
are alike permeable to the inspired air. If the nasal respiration is restricted 
to one nostril, the sense of smell on the other side will of course be dimin- 
ished. We must choose as reagents substances which will not simultaneously 
stimulate the fifth nerve and thereby produce abnormal sensations in the 
nose; oil of peppermint, asafetida, watery tincture of valerian, cologne, etc., 
are suitable. The mode of examination is simple: The patient should close 
one of the nostrils with the finger while the substance to be smelled, in a 
small bottle, is held in front of the other. The report of the patient will 
indicate whether or not there is any perception of smell. Some substances 
of disagreeable odor cause a rapid reflex backward movement of the head 
which can scarcely be suppressed. 

In the absence of other local changes (catarrh) a constant and decided 
difference in the sense of smell of the two nostrils on free nasal respiration 
must be regarded as pathologic. Moreover, the differences within physio- 
logic limits in the sense of smell in the same individual — especially the cer- 
tainty and rapidity of perception — are exceedingly great. In some persons 
the sense of smell attains merely a very rudimentary development. 

General hyperscnsilinnicss of smell as well as a general decrease of the 
same, is significant of functional nervous diseases. In hysteria we not infre- 
quently see unilateral anosmia as a partial phenomenon of hemianesthesia. 
Disturbances of the sense of smell which are certainly not functional, espe- 
cially the unilateral, are found occasionally in cases of increased cerebral 
pressure, particularly in pathologic processes in the anterior cranial fossa 
(tumors, meningitis, fractures of the base of the skull, and the like). 

Besides those primarily quantitative disturbances of the sense of smell 
which have been mentioned, there is also a derangement of the sense of smell 
which is usually associated with simple hyposmia. While these patients have 
an appreciation of smell, they are unable to combine with it their memory 
pictures of smell. This phenomenon is partly due to the fact that certain, 
perhaps especially characteristic, components of the sensation of smell are 
decreased or lost, and the others can no longer be recognized. It is also con- 
ceivable that in these central disturbances there is a phenomenon analogous 
to soul-blindness. Paralysis of the fifth nerve sometimes disturbs the sense 
of smell by dryness of the nasal mucous membrane; facial paralysis makes 
inhalation difficult, and thus sometimes prevents the patient from smelling. 

Abnormal sensations of smell are noted in neurasthenics, hallucinations 
and delusions of smell in hysteria, in paralytics, and in epileptics as an aura 
of an attack. 



188 GENERAL NEUROLOGICAL DIAGNOSIS 



EXAMINATION OF THE SENSE OF TASTE 

(The chorda tympani and the glossopharyngeal nerves, the test of taste, individual 
variations in taste, disturbances of the sense of taste.) 

The sensation of taste depends upon the function of several nerves. In 
the anterior portion of the tongue the nerve of taste is the fifth nerve, or its 
branch the lingual, which by a circuitous path — chorda tympani, trunk of 
the facial — hrings the sensory fibers from the third to the second branch of 
the fifth nerve. The glossopharyngeal nerve transmits the function of taste 
to the posterior portion of the tongue and the palate. A perfectly normal 
sense of taste is only possible when, besides the proper function of the two 
nerves mentioned, the sense of smell is not appreciably disturbed patho- 
logically. 

Taste is best investigated in the following manner: The patient is told 
to protrude the tongue, and we observe whether it is free from coating. Dur- 
ing the whole examination the tongue is to remain protruded. 

The patient informs us by writing upon a piece of paper what sensations 
of taste he has experienced. Upon this paper the four qualities of taste are 
written : Sweet, acid, salt, and bitter. If the patient is conscious of no taste 
he makes a negative motion with his head. He is not permitted to speak 
during the examination for, by retraction of the tongue within the mouth, 
the testing substance may be distributed over the entire tongue. I must call 
attention to the fact that the sense of taste in different individuals varies 
greatly in degree as well as the relative implication of the tongue and the 
palate. 

With a glass rod or a small dropper or pipette one drop of a strong sugar 
solution is distributed over the edge of one side of the tongue. The patient 
must indicate the sensation of taste by making signs with his finger. The 
same process is repeated upon the symmetrical other half of the tongue. By 
nodding his head, the patient may report whether the taste on both sides of 
the tongue is uniform or not. The mouth should be cleansed with a little 
water, and an examination be made with other reagents. The best substances 
for this purpose are the following : Table salt, vinegar, and tincture of quinin. 
The solutions should not be so strong as to produce a burning sensation in the 
tongue or in the nose. On account of the persistence of the taste, solutions 
of quinin should be employed last, and after using vinegar it is well to pause 
a short time before continuing the investigation. 

Apparently there are some individuals who, even under normal circum- 
stances, have no perception of taste while the tongue is protruded. In these 
cases, after distributing the fluid on one side of the tongue this organ is dried 
and drawn back into the mouth. The distribution of the substance over the 
entire tongue is thus prevented, but, on the other hand, the palate becomes 
implicated in the function of taste. 

A bitter taste is said to be chiefly transmitted by the posterior portion 
of the tongue, and this area should be tested in the same way as the anterior 
part. In hysteria there is unilateral and bilateral absence or diminution of 
taste. Among the organic conditions in which disorders of taste occur, we 
must mention affections of the facial nerve in the Fallopian canal, diseases 



EXAMINATION OF THE SENSORY FUNCTIONS 189 

of the fifth nerve, and paralysis in the course of the peripheral glossopharyn- 
geal nerve or its nuclear region. 

Perverted taste is frequent in paralysis ; but many neurasthenics and hypo- 
chondriacs also complain of abnormal, and usually unpleasant, sensations of 
taste. 

A disturbance of taste can be strictly differentiated only after it has been 
several times demonstrated by examination. 



EXAMINATION OF THE SENSE OF HEARING 

(Test of air and bone conduction, Rinne's test, disturbances of hearing and equi- 
librium combined, diminution of the power of hearing, atrophy of the auditory nerve, 
hysterical deafness, commotio labyrinthi, subjective auditory sensations.) 

In the following I shall briefly outline the examination of the organ of 
hearing, and shall mention those points most important to the neurologist. 
In all doubtful cases, and whenever the symptoms referable to the ear and 
the function of hearing are decidedly important in a neurologic sense, the 
ear should be examined by an otologist. 

This is particularly true when there is a possible affection of the labyrinth 
or, in a restricted sense, a nervous defect in hearing. 

The examination of the ear should begin with an inspection by the spec- 
ulum and the reflector, then we test the function by the conduction of air. 
The patient closes one ear with the finger tip, and, standing at his other side, 
we whisper into his open ear with great distinctness and at varying distances 
clear vowel sounds, such as " papa," etc. Then we determine in meters the 
maximal distance at which whispered words are still heard. The other ear 
is then examined in the same way. The employment of a watch for this test 
instead of the whispering voice is not advisable. 

Normal conduction of air is only possible when there is no noteworthy 
change in the conduction apparatus of sound (auditory canal, tympanum, 
tympanic cavity, and Eustachian tube). For the neurologist, the next step 
in the examination of the ear is the determination of the bone conduction 
(craniotympanal conduction). This conduction through the bone is tested 
by striking a deep sound with a tuning fork, which is then placed upon the 
middle line of the skull (for a comparative test of the ears), or upon the 
mastoid process (to test one ear). The patient must state whether he hears 
the sound, and upon which side he hears it. It must, however, be remarked 
that in determining bone conduction the coaction of air conduction cannot be 
fully excluded. 

Normally the tone of the tuning-fork is heard longer through air con- 
duction than through bone conduction. Therefore, as soon as the tone pro- 
duced by bone conduction has died away the tuning-fork is to be held in 
front of the ear, and the patient should report whether the tone which disap- 
peared through the bone is heard anew through the air. 

The so-called Rinne's test depends upon the simultaneous action of air and 
bone conduction as described. Its normal coincidence, i. e., longer perception 
by air conduction, is called a positive result, while the prolonged duration 
of bone conduction is termed a negative result. 



190 GENERAL NEUROLOGICAL DIAGNOSIS 

A positive, i. e., a normal, result of the test favors a nervous or labyrinthine 
defect in hearing, while a negative result, on the contrary, indicates that 
the seat of the disease is in the apparatus of sound conduction. 

A combination of defective hearing with disturbance of equilibrium always 
favors an affection of the middle ear, provided there is no intracranial disease. 

In all cases in which disease of the middle ear is diagnosticated, the deci- 
sion as to whether the labyrinth or the auditory nerve itself is diseased depends 
upon other and usually quite fruitless- observations. 

It is very difficult to utilize the simple data obtained in a case of defective 
hearing. In the present state of diagnosis of the diseases of the ear, the 
results of examination, particularly Rhine's test, must be used with great 
caution. We must remember that in advanced age many persons — according 
to Troltsch about one-third — have defective hearing, at least in one ear. 

Organic pathologic processes, aside from hemorrhages and fractures of the 
base of the skull, consist chiefly of neoplasms and inflammatory processes in 
the posterior cranial fossa and at the base of the brain which are mostly 
unilateral and produce difficulty in hearing. An actual degeneration of the 
auditory nerve takes place in tabes dorsalis; multiple sclerosis, by one of 
its foci, may destroy the auditory nerve. 

Hysteria may produce defective hearing or deafness, especially if it be 
of extreme degree. The diagnosis of hysterical difficulty in hearing and deaf- 
ness may be very perplexing if there are no other hysterical stigmata and 
there is no anesthesia in the region of the deaf ear. The prognosis of hys- 
terical deafness is doubtful. In some cases of hysteria hyperacusia is said 
to have been observed. 

Of other non-organic affections of the hearing I must mention commotio 
labyrinthi which plays an important role in all injuries of the head. The 
diagnosis of this disorder, frequently met with in practice, is often difficult 
and uncertain. 

Subjective perceptions of hearing and rushing sounds in the ear may 
be produced by focal diseases near the trunk of the auditory nerve, more 
frequently, however, and usually in a most distressing form, by arteriosclero- 
sis of the brain. Hysterical subjective perceptions of hearing are, as a rule, 
neither invariable nor permanent. 

If tinnitus aurium occurs paroxysmally and is accompanied by attacks of 
vertigo we are dealing with Meniere's disease. 

SENSORY CONDITIONS OF THE SKIN 

(The impossibility of determining sensation "objectively," test of tactile sensation 
of the skin, of the pain sense, of slight sensation of pain under physiologic conditions, 
of pain sense~ in the deeper and superficial cutaneous areas in tabes dorsalis and similar 
affections; investigation of ser.sation and of skin and muscle pain with the faradic 
current; awakening of sensation during the test of sensation; temperature sensation 
and the nature of the test; locality sense of the skin; occurrence of sensory disturb- 
ances, sensory disturbances in functional and organic diseases; compensatory property 
of the sensory functions; unequal diminution of various cutaneous sensations; disso- 
ciated sensory paralysis, its occurrence; retarded sensation of pain.) 

The conception and the nature of sensation as a conscious faculty excludes 
the recognition of the nature of sensation otherwise than from the reports of 



EXAMINATION OF THE SENSORY FUNCTIONS 191 

the patient. Even those cutaneous reflexes which certainly pass through the 
cerebral cortex are not an absolute and reliable index of sensation, for such 
a cutaneous reflex may sometimes be produced without conscious sensation. 
We see, therefore, the diagnostic value of the patient's reports in this ex- 
tremely important part of the examination, and consequently, in investigating 
sensation, we should refrain from influencing the expected replies of the 
patient. This is all the more necessary as the patient is most susceptible to 
suggestion when describing sensations. 

A complete test of sensation must include the skin, the various qualities 
and the sensibility of the deeper portions of the mucous membranes, and espe- 
cially the joints and the muscles. 

In practice, the employment of complicated instruments is rarely neces- 
sary; rarely is more required than a small brush, a pin, or similar object. 

The cutaneous sensation is best tested by lightly touching symmetrical 
areas at either side of the body — first the head and face, then the trunk and 
the extremities — with the point and then with the head of a pin. It is always 
advisable to stimulate two or three times in rapid succession one or two of 
the small cutaneous areas. By this means we arrive at a general conclusion, 
and protect ourselves from the error of at once deciding upon a disturbance 
in sensation in any area if one incorrect report be given. The normal person 
can always differentiate contact with the point of a pin from that of the head 
of a pin except in those areas of the skin which, like the soles of the feet or 
portions of the palms of the hands, are covered with callus. After each indi- 
vidual touch the patient is to answer — of course without looking — by saying 
" point " or " head/ 5 In comparing the perception of touch in the two sides 
of the body we should only ask: Was the sensation upon the left side the 
same as upon the right? We should never ask whether the sensation was 
stronger upon one side than upon the other. If a difference in sensation 
between the entire right and left sides of the body is reported, we should sub- 
sequently compare the regions and members of that half in which sensation 
is not normal, and limit this difference. If a change in sensation is reported 
in individual members or areas of the trunk, we must ascertain by further 
testing whether this disturbance of sensation is along the course of the 
peripheral nerves, whether it is segmentary, or, finally, whether it is confined 
to limits apparently produced psychically. 

Tests with the pin must sometimes be complemented by the brush; while 
examination merely with a pin is sufficient to demonstrate hemi-anesthetic or 
hemi-hyperesthetic regions over the entire body it does not suffice for the 
exact determination of limited sensory disturbances; a gentle touch with a 
fine hair brush, a piece of cotton, or the like must here complete the inves- 
tigation of tactile sensation. On the other hand, hyperesthesia cannot be 
differentiated with the brush; the point of a pin is much more effective. 
How far the sense of pain is to be taken into consideration in testing with 
a pin cannot here be stated: A test of the sense of pain should never be 
omitted, for a disturbance of this sense usually escapes the notice of the 
patient and even the physician if he does not particularly investigate it. The 
degree of pain sense, as I shall later explain, is extremely valuable in the 
diagnosis. 

The pain sense is tested either by pinching the skin, or by placing the 



192 GENERAL NEUROLOGICAL DIAGNOSIS 

point of a pin not too gently upon the skin and observing whether the pain 
is sufficiently intense, and is experienced at once. A delay in the perception 
of pain is easily recognized. In utilizing these tests we must remember that 
the sensation of pain in different individuals varies greatly within physiologic 
limits. I have observed it to be especially slight . in fat persons poor in 
pigment. 

Moreover, it must be borne in mind that the sense of pain is not the same 
in different portions of the body. 

After demonstrating that the superficial cutaneous areas react painfully 
to the prick of a pin, we still lack proof of the perfect intactness of the pain 
sense. For it is a remarkable fact that the pain sense of the deeper cutaneous 
areas and layers of the skin may be greatly decreased, yet the patient will 
experience pain from the mere prick of a pin. 

For instance, after we have stuck the point of a pin through the skin 
we can often move it to and fro without the patient experiencing any pain, 
while the slight prick in the upper cutaneous areas has caused the usual 
degree of pain. This condition is occasionally seen in hysteria, but in my 
experience much more frequently, and in the majority of cases as one of the 
first symptoms, in tabes dorsalis and paralysis. If, in these cases, we prick 
with a pin only the superficial cutaneous layers we often find nothing patho- 
logic. 

In all of these tests it must be borne in mind that the intensity of the 
pain produced is less if the pin be rapidly introduced. 

The previously described methods of testing sensation without an instru- 
ment may be supplemented by the aid of an electric induction apparatus and 
thus completed. The results of different examinations may then be regis- 
tered and readily compared with each other. A large, moistened, flat elec- 
trode is placed upon whatever point in the median line or at the side of the 
body is to be examined. The other pole, armed with a brush or a flat metal 
electrode, is placed upon the area to be examined. The current should be 
closed and then slowly increased, and the patient instructed to report when 
he feels a slight quivering sensation (the equivalent of contact sensation), 
after which the current is still further increased until he experiences pain. 
With this method it must remembered that the pain produced by a strong 
faradic current does not resemble that produced by the prick of a pin or 
by pinching; for while the latter is felt only in the skin, a strong faradic 
current, besides producing pain in the skin, also makes the muscles painful 
by causing strong contractions therein. This is the key to the apparent 
contradiction noted in the results occasionally obtained when the pain sense 
is tested with a pin and with the electric current. When the muscles do 
not react to faradism, even strong currents often produce no pain; but test- 
ing with a pin may prove that the cutaneous sensibility and the pain sense 
are intact. 

The variation of a few milliamperes of current in different tests is not at 
all remarkable, for the apparatus may have changed, or the degree of mois- 
ture in the skin may vary; but the results of even a single examination may 
also vary. Here a highly important law, operative in all sensory investi- 
gations, is manifested: That, as the resistance of the skin to the galvanic 
current is gradually decreased to a certain extent during the application of 



EXAMINATION OF THE SENSORY FUNCTIONS 193 

the current, so is the sensibility of the skin increased during, and by, every 
sensory examination. 

Of the other important sensory qualities of the skin only the temperature 
sense remains to be examined. This is tested by touching the skin alter- 
nately with a warm and a cold substance, with the wood or the metal of the 
percussion hammer, with cold water (about 5° C), or with hot water (about 
20° to 30° C.) contained in test-tubes. The water should be neither very 
cold nor very hot ; otherwise, pain may be produced. The patient, whose eyes 
should be closed, must state with which tube he has been touched. 

Here it will be noted that the stimulation from the touch of one and 
the same hot or cold application in different portions of the normal body 
is perceptible in varying degrees (according to Goldscheider's examina- 
tions). 

The sense of locality in the skin, i. e., the consciousness of the region 
in which definite cutaneous stimulation occurs, immaterial whether by tactile 
pain or a temperature effect, need not, as a rule, be investigated. If, with 
these methods, the patient states what point has been stimulated, this is usu- 
ally sufficient. If it is necessary to investigate the locality sense somewhat 
more minutely, simple figures, circles, lines, etc., should be drawn with the 
finger upon the skin, and the size, outline, and nature of the figures should 
be described by the patient. 

Disturbances, usually diminution, of the various qualities of cutaneous 
sensation may occur when the sensory conduction from the periphery to the 
cerebrum is at any point organically interrupted or damaged. There are 
also purely functional disturbances, which, as a rule, are more conspicuous 
than the organic ones. They appear either as general or unilateral anesthesia 
of hyperesthesia, limited to extremities or portions of extremities functionally 
belonging together. Anesthesia in areas of functional nature is rare, but 
hyperesthesia of certain areas is common. Organic disturbances of the sensi- 
bility of the skin follow the peripheral nerves or the distribution of the spinal 
roots (radicular type) according to their seat and area of distribution. Hemi- 
anesthesia or hemi-hyperesthesia of organic nature is rare, and points to a 
disturbance of the central sensory tracts, the lemniscus, or the posterior parts 
of the capsular region. As already indicated, in the sensory sphere there is 
an extraordinary functional faculty of compensation. From this originates 
the important diagnostic law never to conclude because sensation is unim- 
paired that the sensory tract under consideration is intact. When the func- 
tion of the peripheral sensory fibers is lost the sensory disturbance is never 
distributed in its full intensity over the entire area innervated by the diseased 
nerve. 

The question now arises: Are the various qualities of cutaneous sensation 
invariably the same because always implicated in a uniform manner, or, as 
a rule, is only one sense of the skin, perhaps the tactile, implicated, while 
the other functions are normal or implicated to a much less extent? This 
question cannot be briefly and generally answered either affirmatively or nega- 
tively ; for the one as well as the other condition may be observed. In nervous 
diseases of peripheral origin all sensory functions of the skin are uniformly 
diminished ; in tabes dorsalis, in paralysis, and in syphilitic spinal disease the 
pain sense is usually more markedly implicated than the tactile sense, but 



194 GENERAL NEUROLOGICAL DIAGNOSIS 

here also there may be a uniform diminution of all the qualities of cutaneous 
sensation. 

Hysterical disturbances of sensation in the skin, when distributed over 
the entire body, are more frequently manifested by the implication of the pain 
sense alone than of both the pain and tactile sense. He mi-anesthetic and 
monoplegic hysterical disturbances generally involve the pain sense and all 
other cutaneous sensations; in fact, they form one of the most marked sen- 
sory disturbances with which we are acquainted. 

From what has been stated it is evident that patients may show the great- 
est variety of disturbed cutaneous sensations either in combination or disso- 
ciated. Usually, however, we understand by dissociated sensory disturbance 
(" dissociated sensory paralysis ") a definite disturbance; i. e., the tactile sense 
of the skin may be retained while the pain and temperature senses may be 
greatly diminished or entirely absent. In such cases the point of a pin intro- 
duced far under the skin is distinctly felt, but is not painful. " Dissociated 
sensory paralysis " is found most invariably and conspicuously in syringo- 
myelia. It is also found, less marked but much more frequently than is usu- 
ally assumed, in neuritic processes, in tabes, and in paralysis, as well as in 
alcoholic and hysterical persons. In the aged, the leg covered with varices 
often shows certain dissociated sensory paralyses, and a similar condition is 
occasionally observed in normal persons who are obese. 

Before discussing sensation in the muscles, the bones, and the joints, we 
must mention another rare phenomenon of cutaneous sensation : Delayed pain 
sensation. This phenomenon appears during the test if the patient is told 
to say " now " as soon as he is aware of contact, and to say " oh " as soon 
as he experiences pain. Tabetics who exhibit retarded pain sensation during 
this test immediately respond to a pin prick in the skin with " now/' and after 
some time, often one or two seconds, they say " oh," showing that they have 
experienced pain. 

SENSIBILITY OF THE DEEPER TISSUES 

(The importance of sensation for motility, examination of sensation in passive 
movements, test of the stereognostic sense, of the sensation of tugging, vibratory sen- 
sation, disturbances of sensation in the deeper areas.) 

The examination of the sensation of the deeper parts, the muscles, liga- 
ments, tendons and joints, is important because only by the combined action 
of the intact sensory conditions are the previously mentioned portions of 
the motor apparatus rendered capable of exercising their complete activity, 
particularly their faculty of locomotion. This shows that in disturbances 
of the finer movements the sensibility of the deeper parts must always be 
examined. Unfortunately we are unable to test the sensation of the indi- 
vidual parts separately, since we only possess methods for testing certain 
sensory complexities, in which normally the individual sensory components 
of the deeper parts are usually intact. These sensory complexities which 
may be easily tested are the following: The sensation of the position of the 
limbs, the exact sensation of active or passive movements, sensation for the 
amount of pulling exerted upon a limb, and, finally, the stereognostic sense, 
i. e., the faculty of perceiving by the touch, without the aid of any other sense, 



EXAMINATION OF THE SENSORY FUNCTIONS 195 

the shape and size of an object (for instance, an object placed in the hand). 
All of the previously mentioned sensory qualities are derived from the sensa- 
tion of the deeper areas. Only in the stereognostic sense does the sensibility 
of the skin as well as the sensation of deeper areas become of marked impor- 
tance. It is true that in the other previously mentioned symptom-complexes 
it is impossible entirely to exclude cutaneous sensation. 

The investigation had best be conducted in the following manner: After 
the patient closes his eyes, an arm or leg (the latter if he is in the recumbent 
posture) is slowly and steadily moved from its normal position of rest into 
another position, for instance, the limbs are crossed, or they are flexed at 
a certain angle, etc.; we then ascertain whether the patient is distinctly con- 
scious of the nature and direction of the movements which have brought his 
limbs into this final position, or position of rest. A normal person will dis- 
tinctly recognize this. Passive movements in the various joints of the ex- 
tremities must naturally be modified according to the anatomical relations of 
these joints. The effect of gravity must be excluded by a proper position. 
If the elbow-joint, the wrist-joint, the knee-joint, or the ankle-joint is to be 
moved, this area should be supported above and below the joint by the hands, 
and a mild but uniform rotary pressure should be exerted while passive move- 
ment of the joint is slowly performed. It usually suffices if this test is per- 
formed with the fingers or toes, as this enables us to detect any disturbance 
that is present. It is a mistake to bring about the movement by supporting 
merely the tip of the toe or the finger ; on the contrary, in testing the fingers 
the joint to be moved should be so encircled by two or three of the physician's 
fingers above and below the joint as to produce uniform pressure and cutaneous 
irritation on all sides — in the toes this is sometimes very difficult. In this 
position we attempt slight flexion, extension and lateral movement of the 
fingers or toes. In examination of the foot the toe which is to be moved 
must not come into contact with the other toes. Unintelligent persons must 
be instructed not to make any active movement of the joints to be tested. 
If, in spite of this, the patient constantly produces muscular contractions, this 
almost certainly proves that the sensory sphere is affected; for the patient 
who has not fully understood the passive movement will instinctively try to 
correct his incomplete perception by the aid of active muscular contractions. 
From the sum of the resistance experienced in these active movements (pro- 
duced by the hand of the investigator) he endeavors to estimate the change 
in position passively produced. 

A normal person recognizes the slightest passive motion in the fingers. 
But in the toes, even a healthy person may occasionally, on the first attempt, 
confound the third with the fourth toe. In no instance should we base our 
conclusions upon a single answer which may be incorrect. 

Testing the "stereognostic sense/' the combination of cutaneous sensation 
and sensation of the deeper parts is also quite simple. The patient, whose 
eyes are closed, is handed some familiar object, a lead-pencil, a knife, a coin, 
or the like, and is asked if he recognizes it. The normal person will do so 
immediately. If the patient under examination feels the object, or takes it 
first in one hand and then in the other, it proves that his power of recog- 
nition is defective. If the stereognostic sense is greatly impaired, tne object 
may be taken from the hand of the patient without his knowledge. As a 



196 GENERAL NEUROLOGICAL DIAGNOSIS 

rule, the patients themselves are conscious of this disturbance of the stere- 
ognostic sense, but refer it to the motor sphere. Like ataxic patients, they 
usually complain of " weakness " in the hands. 

These methods of examination usually suffice in practice. A number of 
instruments and apparatus for more exact tests are described in physiologic 
text-books. For clinical purposes there is one instrument which enables us 
to test the patient's ability to estimate correctly the force exerted when 
we pull his extremities. This apparatus for testing the lower extremities 
consists of two stockings, in the heel of which there is a pocket in which a 
ball about the size of an apple is placed. The stockings are pulled on like 
ordinary stockings and, while the patient's eyes are closed, we put into the 
pockets a few of the balls which are of the same size, but of different weight. 
The patient must now estimate the relative weight of the different balls. 

There is another method for investigating deep sensation which, on account 
of the uncertainty of its results, has not been generally adopted for clinical 
purposes. 

If a large, deep-toned tuning-fork is placed on a part of the body, espe- 
cially where the bone lies close to the skin, the person experiences a, peculiar 
buzzing sensation which, according to Egger, arises from the periosteum, the 
ligaments, and the capsules of the joints, and which Seiffer and Rydel have 
designated " vibratory sensation." Most likely the skin is also involved in the 
perception of vibrations. This vibratory change is noted in diseases of the 
sensory tract, and is sometimes found when all other sensory qualities are 
implicated. Sometimes, however, the disturbances of the vibratory sense are 
of elective character, inasmuch as they accompany derangements of the sense 
of location, also those of passive movements, and the pain and temperature 
senses. 

Disturbances of deep sensation, which may be investigated by passive 
movements with closed eyes as well as by testing the stereognostic sense, may 
appear in combination or singly, like the disturbances of the vibratory sense 
and disturbances of cutaneous sensation. With disturbances of cutaneous 
sensation, they form a common occurrence in tabes dorsalis. 

In peripheral diseases, as a rule, deep sensation is deranged only in the 
most severe cases when several nerves of an extremity are simultaneously impli- 
cated. 

Among the cerebral affections, disturbances of the sense of location and 
of the stereognostic sense are most frequently met with in those of the cere- 
bral cortex; here they occur isolatedly, without a disturbance of cutaneous 
sensation. Positive disease of the brain stem with the same symptoms has, 
however, been described. 

Sensation of the deep parts is often disturbed in hysteria; usually there 
are also cutaneous disturbances of sensation; in rare cases these are absent. 



EXAMINATION OF THE REFLEXES 

THE TENDON REFLEXES 

(Classification of the reflexes, jaw reflex, triceps reflex, forearm periosteal reflex, 
patella reflex; manner of testing the patella reflex, difficulties of its production, its 
exhaustion and strength; patella clonus; Achillo-tendon reflex, manner of testing it; 



EXAMINATION OF THE REFLEXES 197 

spontaneous production of foot clonus; mechanism of tendon reflexes and their disturb- 
ance, disturbance of tendon reflexes by disease within the reflex arcs themselves, increase 
of tendon reflexes by disturbances external to the reflex arcs, absence of tendon reflexes 
in disturbances above the reflex arcs, in shock to the spinal cord, from purely mechan- 
ical causes ; hysterical clonus and " pseudo-clonus." ) 

According to whether reflex movements are produced by stimulating a 
tendon, or the skin and mucous membranes, or by the irritation of certain in- 
ternal parts, we differentiate three varieties of reflexes : The tendon reflexes, 
the cutaneous reflexes, and the " internal reflexes." 

The simplest, and at the same time the most important of tlese, are the 
tendon reflexes. From the extraordinarily large number of tendon reflexes 
which may be normally produced in man only the following need, as a rule, 
be tested : 

The Jaw Reflex. — This is produced by placing upon the lower incisor 
teeth of the slightly opened mouth a spatula or something similar which ex- 
tends from 1 to 3 cm. into the oral cavity, the greater portion of it pro- 
truding from the mouth. We hold the projecting end, and tap with the 
percussion hammer upon the upper surface of this portion. By this move- 
ment the tendons of the muscles which elevate the lower jaw are suddenly 
stimulated, the muscles react by a brief contraction, and the lower jaw is 
slightly raised. 

Triceps Reflex. — Physiologically considered the triceps reflex is homolo- 
gous to the most important of all reflexes, the patella tendon reflex. The 
latter is produced by the action of the extensor tendon of the leg, the former 
by the action of the extensor tendon of the forearm. The clinical importance 
of the triceps reflex is by no means equal to that of the patella tendon reflex. 
To test the triceps reflex the forearm is loosely flexed, the internal condyle 
being lightly supported, and we tap with the hammer against the tendon of 
the triceps. As the tendon of the muscle is very short, it is best to ascertain 
by palpation just where the muscle terminates and the tendon begins. If we 
neglect this, we are liable to strike with the hammer the muscle instead of 
the tendon, and thus produce a direct and mechanical muscle stimulation 
instead of the reflex. The movement which follows stimulation of the tendon 
is a quick but very distinct extension of the forearm. 

By tapping in the region of the styloid process of the radius we may pro- 
duce a reflex — probably conducted from the periosteum — which consists in a 
slight flexion of the forearm. This reflex is of no clinical significance. 

Patella Tendon Reflex. — The most important tendon reflex is the patella 
tendon reflex. It may be evoked with the patient in either the recumbent or 
sitting posture; whether the test had best be performed in one or the other 
position, must be decided in the individual case. Where the test is doubtful 
we should try both positions. In the sitting posture, the legs must either be 
flexed at an obtuse angle or one must be loosely crossed over the other, the 
muscles being flaccid. We must always be sure that the muscles, particularly 
those on the flexor side of the thigh, are actually relaxed. 

Now the physician gives a quick and not too forcible tap at about the 
middle of the quadriceps tendon below the patella. The normal response to 
this tap is a slight extensor movement of the leg, and this at once ceases. 

Simple as the production of this reflex is in most cases, in others it may 



198 GENERAL NEUROLOGICAL DIAGNOSIS 

cause great difficulty. The most important part of the process we have already 
indicated: All muscle tension in the legs must be prevented. This is best 
done by diverting the attention, by talking to the patient, by giving him arith- 
metical problems, etc. The diversion of the motor impulse into other tracts 
may also aid us. For this purpose the patient may grasp the hand of the 
physician, or he may lock his fingers together and pull with all his might 
as if to tear them apart but without detaching them (Jendrassik's grasp). 
If, notwithstanding this, the patient does not relax — and in hypochondriacs 
this is not rare — we sometimes best attain our purpose by having the patient, 
instead of sitting on a chair or in bed, sit upon a table in such a way that 
his legs swing like a pendulum. Or, while the patient is sitting quiet and 
the physician is apparently investigating another part of the body, the exam- 
iner quite unexpectedly taps the quadriceps tendon. 

If this is not successful, we may sometimes produce the reflex while pal- 
pating the tendon. One hand is placed over the patella in such a way that 
the tips of the fingers touch the ligament, and then we tap lightly with the 
hammer the finger which rests on the tendon. If there is the slightest con- 
traction of this muscle, it is at once felt by the finger over the tendon. Some- 
times it is advisable, instead of tapping the middle of the tendon, to tap a 
lateral portion. We should never pronounce the patella reflex to be absent 
until all of these maneuvers have been tried, if necessary repeatedly. Re- 
markable as it may appear, it is nevertheless a fact that even experienced and 
skilful investigators have erred in assuming the absence of the patella tendon 
reflex, while others, more patient, have succeeded in eliciting it. It is some- 
times difficult to form an opinion as to the presence or absence of the patella 
tendon reflex because the feeble reflex produced upon the first attempt does 
not subsequently occur. In such cases (multiple neuritis, myelitis, incipient 
tabes) the reflex is abnormally exhausted. 

The normal strength of the tendon reflex is very difficult to determine. 
Generally, the tip of the foot moves about 10 cm. on extension of the lower 
leg. In children the normal reflex is more active. 

The patella reflex may be increased in varying degrees : The leg may show 
a single but greater excursus than normal; instead of responding by a single 
movement, there may be several contractions steadily decreasing in strength. 
With an extreme increase in the reflex, a tap even in the vicinity of the patella 
tendon produces a reflex-like contraction. We must differentiate from this 
the half voluntary, half reflex movements (flexion of the leg, adduction of 
the thighs, trunk movements) which appear in very irritable patients when 
we suddenly tap for the patella tendon reflex. Hysterical patients, particu- 
larly neurasthenics, on the production of the reflex often experience a very 
unpleasant feeling of irritation throughout the entire body. 

Whenever we find the patella tendon reflex increased, we must try to 
elicit the so-called patella clonus. For this purpose the patient should lie 
upon his back with the legs extended ; there must not be the slightest tension 
of the quadriceps. 

The patella clonus is produced by grasping the patella firmly with the 
thumb and index finger while the root of the hand and the forearm are placed 
upon the thigh. Suddenly, with an energetic jerk, the patella is brought 
straight downward without, however, permitting it to slip from between the 



EXAMINATION OF THE REFLEXES 199 

index finger and thumb. If there is clonus of the patella, this will be repeat- 
edly drawn up by the action of the quadriceps muscle while the investigator 
tries to hold it down. An almost continuous, vibratory movement of the 
patella is produced, this being quite uniform and persisting so long as the 
physician draws the patella downward. This physio-pathologic process corre- 
sponds exactly to that in the production of the simple patella reflex : By pull- 
ing the patella downward — just as when we tap with the hammer upon the 
tendon — the tendon itself is somewhat elongated, and there is simultaneously 
an irritation of the sensory fibers of the tendon. This reflex stimulation leads 
to a contraction of the quadriceps; the patella rapidly moves upward, but is 
again immediately drawn down by the hand of the examiner. The sensory 
irritation from this downward tug produces simultaneously a reflex in the 
tendon, and the reflex contraction of the quadriceps which at once appears 
and again draws the patella upward. This action is repeated as long as the 
hand of the physician draws the patella downward. 

In producing the patella clonus we must be careful to press the patella 
down with the tips of the index finger and thumb and not with their basal 
phalanges, for if we encircle the patella with the angle formed by the basal 
phalanges of the thumb and index finger, in grasping the patella we involun- 
tarily touch the leg of the patient with the free distal phalanges of the thumb 
and other fingers and by a too firm grasp prevent the tugging of the patella. 

A phenomenon similar to patella clonus occurs when the quadriceps is vol- 
untarily, although unconsciously, made tense (as by hysterics and neuras- 
thenics). In this case also a to and fro movement of the patella may appear, 
but it is never the true patella clonus (pseudo-clonus). 

Among the important tendon reflexes is the tenclo Achillis reflex. This 
may be tested by grasping the tip of the patient's foot when he is in the 
recumbent posture, forcing the tip and the foot slightly upward, and at the 
same time flexing the leg at the hip and knee-joint, and then delivering a 
blow with the plexor upon the posterior of the passive and tense tendo Achillis. 
The tendon responds with a brief contraction of the muscles appertaining to it, 
and the tip of the foot moves downward. This may also be tested while 
the patient is lying upon his abdomen; he must so relax the muscles that 
the physician can flex the leg slightly toward the thigh, and then tap the 
tendon; or the patient may kneel upon a chair in such a way that his feet 
extend beyond the edge of the chair, and, while in this position the physician 
taps the tendon. 

In producing this reflex the main thing is for the patient to relax his 
muscles completely, and with ignorant or very irritable patients this result 
is more difficult to attain than the production of the patella tendon reflex. 
When the Achilles tendon reflex is greatly exaggerated, it becomes a clonus, 
and the mechanism of this clonus and its analogy to the mechanism of the 
patella clonus may be readily understood when I state that the foot clonus 
is produced by relaxing the muscles of the leg — best with the patient in the 
recumbent posture — and briskly moving the tip of the foot dorsally and 
upward, but not holding it too firmly in this position. As long as the tip is 
forced upward, the foot makes flexure and extensor movements in rapid suc- 
cession. Sometimes the mere placing of the tip of the foot upon the floor 
at a right angle, or strong flexure of the knee is sufficient to produce the foot 



200 GENERAL NEUROLOGICAL DIAGNOSIS 

clonus. In such cases the patients complain that the foot " trembles " when 
it is placed upon the floor. 

Before reviewing those cases in which a change in the tendon reflexes is to 
be expected, I must briefly recall the mechanism of tendon reflexes. The 
stimulation produced by tapping the tendon is conducted centripetally through 
the sensory nerves to the posterior roots of the spinal cord, which it enters. 
Corresponding to the anatomical division of the posterior roots into long tracts 
which pass upward to the nuclei of the posterior columns and short fibers 
which penetrate the gray substance of the cord, that is, the collaterals, a 
part of the sensory irritation which is brought to them becomes the sub- 
stratum for the conscious sensory perception transported upward, and in part 
it forms the foundation of reflex phenomena in the motor cells of the ante- 
rior horn. Those deviations of the posterior root fibers which perform the 
last mentioned function are therefore called reflex collaterals. In the trans- 
verse section of the spinal cord they extend in arches from the port of entrance 
of the root into the anterior horn. These, as we have previously seen, belong 
to the elements which are capable of producing power in the anterior horn 
cell. If the stimulation applied to the tendon of the muscle extends to the 
anterior horn in the manner described, the motor cell sends a current of 
power downward through the anterior spinal cord root into the peripheral 
motor nerve. By means of its terminal plate the latter transmits the stimu- 
lation to the muscle, and this responds with a single rapid contraction. This 
reflex process may be regarded as a typical example of tendon and periosteal 
reflexes. 

The reflex processes may be quantitatively disturbed when there is a dis- 
turbance at any point of the reflex tract. In diseases of the sensory end- 
organs or of the sensory nerves, the reflex is diminished or absent, for the 
stimulation cannot be transmitted to the anterior horn cells which form the 
reflex center (marked cutaneous changes, especially thickening, neuritis, or 
the severance of nerves). In diseases of the posterior roots and in destruc- 
tion of the reflex collaterals (tabes dorsalis or hemorrhages into the poste- 
rior portion of the transverse section) this causes the reflex to be diminished 
or absent. The same is true of all pathologic processes which destroy the 
cells of the reflex center in the anterior horn (spinal muscular atrophy, cavity 
formation, hemorrhages, inflammatory processes in the anterior horn, etc.). 
In slight affections, particularly in functional disturbances, of the anterior 
horn cells, the tendon reflexes are increased rather than decreased: For in- 
stance, in intoxications (strychnin), particularly when there is general hyper- 
irritability of the gray substance in the central nervous system, in hysteria, 
in neurasthenia, and in nearly all of the so-called functional nervous affec- 
tions, also in anemic and other general debility, in the stage of convalescence 
from internal diseases, and in many similar conditions. 

If the descending portion of the reflex arc — anterior spinal root, motor 
nerve, muscle — is diseased, corresponding to the severity of the affection, 
there is generally a decrease or even absence of the tendon reflex. At the 
onset of some diseases, as in the so-called " irritable stage " of neuritis, the 
reflex is said to be increased. It is difficult to decide whether this increased 
reflex in certain stages of neuritis is to be attributed to an increased irrita- 
bility of the motor nerve or to concomitant secondary phenomena. In severe 



EXAMINATION OF THE REFLEXES 201 

cases of motor neuritis or other grave affection of the motor nerve, as well 
as in the degeneration of muscular tissue, and when there is loss of contractile 
substance (dystrophy), the tendon reflex is absent. It follows from what 
has been stated that in organic diseases within the reflex arc the reflex is 
generally diminished or absent. 

We have seen that the source of motor power for the tendon reflex, the 
anterior horn cell of the spinal cord, is not an absolutely substantive organ; 
on the contrary — aside from other considerations — it is in a high degree de- 
pendent upon the brain, and is constantly influenced by this organ in its 
production of power. We have also seen that with a disturbance of the relation 
between the anterior horn cell and the brain, the continuous output of power 
from the anterior horn cell, which normally is strictly limited to the produc- 
tion of so-called muscle tonus, is increased, and leads to hypertonia and spastic 
conditions, and thus markedly changes not only the rest condition of the 
muscles but also the voluntary movements of the body. 

An analogous change occurs in the tendon reflexes as soon as ttie connec- 
tion between the anterior horn cells and the brain is interfered with. Here 
also there is a tendency on the part of the uncontrolled anterior horn cell to 
a waste of power: The power sent to the descending reflex arc is abnormally 
great, the tendon reflex is increased, not rarely there is clonus. This increase 
of tendon reflex in disease of the pyramidal tracts which connect the brain 
and anterior horn of course occurs only when the diseased focus is situated 
centrally from the spinal reflex center. It is immaterial in this whether 
disease of the pyramidal tract originates in the spinal cord itself (myelitis, 
spastic spinal paralysis, syringomyelia, etc.), in the medulla (bulbar hemor- 
rhages, tumors, etc.), or in the brain (cortical and sub-cortical pathologic foci 
of various kinds, capsular processes, apoplexy, pontine diseases, or those situ- 
ated in the posterior cranial fossa, etc.). This increase of tendon reflexes 
by implicating the pyramidal tracts in any part of their course might well 
be designated classical increase of tendon reflex, since it represents one of the 
most important and most common clinical phenomena. When it appears 
bilaterally, it may sometimes be confounded with a functional and general 
increase in the reflexes; on the other hand, when unilateral it is invariably 
uniform, and by its peculiarity may be recognized as an organically produced 
reflex disturbance. 

I cannot deny that an increase of reflexes in disturbances in the course 
of the pyramidal tracts is not the absolute rule. Tinder some circumstances, 
the tendon reflex, instead of being increased, wholly disappears. This is the 
case in some congenital affections, or those acquired in early youth, usually 
those in which there are cerebral (porencephalic) foci; but this phenomenon 
is most common when there is complete destruction of the transverse section 
of the spinal cord, even when this takes place far above the reflex arc. At 
first sight this paradoxical condition of the tendon reflexes appears to nullify 
our whole diagram, previously given. Subsequent investigations, however, 
have furnished conclusions which bridge over or at least qualify this contra- 
diction, either from the fact that with complete and high severance by trans- 
verse section the tendon reflex is absent, because certain hypothetical " reflex 
tonic" fibers pass from the cerebellum to the spinal cord — therefore fibers 
which stimulate the reflex are destroyed — or because even with high-seated, 



202 GENERAL NEUROLOGICAL DIAGNOSIS 

long existing, transverse lesions the gray substance of transverse sections of 
the spinal cord much deeper situated are nevertheless damaged — at least func- 
tionally — so that the stimulation travelling from the reflex collateral to the 
anterior horn no longer meets a cell which is absolutely capable of transmitting 
reflexes, although possibly it is otherwise still capable of function. This 
latter view, which assumes a disease descending from the transverse lesion 
to the gray anterior horn substance (its effect upon the tendon reflexes being 
perhaps analogous to that of poliomyelitis), has been confirmed by autopsies, 
but still fails to satisfy us. A tenable theory should assume that when the 
tendon reflexes below the transverse lesion are absent the tendon reflexes 
above this lesion should also be absent. For if the tendon reflex is absent 
because the gray anterior horn substance situated caudally from the trans- 
verse lesion is diseased, we cannot understand why this change in the gray 
substance should not also extend centrally from the transverse lesion. 

The absence of the tendon reflexes immediately after a severe cranial hem- 
orrhage as well as during profound loss of consciousness is attributed to 
" shock " of the gray anterior horn substance. These absent reflexes are to 
be strictly differentiated from those conditions in which rigid active or passive 
contractures make the production of a reflex or any other movement mechan- 
ically impossible. 

When there is a mere increase of tendon reflexes, clonus is noted if the 
increase of reflex reaches a certain degree. Here I must reiterate that true 
clonus is met with in hysterical conditions. A more common functional affec- 
tion is the previously mentioned " pseudo-clonus " which is produced by uncon- 
scious voluntary muscular contractions, and differs from true clonus in that 
the contractions soon lessen and cease. 

The most common form of clonus is that involving the patella and the 
foot. Clonus of the hand is extremely rare. 

CUTANEOUS REFLEXES 

(Toe reflex, difficulty of its production; Babinski's phenomenon, its occurrence, 
cremaster reflex, abdominal wall reflex and its test, eyelid reflex, sneezing reflex, retch- 
ing reflex, palate reflex, absence of pharyngeal reflex in hysteria, in epilepsy, and in 
smokers; nature of the cutaneous reflex, analogies between the toe reflex and other 
cutaneous reflexes, action of the cutaneous reflexes in cerebral and spinal diseases. 
Antagonism between tendon and cutaneous reflexes, relation of the cutaneous reflexes 
to the pyramidal tracts, action of cutaneous reflexes in diseases of the peripheral nerves 
and of the anterior horns, increase of cutaneous reflexes in tabes dorsalis and in neu- 
rosis, slight importance of cutaneous reflexes for the condition of conscious sensation.) 

In examining the reflexes of the skin and mucous membranes, I shall 
mention only those which are clinically most important. First among these 
is the toe reflex. The investigations of Babinski have taught us that the 
normal toe reflex is this : Slight irritation of the sole of the foot, for instance, 
as when we stroke it with the handle of the hammer, will produce a slight 
plantar flexion of the four external toes and simultaneously a slight adduc- 
tion of the same. This toe movement is the effect of a contraction of the 
interossei muscles. With this movement of the toes, even on very slight irri- 
tation of the sole of the foot, other reflex muscular contractions often occur 
in the tensor fasciae latse, in the sartorius, and in the adductors. But, for the 



EXAMINATION OF THE REFLEXES 203 

clinical examination of the reflexes, the signs on the part of the toes are the 
most important. If, instead of a plantar flexion of the toes on the application 
of a mild irritant to the sole of the foot, there is a single dorsal movement 
of the great toe, usually slow, or a dorsal movement of all the toes simul- 
taneously, we are dealing with a pathologic toe reflex. This pathologic form 
also occurs when simultaneously the great toe is moved upward and the other 
toes are moved downward. 

Whether the latter reflex phenomenon can actually be regarded as a " patho- 
logic form " of the normal toe reflex, or whether we are dealing with a reflex 
phenomenon quite independent of the normal reflex, is not yet certain. 

The correct production of the toe reflex is more difficult than that of any 
other reflex. In spite of the greatest care, contradictory and imperfect results 
will often be obtained in the different examinations. 

The result of the examination depends largely on whether or not the 
attention of the patient is called to the processes in his foot. Hence I advise 
that we produce the reflex as soon as the patient is in the recumbent posture 
with his legs bared, i. e., before we test the coarse power or make any other 
examination of the legs which may attract the attention of the patient to 
his feet and legs. For the same reason it is never advisable to produce the 
reflex several times in immediate succession. Another precaution in the first 
examination is to apply the irritation immediately to the desired point, i. e., 
about the middle of the internal border of the foot; and irritation should 
not be too rapidly nor too briskly produced, nor be too intense. If the stimu- 
lation is too strong a so-called flight movement appears with marked flexion 
of the knee-joint and dorsal flexion of the entire foot and toes which may 
completely suppress the normal sole reflex. 

If we note that the tendon of the extensor hallucis longus is prominent 
even during rest, or that the great toe is extended, we are dealing with an 
almost tonic toe reflex, and may feel assured that irritation of the sole of the 
foot will produce distinct extension of the great toe. 

The pathologic form of the toe reflex and an increase of the patella ten- 
don reflex are found, although not invariably, in disturbances and interrup- 
tions of the pyramidal tract. Absence of the pathologic toe reflex, however, 
does not indicate that the pyramidal tract is intact. Babinski reported the 
pathologic form after epileptic attacks. 

The fact that in children up to the age of about nine months — therefore 
so long as the pyramidal tracts are still undeveloped — only dorsal movement 
follows irritation of the sole of the foot is no less interesting scientifically 
than it is practically important. In the adult dorsal movements represent 
the pathologic form of the reflex, and whether these are ever actually observed 
in hysterical conditions has not yet been determined, since observations to the 
contrary are rare. The quantitative change in the reflex phenomenon plays 
no role in the toe reflex. 

Besides the toe reflex we must consider clinically the cremasteric reflex 
and the various abdominal reflexes. While the patient stands or is in the 
recumbent position the cremasteric reflex is tested by stroking the handle of 
the percussion hammer upon the internal side of the thigh from the level 
of the scrotum downward. The reflex consists in this; the contraction of 
the cremaster muscle causes the testicle upon the stimulated side to rise 



204 GENERAL NEUROLOGICAL DIAGNOSIS 

for a few seconds. This reflex is of extraordinary constancy; in a recent 
hemiplegia its absence has a certain practical importance because, in spite 
of unconsciousness being still present, it enables us to determine which side 
is paralyzed. 

The abdominal reflex, or reflex of the abdominal walls, consists of rapid 
contractions produced in the muscles forming the wall of the belly by irrita- 
tation of the lateral regions of the abdomen. We differentiate several reflexes 
of the abdominal wall according to the height of the stimulated cutaneous 
area. According to Oppenheim, it is sufficient for practical purposes if we 
test the supra- and infra-umbilical reflexes. The stimulation is best pro- 
duced by a single, rapid, and gentle stroking of the lateral abdominal wall 
with a not too sharply pointed lead-pencil or the like. Normally there is 
a rapid contraction of the abdominal muscles upon the stimulated side. When 
the reflex cannot be obtained with the patient in the recumbent posture, it 
is often evoked if he stands. This is particularly true of very fat patients, 
and of those with very flaccid abdominal walls. If the abdominal walls are 
tense, as is often the case in nervous patients and in those with abdominal 
pain, or pain in the lower extremities, the production of the reflex is difficult. 
Notwithstanding all precautions, and even when there are no pathologic 
conditions, it is sometimes impossible to evoke the abdominal reflexes. 

Clinically the most important mucous membrane reflexes are the eyelid 
reflex, the retching reflex, the palate reflex, and, perhaps, also the sneezing 
reflex. The eyelid reflex is the closing of the palpebral fissure on touching 
the conjunctiva. The sneezing reflex is an attack of sneezing as soon as the 
mucous membrane of the nose is slightly tickled. The retelling reflex is rela- 
tively the most important of the mucous membrane reflexes; this is tested 
by depressing the tongue of the patient and touching the posterior pharyn- 
geal wall with a brush, a piece of paper or the like; this induces retching 
which in rare cases (in alcoholics) may even be followed by vomiting. 

The reflex produced in the soft palate by lightly touching the uvula or the 
soft portions is manifested by the contraction and elevation of the palate. 

The behavior of the last two reflexes in the pharyngeal mucous membrane 
should always be tested in the various bulbar affections, since they often fur- 
nish data for localization. In a large proportion of hysterical and epileptic 
individuals, these reflexes of the pharyngeal mucous membrane are either 
diminished or abolished. As the same condition is also found in normal 
persons (particularly in smokers), and as many epileptics continue for years 
under the influence of the bromids which decrease reflexes, it appears to me 
doubtful whether the absence of these reflexes in hysteria and epilepsy should 
always be attributed to a pathologic condition. 

Since the question now arises whether there is such a simultaneous action 
of the cutaneous reflexes as has been described in the various nervous diseases, 
we must first briefly consider the physiologic nature of cutaneous reflexes. 

It is clear that the cutaneous reflexes, like the tendon reflexes, have an 
afferent sensory and an efferent motor branch in their reflex arcs. It is also 
evident that the output of power, the motor innervation of the descending 
branch, takes places in the anterior horn cells of the spinal cord. Concern- 
ing most of the reflexes it is still a mooted question whether the stimulation 
from the afferent branch of the reflex arc to the anterior horn cells is directly 



EXAMINATION OF THE REFLEXES 205 

and quickly transmitted from the spinal cord — as in the tendon reflexes — or 
whether the transmission of the afferent sensory irritation (which in the 
tendon reflexes rises to the cerebrum) is first to the brain itself, thence along 
the tracts of motor innervation, being finally transported to the area of power 
discharge in the spinal cord. The latter possibility does not practically differ 
greatly from a third one, according to which the reflex center of the cutaneous 
reflexes is situated in the spinal cord, but depends largely upon the condition 
of the brain, and is constantly influenced by this organ. 

As stated, the foregoing questions are by no means solved. It is not 
likely that all cutaneous reflexes permit the same conclusion as to their reflex 
centers. For the normal toe reflex which has been much studied in the last 
few years it is very likely true that the reflex transmission, i. e., the trans- 
mission of the afferent stimulation, is a descending one from the cerebrum. 
For the other cutaneous reflexes this does not seem so certain, although clin- 
ical analogies favor it to some extent. 

The clinical analogies referred to between the other cutaneous reflexes and 
the toe reflex are the facts that in cerebral foci, especially when the pyramidal 
tract is interrupted, there is generally a decrease or absence of the cutaneous 
reflexes upon the side opposite to the pathologic focus. My reference to the 
absence of the toe reflex in cerebral diseases must not be regarded as contra- 
dictory, for I previously explained that the so-called inversion of the normal 
toe reflex into its pathologic form (Babinskfs sign) is probably to be ac- 
counted for by the actual disappearance of the toe reflex, instead of which 
there is an entirely new, but different, reflex condition. Here the other cutane- 
ous reflexes correspond to the normal toe reflex, in that they are sometimes 
present notwithstanding the cerebral interruption of the pyramidal tract. 
Moreover, in high-seated and marked transverse changes in the spinal cord, 
especially with complete cross section, all of the normal cutaneous reflexes 
disappear. In cases of high-seated transverse disturbance of the spinal cord, 
those reflex movements which appear on dorsal flexion as a quick uplift and 
retraction of the leg after stimulating the skin of the foot are perhaps also 
to be regarded as a pathologic form of the toe reflex, and present new patho- 
logic reflexes which are normally absent in this form. Here the reflex trans- 
mission may be certainly referred to the spinal cord, since the path to the 
brain is destroyed. Prom the action of the cutaneous reflexes in central 
diseases just outlined, the antagonism in these diseases between the tendon 
and cutaneous reflexes will become conspicuous. This antagonism is often 
shown not only by the diminution or absence of the cutaneous reflexes when 
the tendon reflexes are increased, but also because the tendon reflexes are 
invariably and uniformly either absent or increased; the cutaneous reflexes, 
however, are very inconstant and have diagnostically a varying importance. 
Their manifold nature furnishes a hint as to their complicated relations to 
the cerebrum. That the pyramidal tract, in particular, is intimately related 
to the cutaneous reflexes may be concluded from the fact that (according to 
Striimpell) there are normally fewer cutaneous reflexes in the upper than 
in the lower extremity. Accordingly, with the increasing functional devel- 
opment of an extremity, i. e., with the increasing number of distinct move- 
ments which one extremity is capable of performing, the cutaneous reflexes 
decrease in number or are abolished. 



206 GENERAL NEUROLOGICAL DIAGNOSIS 

In diseases and interruptions of the afferent and efferent branches of the 
reflex arc, as well as in highly destructive processes in the region of the 
anterior horn cells which must furnish the energy for the motor reflex con- 
tractions, the action of the cutaneous reflexes is generally analogous to that 
of the tendon reflexes ; i. e., under the previously mentioned conditions the 
cutaneous reflexes are either decreased or abolished,; For instance, in all forms 
of anesthesia due to peripheral cause, in peripheral paralysis, in neuritic proc- 
esses, and the like. That the cutaneous reflexes are often actively increased 
in tabes dorsalis — a disease in which there is usually a great diminution of 
sensation — instead of being decreased, may perhaps be accounted for by the 
irritative conditions in the diseased afferent branch of the reflex arc. In favor 
of this view is the circumstance that, in such cases, besides the anesthesias, 
or, better, the analgesias of the deeper cutaneous areas, there are often extreme 
tactile hyperesthesias of the superficial cutaneous areas. Perhaps this increase 
of the cutaneous reflex in tabes is only functional, and may be attributed to 
the neurasthenic condition which frequently accompanies tabes, for in many 
cases of neurasthenia and in most other neuroses the cutaneous reflexes as well 
as the tendon reflexes are increased. 

Before concluding our description of the cutaneous reflexes, I must again 
issue a warning not to attach too great weight in diagnosis to the action of 
the cutaneous reflexes, above all not to draw too hasty conclusions as to con- 
scious sensory conditions from their behavior. In functional diseases, but 
particularly in hysteria, we meet with conspicuous abnormalities in the action 
of the cutaneous reflexes and in conscious sensation. It would be a great 
error if, for example, we should diagnosticate anesthesia of the palate from 
the absence of the palate reflex. 

THE INTERNAL REFLEXES 

(Pupillary reflex, width of the pupils, occurrence of myosis and mydriasis in organic 
diseases, in epilepsy, and in other neuroses; anisocoria, its occurrence; sympathetic 
pupil reaction, light reaction of the pupil, testing the light reflex, its difficulties; dura- 
tion of the light reaction, abnormally rapid and sluggish light reaction; sluggish pupils 
in the aged, rigidity to light of the pupils, hemiopic pupillary reaction, accommodation 
reaction of the pupils, Argyll -Robertson sign, its occurrence; ophthalmoplegia interna; 
relative preponderance of the reaction of accommodation over the reaction to light, 
Piltz's sign, consensual pupillary reaction, reaction of the pupils due to psychical irri- 
tation. — Reflexes of the discharge of feces and urine, questions regarding the action of 
the fecal and urinary reflex, diagrammatic presentation of the fecal and urine reflexes, 
action of the bladder and of the intestine in diseases of the brain and the upper por- 
tions of the spinal cord, and in diseases of the sacral cord ; the importance of mechanical 
factors in the evacuation of feces and urine, fecal and urinary reflexes in neuritic 
processes, in neuroses, in hysterical bladder disturbances. — Sexual reflexes in man, in 
woman ; sexual frigidity in woman ; disturbance of the sexual reflex in the male, in 
organic diseases and in neuroses; priapism, pollutions, menstrual disturbances.) 

Under the designation internal reflexes, we shall group for purely practical 
purposes the pupillary reflex, the reflex of the discharge of urine and feces, 
and the sexual reflex, notwithstanding the fact that an intimate relationship 
exists only between the reflexes for the discharge of feces and urine, and 
possibly for the sexual reflex. 

The first and most important of these reflexes we may objectively test; 



EXAMINATION OF THE REFLEXES 207 

in the other internal reflexes we are, as a rule, dependent upon the reports of 
the patient. 

The pupillary reflex should always be tested at the beginning of the ex- 
amination. At this time we should observe whether the pupils are equal in 
size and whether their width corresponds to physiologic limits. In emmetropic 
persons the pupils, viewed in a clear, diffused daylight, have a diameter of 
2 to 3 mm. Marked increase or decrease of this limit is to be regarded as 
pathologic. Abnormal narrowness of the pupil, myosis, is found in the aged, 
in tabes dorsalis, in syringomyelia, and occasionally in progressive paralysis, 
in cerebral syphilis, in morphinism, in nicotinism, in the early stages of in- 
creased pressure on the brain, and in paralysis of the cervical sympathetic. 
Abnormal dilatation of the pupil, mydriasis, is found in organic diseases of 
the brain, particularly in brain tumor, also in paralysis, in cerebral syphilis, 
in high-graded increase of cerebral pressure, in amaurosis, in paralysis of the 
oculomotor nerve, after the administration of atropin, etc. Abnormally dis- 
tended pupils are found in some hysterical patients, particularly in epilep- 
tics. The pupils of apprehensive neurasthenics and hypochondriacs are often 
conspicuously dilated. 

In deciding as to the uniformity or inequality of the pupils we must not 
make too subtle distinctions. Very slight differences in the pupils are ob- 
served even in health. Conspicuous inequality of the pupils {anisocoria) is 
found in all of the organic diseases in which myosis or mydriasis occurs, 
provided the anatomical change is not uniformly distributed to both sides, 
or if the condition is unilateral. Although inequality of the pupils is occa- 
sionally seen in functional diseases, yet decided anisocoria indicates the exist- 
ence of an organic disease. After we have acquainted ourselves with the 
condition of the pupil during rest, we may proceed to test the pupillary reflex. 

Before describing the most important pupillary reflex, the light reflex, 
I must briefly mention the so-called "sympathetic pupillary reaction." This 
consists in a dilatation of the pupil on pinching or faradization of the skin 
of the nape of the neck. Only rarely are we in a position to make this inves- 
tigation. 

The light reflex is a contraction of the pupil upon the reflection of light 
into the eye, and its dilatation with a decrease of light. The reaction of 
each eye to light must be tested separately. While one eye is examined the 
other should be lightly covered. The light must be bright, whether it be 
daylight or artificial illumination. During the examination the patient 
should look into the distance, and not at a near object. 

Testing the reaction to light by means of a lighted match or a candle 
instead of daylight is occasionally a source of error, for the patient uncon- 
sciously accommodates his vision to the object held before him, and thus 
contracts his pupils. It is better to make the test with the patient seated 
immediately before a window where the light is good, and he should cover 
one eye with his hand. While the patient looks off into the distance the 
physician covers the other eye, the one to be examined, with his own hand. 
After a few seconds he withdraws his hand, and notices whether the pupil 
is narrower. If the reaction to light is very slight or doubtful, it is well to 
keep the patient in darkness for some time, if possible for a few hours. We 
then examine to see whether the reaction to light has improved. 
15 



208 GENERAL NEUROLOGICAL DIAGNOSIS 

Even after following these directions it is not always easy to come to a 
conclusion. The light reflex of the cornea often interferes, particularly if the 
pupils are narrow. Of course, myosis makes the judgment of the individual 
case difficult because, if the pupil is contracted, the variations in the size of 
the pupil are naturally less than with a normally wide pupil. 

Under these circumstances, as well as when examining during a spasmodic 
attack, in coma, etc., a small electric pocket-light is very valuable. This can 
be brought very close to the eye, cannot injure it, and gives a very intense 
light. 

The reflex contraction and dilatation of the pupils is normally very rapid, 
and more so in young persons and children than in older persons. Abnor- 
mally rapid reaction to light is occasionally seen in excitable neurasthenics 
or hysterical persons. Sluggish and imperfect reaction to light as a pro- 
dromal stage to complete rigidity to light, as a rule favors the organic nature 
of the accompanying symptoms. Only in the aged do we observe a sluggish 
reaction or even the absence of reaction to light without pathologic significance. 
Hence, I advise great care in using pupillary sluggishness as a diagnostic sign 
if the patient has reached the sixtieth year of age. True rigidity to light is 
observed only in organic diseases. Besides diseases of the eye we must con- 
sider the following: Total atrophy of the optic nerve, diseases of the nuclear 
region of the oculomotor nerves, tabes dorsalis, progressive paralysis, multi- 
ple sclerosis, brain tumor, hydrocephalus. 

A distinctive reaction to light is the so-called hemiopic reaction of the 
pupil. This is characteristic because in the hemiopic the pupillary reflex 
appears only when the retinal half which still sees is influenced by the light, 
while there is no illumination of the blind retinal half. The demonstration 
of the hemiopic reaction is exceedingly difficult, for the stimulation of light 
in the manner described is not readily produced. 

So far we have been considering rigidity to light, that is, the reaction 
of the pupil to light. There is another reaction of the pupil, of great clin- 
ical importance, which like the reaction to light should be investigated in 
every case. This reaction does not strictly belong with those we are investi- 
gating, for it is not a reflex but a coordinated movement. However, it is 
best described when considering the reaction to light. The dilatation and 
contraction of the pupils is not only reflex, indicating the effect of light or 
greater stimulation by light, but with a number of movements of the external 
muscles of the eye and of the lids is also synergistic. 

Upon near accommodation and consequent convergence of the eyes, as, 
for example, when looking at the finger, the pupils contract, and on looking 
into distance they dilate. This is the most important synergistic reaction 
of the pupil, the reaction of accommodation. As a rule, the eyes may be 
simultaneously tested for accommodation. 

From the physiologic variations in the reaction to light and accommo- 
dation it is evident, a priori, that in their pathologic relations these reactions 
are independent of each other. Clinical experience confirms this. For in- 
stance, the most important difference between these reactions, the reaction to 
light, often completely disappears while the reaction to accommodation is 
undisturbed. This sign, the Argyll-Eoberston phenomenon, is commonly ob- 
served in tabes dorsalis and in progressive paralysis, occasionally in focal dis- 






EXAMINATION OF THE REFLEXES 209 

eases of the brain. It is never the indication of a functional nervous dis- 
ease. The inverse, rigidity of the pupil to accommodation with a normal 
reaction to light, is practically never observed — certainly not when the exter- 
nal muscles of the eye function normally. [This statement is perhaps too 
sweeping in certain cases of neuritis, as for instance in a number of cases 
that occurred in the Manchester epidemic, response of the eye for accommo- 
dation disappeared while the response to light persisted without involvement 
of the extrinsic muscles. However, the occurrence is so rare as to make the rule 
as stated by the author practically universal. — Ed.] Absence of reaction to 
light as well as to accommodation, so-called ophthalmoplegia interna or com- 
plete pupillary rigidity, is common. Complete pupillary rigidity, except when 
the eye itself is diseased, is always a sign of serious organic intracranial affec- 
tion. Combined with other objective nervous symptoms it is found in paralysis, 
in tabes, in multiple sclerosis, in brain tumor, in hydrocephalus, and in simi- 
lar diseases. Internal ophthalmoplegia appearing alone usually points to a 
syphilitic cerebral disease. 

A decided decrease of the reaction to light and accommodation, or even 
its almost complete absence, is found in chronic alcoholism and in the aged. 
In all of these cases, however, the reaction to accommodation is greater than 
the reaction to light. In fact, under quite normal conditions, the impression 
is given that the contraction of accommodation, in comparison with the true 
reflex contraction, is more powerful and intense. 

One of the synergistic pupillary reactions has been much studied during 
the last few years, particularly by Piltz : In many paralytics and tabetics, but 
also in patients with other affections, and even in a certain proportion of 
normal persons, a contraction of the pupil follows the firm contraction of 
the eyelids by the orbicularis. The eye should be tightly closed, when the 
physician, opening the lids and holding them apart, prevents their closure, 
at the same time observing whether the pupils contract. As contractions at 
once cease if the eyes are not tightly closed, it is evident that contraction soon 
passes into dilatation. Hence, in testing for Piltz's sign, after opening the 
eye, and following the preceding brief contraction, the pupil dilates and we 
obtain an apparently paradoxical reaction to light. Some authors who have 
observed this believe it to be an actual paradoxical reaction to light. 

The appearance of Piltz's phenomenon is independent of the previously 
described reaction of the pupil, and is especially marked if there is rigidity 
to light or a combination of rigidity to light and to accommodation. 

Among the synergistic pupillary reactions we must also mention consensual 
dilatation or contraction of both pupils. Normally, a change in the diameter 
of the pupils is simultaneous and uniform, immaterial whether this be due 
to reflex irritation or to coordinated movement. As is evident, the consensual 
reaction is easily tested. 

Finally, to prevent any possibility of error, I must call attention to the 
remarkable fact that, under some circumstances, the mere presence of light 
or dark objects may cause a contraction or dilatation of the pupil. This is 
a somatic reflex due to psychical irritation — the presence of an optical memory 
picture. 

As previously stated, the internal reflexes, the discharge of feces and urine 
and the sexual reflex, can be determined only by the reports of the patient. 



210 GENERAL NEUROLOGICAL DIAGNOSIS 

We should inquire whether the discharge of urine and feces is normal as to 
quantity and frequency, whether voluntary evacuation is regular and without 
effort or delayed and difficult, whether the discharge may be interrupted at 
will, whether there are involuntary evacuations, finally whether the passage 
of urine and feces is accompanied by normal sensations. 

The mechanism of the reflexes for the discharge of urine and feces is 
much more complicated and less familiar to us than the mechanism of the 
cutaneous and tendon reflexes. The disturbances of the internal reflexes may 
best be considered by employing the diagrammatic method, and comparing 
the reflexes themselves with the diagram of tendon reflexes or, even better, 
with that of voluntary muscle innervation. 

Therefore the evacuation of urine and feces, while considered as analo- 
gous to the innervation of any muscle of the body, is a purely voluntary 
movement. 

This "voluntary movement," which promotes discharge from the bladder 
and bowel, may normally, like any other voluntary movement, be so inner- 
vated at any time as to cause an action, and it may at any time be interrupted. 
It differs from true voluntary movement only because the innervation, as a 
rule, follows an irritation from the periphery (the bladder or the bowel). If 
the central portion of the voluntary tract leading from the brain to the 
anterior horns of the sacral cord, in which is located the power station for 
the muscles of the bladder and of the rectum be damaged, analogous condi- 
tions are to be looked for in the peripheral portion of the nervous apparatus 
like those which occur in interruption of the pyramidal tract in spastic paral- 
ysis of the extremities; that is, the peripheral reflex arc attains a certain 
independence by interruption of the pyramidal tract, and it acts alone or 
to irritation constantly coming from the posterior roots to the anterior horn 
cells sufficiently to keep the muscles in a permanent state of tonic contracture 
(as, for example, in spastic intestinal torpidity) ; or periodically there are 
brief tonic discharges of power in the anterior horn cells whenever the sen- 
sory irritation from the internal organs reaches a certain height (as inter- 
mittent automatic evacuation of the bladder). Besides — and this is the most 
conspicuous disturbance — the power of voluntary discharge as well as of vol- 
untary interruption ceases. 

Therefore, any pathological process which interrupts the central reflex tract 
in the brain or in the upper portions of the spinal cord may cause constipation 
and retention of urine. Under some circumstances, there may be involuntary 
evacuations of the bladder as well as retention. 

Proceeding from this standpoint, and assuming that the power station 
in the anterior horns for the muscles of discharge is destroyed, a condition 
results which resembles flaccid paralysis, and want of tone in the expelling 
as well as retaining muscles causes a constant discharge of the contents of 
the intestine and bladder (dribbling of urine). The latter clinical condition 
is observed in hemorrhage and other destructive processes in the lower por- 
tion of the spinal cord. 

Analogously as in a complete and high interruption of the spinal cord, 
and in severe shock to the cord, instead of an expected spastic paresis of the 
extremity we observe a complete flaccid paralysis and absence of the tendon 
reflexes (a condition similar to that in disease of the anterior horns), and this 



EXAMINATION OF THE REFLEXES 211 

change in the reflex for the bladder and rectum which has just been described 
(corresponding to a destruction of the motor spinal power station) is fre- 
quently seen in severe shock, or after complete transverse interruption of the 
spinal cord. 

The diagram I have just given, which by analogy enables us to understand 
disturbances of the internal reflexes, shows deviations other than those due 
to internal neuro-physiologic peculiarities in the nature of these reflexes — 
just as we never have a rule without exceptions but are constantly forced to 
use expressions like " occasionally " or " sometimes " — in that many purely 
mechanical factors are involved in the evacuation of the bladder and rec- 
tum. Of course, these mechanical factors are influenced by the appearance 
of nervous disturbances which lead to a further change in the function of 
the pathologically altered powers of expulsion. 

If in the preceding we have considered only spinal and cerebral diseases, 
this was because these diseases represent the majority of the disturbances of 
the internal reflexes; but neuritic processes, particularly polyneuritis, more 
frequently than is assumed produce disturbances in the discharge of urine. 
If we believe either the motor or sensory nerve, or both, to be diseased we 
have the explanation of the existing bladder disturbance. 

Functional diseases frequently produce quantitative irregularities in the 
function of the internal reflexes. Among these is constipation in hysteria and 
neurasthenia, the so-called pollakiuria (abnormally frequent micturition), 
polyuria (excessive quantity of urine), the impossibility in neuropathies of vol- 
untary urination in the presence of others, hysterical anuria, the occasional 
impossibility of passing feces during psychical preoccupation, enuresis noc- 
turna, etc. Unquestionably, although rarely, there may be an actual disturb- 
ance of the mechanical function of the bladder which, in the absence of any 
organic change, must be regarded as hysterical. For instance, I some time 
ago treated a person suffering from paralysis of both legs and occasional in- 
continence of urine, in whose case, on account of the urinary difficulty, a 
diagnosis of organic disease of the spinal cord had previously been made by 
a very prominent physician. The sudden cure of the paralysis and the disap- 
pearance of the other symptoms proved the hysterical nature of the affection. 

It is self-evident, but must be reiterated, that when the evacuation of 
urine and feces is disturbed, we must exclude the possibility of an internal 
or surgical affection of the intestine, of the bladder, of the prostate, etc., before 
we assume a nervous affection. 

The last of the internal reflexes, the sexual reflex, belongs to the vaso- 
motor cutaneous reflexes. In the male its motor components are comprised 
in the muscular exertion necessitated by the filling of the corpora cavernosa 
(erection), in the discharge of semen, and in its expulsion through the urethra. 
In the female it probably consists chiefly in the filling of the corpora cavernosa, 
and in the discharge of a physiologically unimportant glandular secretion. 

Corresponding to their physiologic functions, disturbances of the male 
sexual reflexes are more important clinically than those of the female. 

In regard to the sexual reflex, women occasionally report that during 
cohabitaton ejaculation occurs too late or not at all, or that a certain psychical 
impediment disturbs the reflex action, that libido is almost entirely lacking, 
and that they have noted these conditions. In these cases, usually occurring 



212 GENERAL NEUROLOGICAL DIAGNOSIS 

in hysterical or otherwise neuropathic individuals, there is sexual frigidity. 
We have little knowledge of disturbances of the sexual reflex of the female 
from organic diseases. 

If the male complains of disturbance of the sexual reflex, we must ascer- 
tain by questioning whether erection and ejaculation are normal, and whether 
the former continues a normal length of time. Absence or incomplete erec- 
tion, as well as its abnormal duration, is an early indication of spinal disease, 
particularly of tabes. In practice, however, these symptoms are decidedly 
more common as the expression of functional weakness and inhibition, as in 
hypochondriasis and neurasthenia, than in organic diseases. 

Among disturbances of the sexual reflex of an opposite nature, priapism 
must first be mentioned; it may be regarded as analogous to the spastic state 
of the extremity which occurs in high-seated interruption of the spinal cord, 
as a tonic spasm or spastic condition of the muscles which produces a regurgi- 
tation of blood in the corpora cavernosa, and thus causes erection. Priapism is 
a rare symptom. Corresponding to the occurrence of spasm in the extremities, 
it is found in high-seated interruption of the conduction in the spinal cord. 

Priapism is said to occur also in functional diseases. 

Another increase of pathologic activity in the realm of the sexual reflexes 
is formed by pollutions. These are found in organic and in functional dis- 
eases. They usually occur at night, rarely during the day. In the latter 
case they are produced by most heterogeneous psychical stimuli, fear, fright, 
anger, etc. Nearly all of the patients in whom this unfortunate condition is 
noted are extremely neurotic. 

As a supplement to the so-called internal reflexes, we may briefly consider 
menstruation, for in menstruation also there is probably a reflex process. 
Disturbances of menstruation are very common in women with nervous affec- 
tions; the menses may occur with abnormal frequency or be rare; the flow 
may be abnormally profuse or very scant. Therefore we must always inquire 
into these conditions. In patients who are severely predisposed to neuropathic 
conditions, menstruation often ceases because of general hypoplasia or aplasia 
of the genitalia, but amenorrhea occurs even without tangible change in the 
genital apparatus or in the general constitution, apparently as a purely nerv- 
ous affection, in depression, hysteria or similar conditions. Of amenorrhea 
in organic nervous diseases, particularly spinal affections, we know very little. 

In the preceding I have endeavored to give a synopsis of the most impor- 
tant points to be borne in mind during the examination of patients with 
nervous disease. To avoid prolixity, some important points have been briefly 
discussed; other significant diagnostic and symptomatic realms, such as elec- 
tro-diagnosis, central speech disturbances, etc., have not been considered at all 
because they form the subject of special articles in this book. 

I desire to call special attention to one point not yet discussed, for expe- 
rience shows that this point is unfortunately somewhat overlooked. We should 
always bear in mind that neurology is a daughter of internal medicine, there- 
fore no neurologic investigation can be regarded as complete unless the con- 
dition of the internal organs, especially of the heart and kidneys, has been 
thoroughly investigated, for only when we are in a position to decide as to 
the condition of the internal organs will our diagnosis of the pathologic nerv- 
ous conditions be based upon a firm foundation, 






MODERN AIDS IN THE DIAGNOSIS OF DISEASES OF 

THE BRAIN 

By E. REDLICH, Vienna 

If we date the beginning of the recent advance in the diagnosis of diseases 
of the brain which we are about to discuss at 1879, the year when Nothnagel's 
" Topical Diagnosis of Cerebral Diseases " appeared, we may state that prior 
to that period the diagnosis of cerebral diseases had in a relatively short time 
made rapid advances. Minute investigation and symptomatology, combined 
with the utilization of important and newly acquired physiologic factors, 
beginning with the electrical cortical irritation of Fritsch, Hitzig, and others, 
our insight into the normal anatomy of the central nervous system, the exact 
pathological anatomy and histology of cerebral disease — which so far as their 
importance goes were almost natural experiments in man — have led to the 
so-called topical diagnosis of cerebral diseases, i. e., to the localization of the 
parts of the brain affected, to a precision and minuteness which, compared 
with the former and often unsatisfactory methods of diagnosis, have awak- 
ened among physicians the liveliest interest in, and admiration for, neurology. 
The second and no less important question as to the nature of the process 
present of course permitted even a less positive decision, on account of the 
limited methods of examination then known ; in the majority of cases there 
was little or nothing to aid us in arriving at direct conclusions. The history, 
the age of the patient, the pathologic changes going on in his body, the fre- 
quency of certain diseases, permitted at most merely a guess as to the nature 
of the process, and then we had to reckon with the factor of its probability. 

Since the period mentioned, the diagnosis of diseases of the brain has 
advanced in all directions, although perhaps not so rapidly as it formerly did. 
Many of the opinions then formed were modified by subsequent investigations, 
others became more firmly rooted, many new ones have been added. If I may 
be permitted to use the expression, the territory of the diagnosis of diseases 
of the brain has been greatly extended. 

It is hardly possible to review the present state of diagnosis of diseases 
of the brain within the scope of a short article. This, in its most concise 
form, would require many pages. I prefer, therefore, to describe a number 
of aids which we may invoke in the diagnosis of diseases of the brain as well as 
its neurologic symptoms. Much of what I shall state is not new, although most 
utilized in recent times, and having lately gained greater publicity than for- 
merly; other things are new, perhaps too new, being still in many respects 
subject to discussion, so that it is to-day impossible to give an absolute opin- 
ion concerning the value of individual methods, Neurology has adopted many 

213 



214 DIAGNOSIS OF DISEASES OF THE BRAIN 

of the modern methods of medicine, and has attained such practical success 
as to lend certainty to its conclusions. In some cases it has become possible 
by this means to lay bare the nature of underlying processes, and, under some 
circumstances, for instance, by demonstrating specific microorganisms, even 
the etiology of diseases of the brain has been made clear. We may readily 
understand how important this is in the operative treatment of diseases of 
the brain. Of course, in its endeavor to attain favorable results, surgery is 
often a step in advance of diagnosis. But in the brain, as elsewhere, explora- 
tory operation has often been resorted to for the purpose of diagnosis. 

Every examination should begin with inspection, not of the diseased organ 
but of its covering, the skull. Certain abnormalities of the skull, a premature 
closure of the sutures, microcephalia or macrocephalia, its general size or 
diminutive size, are naturally more valuable points to the psychiatrist, since 
they reveal, above all, the general damage to the brain. Even more significant 
are circumscribed flattenings of the skull, since, under some circumstances, 
these indicate circumscribed lesions of the brain, such as porencephalitic proc- 
esses dating from childhood. For instance, we sometimes find a unilateral 
decrease in the size of the skull in cases of cerebral infantile paralysis, and 
on the side opposite the paralysis. In a case of epilepsy recently demonstrated 
by Infeld this unilateral decrease in the size of the skull was the only visible 
residuum of such a cerebral process. Of course, in such cases mere inspection 
of the skull is supplemented by its mensuration, for which we possess a 
number of methods the details of which I shall not discuss. 

It is well known that Gall was inclined to base many of his conclusions 
on the mere inspection and examination of the skull; he believed that cer- 
tain mental qualities, individual traits and faculties, of course as philosophico- 
psychologic abstractions rather than physiologico-psychologic functions, were 
developed in various individuals, and by definite factors corresponded to dif- 
ferent formations. Gall's teaching was long regarded as a curious survival 
of a far distant period, but Mobius recently discovered some grains of wheat 
among this chaff. For example, he believes with Gall that mathematical 
endowment is indicated by a marked development of the upper external angle 
of the orbit. He investigated Gall's theory that the occiput developed accord- 
ing to the strength of the sexual function, etc. ; in short he attempted to 
" sustain " Gall's opinions. This met with bitter opposition from neurologists, 
especially Eieger; I believe that we need not dwell on this subject, since it 
furnished no absolute facts nor valuable conclusions. 

We will now discuss what is of greater practical importance. Inspection 
of the skull affords certain indications in the diagnosis of acromegalia, that 
peculiar disturbance of growth which, we must to-day assume, is the result of 
disease of the hypophysis. The general enlargement of the skull, the charac- 
teristic enlargement of the lower jaw, the increased size of the soft parts, 
of the lobes of the ear, also of the nose and the tongue, are so conspicuous 
to the expert that they directly indicate the diagnosis, although the enlarge- 
ment of the hands and feet, the other symptoms, the history, etc., form abso- 
lute proof. The diagnosis of hydrocephalus can usually be made at sight. 
Among its typical symptoms are the semiglobular enlargement of the whole 
skull, the characteristic prominence of the frontal and occipital portions, the 
protrusion of the eyes from the forcing downward of the orbital plate, some- 






DIAGNOSIS OF DISEASES OF THE BRAIN 215 

times erroneously regarded as exophthalmos, and more correctly designated 
as flattening of the eye, the prominence of the temporal region, etc. In the 
still open fontanelles of very young children, the rigid tension of the skull 
contents and the increased cerebral pressure may be directly demonstrated. 
Under some circumstances, even in adults, tumors of the skull and marked 
cerebral pressure may lead to a loosening of the sutures, and to openings in 
the skull. Tumors at the top of the skull or even those of the brain, by 
eroding the bone may proliferate outwardly, and often lead to palpable and 
visible protuberances of the skull. This permits the direct inspection of a 
cerebral tumor. As a curiosity I must mention a case of WestphaPs in which 
cerebral echinococci produced in this way an external opening which could 
be felt under the skin. In the diagnosis of traumatic epilepsj^ so-called reflex 
epilepsy, as well as in essential epilepsy, and in traumatic cerebral abscess, 
it is obviously important to find cicatrices upon the skull, even though only 
in consequence of an injury during the attack. Under some circumstances 
these will not only confirm the diagnosis but will indicate the necessity of 
operative interference. In such cases it is advisable to shave the skull prior 
to examination. 

In the veins of the skull, just as in other parts of the body, local stasis 
may occur in consequence of an obstruction to the circulation, and circum- 
scribed, visible, venous dilatation or edema appear. This occurs most fre- 
quently in sinus thrombosis; thus, thrombosis of the sinus cavernosus leads 
to dilatation of the frontal veins, to cyanosis of the frontal region, and to 
swelling of the eyelids, this condition being proven by the demonstration 
of dilated veins in the retina by ophthalmoscopic investigation. Thrombosis 
of the transverse sinus leads to edematous swelling of the soft parts behind 
the ear, and to dilatation of the veins in this region, which are also prominent 
symptoms of suppuration of the mastoid process; thrombosis of the longi- 
tudinal sinus causes dilatation of the veins and edema along the median line 
of the skull. These local venous dilatations are also occasionally found in 
brain tumors, in superficial tumors, even in those directly above the brain 
tumor. I must mention that upon inspection we sometimes find small ecchy- 
moses upon the skin of the forehead, in the conjunctivae, or in the face, etc., 
after severe epileptic attacks, and this is of great significance since, as a rule, 
the diagnosis of epilepsy cannot be directly made if the physician is not an 
eye-witness of the attack, but must rely solely on the history. 

It is self-evident that the X-rays, which have become so important in medi- 
cine, should have been resorted to in the diagnosis of diseases of the brain, 
but so far with but little success. The X-ray examination is of decisive im- 
portance in searching for projectiles in the skull. Exostoses and openings in 
the skull can also thus be diagnosticated with certainty. An attempt has been 
made to find many other things with the X-ray photograph — osteoporosis of 
the bones of the skull, hemorrhages in the meninges, thickening of these struc- 
tures, etc. It was hoped that brain tumors could in this way be directly 
demonstrated, and thus give to the diagnosis of traumatic neurosis, this true 
cross of the neurologists, a firm basis. But here the greatest care is neces- 
sary to prevent gross errors. I shall quote a case of Slavyk which presented 
clinically the symptoms of brain tumor. In the X-ray picture there was a 
marked shadow in the region of the sella turcica and extending to the parietal 
16 



216 DIAGNOSIS OF DISEASES OF THE BRAIN 

region. The autopsy, however, revealed the presence of a tumor of the pineal 
body, the shadow having nothing in common with this tumor. All experts 
in X-ray examinations can report similar errors. Generally, the bones of 
the skull form an impenetrable wall for the structures contained within, so 
that no shadow can be cast. Under very favorable circumstances, X-ray 
examination may reveal facts which are important in the diagnosis of endo- 
cranial processes, especially of tumors; for example, when marked calcifi- 
cation has taken place in the tumor, which may then be manifested by a 
distinct shadow. In three cases of tumor of the hypophysis — reported by 
Oppenheim-Cassirer, Fuchs, and Embden — the skiagraph revealed a dilata- 
tion of the sella turcica, therefore a result of the tumor which best supported 
the diagnosis. 

In physical diagnosis, percussion follows inspection and palpation of the 
skull; and is also of some importance. We first test the sensitiveness of the 
skull to percussion ; this may be either diffuse or circumscribed. Diffuse sensi- 
tiveness to percussion is often associated with sensitiveness to pressure which 
is distributed over the whole skull, or at least to great areas, and is a symptom 
which can only be utilized with extreme care. This is frequently observed in 
functional neuroses, especially in neurasthenia, hysteria, traumatic neuroses, 
etc. There is a form of headache designated as indurative headache; in this 
condition, according to general opinion, small infiltrations and palpable nod- 
ules appear in the galea aponeurotica ; conspicuous sensitiveness in extensive 
areas to pressure and to percussion being here characteristic. In organic 
lesions of the skull and of the brain, there may be a diffuse sensitiveness 
of the skull to percussion, just as in disease of the bones of the skull ; Oppen- 
heim reports that this is occasionally a sign of osteoporosis and thinning of 
the bones of the skull. The condition is observed in disease of the meninges, 
in pachymeningitis, in tuberculous meningitis, in disease of the brain proper, 
such as tumors, etc. Much more important is a local sensitiveness to percus- 
sion sometimes combined with local sensitiveness to pressure. This may also 
be noted in the form of the well known clavus in neuroses, in neurasthenia, 
and in hysteria, and frequently in traumatic neuroses at or near the point 
of injury. In other cases, such a circumscribed sensitiveness points to organic 
disease. Diseases of the cranial bones, tumors and inflammatory affections 
of the skull, often run their course with excessive painfulness in the corre- 
sponding area. Brain abscess, and especially brain tumor when it presses 
against the bones of the skull and irritates them in circumscribed areas, may 
produce circumscribed sensitiveness to percussion, and thus prove a valuable, 
local, diagnostic sign. Sometimes, at least, the fossa of the skull in which 
the tumor has developed may be determined by the circumscribed sensitiveness 
to percussion. Of course, this may prove a source of error; percussion over 
a tumor of the frontal region will sometimes produce pain in the occiput, and 
vice versa. Many errors in diagnosis have arisen in this way. Sometimes we 
must be content with deciding by means of this local sensitiveness in which 
hemisphere the affection is situated, for when there are no local symptoms 
such as paralysis of the cranial nerves, paralysis of the extremity, or a differ- 
ence in the reflexes, etc., this may be doubtful. 

In conclusion, we may state the following : In every case of cerebral affec- 
tion the sensitiveness of the skull to percussion should be tested, and this test 



DIAGNOSIS OF DISEASES OF THE BRAIN 217 

is valuable, but we do not base our diagnosis wholly upon it; on the contrary, 
we come to a definite conclusion only when the results thus obtained are in 
agreement with the other symptoms. 

Of late, regular percussion of the skull, i. e., differentiating the note 
obtained by its percussion, has attracted much attention. We place one finger 
upon the skull and percuss with another finger, the patient keeping his mouth 
closed. Gilles de 1a Tourette and Chipault, for example, report that on per- 
cussion of the skull the note is shorter the thinner the skull, and vice versa; 
therefore, the note obtained by percussion of the occiput is shorter than over 
the frontal bone. In the child it is clear, in the adult dull, and it again 
becomes clear in the aged. Sometimes there is a circumscribed dull note 
over a brain tumor; but this is an isolated occurrence. A tympanitic note, 
particularly when the bones are very thin, sometimes indicates that a brain 
tumor is present; however, as was shown by Oppenheim and Bruns, in the 
nursling a tympanitic note is physiologic. A significant finding is the cracked 
pot sound. This is always evoked when the continuity of the skull is inter- 
rupted, and it is therefore physiologic in the child as long as the sutures re- 
main open. Under pathologic conditions it may be found in hydrocephalus 
or in brain tumor, provided these have led to an opening of the sutures, also, 
as Gilles de la Tourette and Chipault report, in fractures of the skull. This 
cracked pot sound may also be circumscribed, sometimes in the vicinity of 
the tumor, but also at a distance from it as in the case of Wollenberg: With 
a tumor over the right occipital lobe, the cracked pot sound was elicited over 
the left portion of the frontal bone. We therefore see that in the diagnosis 
of cerebral processes the results of actual percussion are quite scanty; and it 
is only under especially favorable circumstances that we obtain valuable data. 
Nevertheless, it must be borne in mind that in doubtful cases any positive 
symptom is of value. 

In pursuance of our plan auscultation follows percussion. Normally in 
the adult this is usually without result, i. e., no endocranial murmurs are 
heard. In infancy the case is different. In nurslings and small children in 
whom the fontanelles are not closed, as well as in cases of rickets, a systolic 
murmur is said to be present. Oppenheim also heard this murmur in an 
adult with marked anemia. In pathologic conditions auscultation of the 
skull is under some circumstances of great value. Murmurs synchronous with 
the pulse have been observed in arteriosclerosis, and in an adult case of 
acquired hydrocephalus by Fuchs; in the latter case the murmur apparently 
originated in the ear and was perceptible to the patient. We know that these 
subjective murmurs are very distressing to the patient and difficult to control 
therapeutically. Sometimes the murmur can be heard by placing the ear upon 
the head, or even at a little distance from it. This is the case with tumors 
very rich in vessels or tumors which press upon a large vessel, especially if 
near the surface of the skull; rhythmical murmurs synchronous with the 
pulse are then heard, and most distinctly when the patient holds his breath. 
They are most common in aneurysm of the basal cerebral arteries, for example, 
in aneurysm of the internal carotid and the vertebral, sometimes even in the 
form of musical murmurs which are audible at a considerable distance. Of 
course, in such cases the murmur disappears if we press the common carotid 
artery. The point at which this murmur is most distinctly heard sometimes 



218 DIAGNOSIS OF DISEASES OF THE BRAIN 

corresponds to the seat of the aneurysm; but the presumable locality should 
coincide with the other symptoms. The findings must indicate a tumor, 
therefore a progressive affection which limits space, before we can make a 
diagnosis of aneurysm. 

In utilizing the history, diagnosis may go even further, as is proven by a 
case Karplus observed in which, accompanied by severe pain, a musical mur- 
mur appeared which was noticed by the patient, but which was also objectively 
heard over the left anterior half of the skull, was isochronous with the pulse, 
and disappeared on pressing the carotid artery. Karplus diagnosticated rup- 
ture of an aneurysm of the internal carotid artery in the cavernous sinus, and 
had the common carotid tied whereupon the murmur disappeared: The au- 
topsy (death occurred in consequence of consecutive softening) showed the 
correctness of his reasoning. 

We now turn to a method of investigation which, although of recent date, 
has become of great importance — lumbar puncture. Originally employed for 
therapeutic purposes, this method invented by Quincke soon became very val- 
uable in diagnosis because it permitted the product of secretion of the central 
nervous system, or of its membranes, to be investigated directly. 

The technic of lumbar puncture and its results under normal conditions 
have been described in an article on the subject in this volume (see Lumbar 
Puncture, by Quincke) and we at once turn to pathologic conditions and the 
diagnostic employment of lumbar puncture. The most frequent change noted 
is an increase of pressure; it may appear when the composition of the fluid 
is otherwise unaltered, while in other cases there is a change, both macro- 
scopical and microscopical, in the cerebrospinal fluid. Increase in pressure 
often becomes so great that the fluid which would normally exude drop by 
drop actually flows out in a stream. On estimating the pressure in these cases, 
it has been found to equal 300-500 mm. of water, and even 1,000 mm. (nor- 
mally 40-150 mm.). Such simple increase of pressure is found in hydro- 
cephalus, with rare exceptions in cases in which the communication of the 
ventricles with the subarachnoid spaces of the spinal cord is disturbed, in 
brain tumor, and in abscess, here also with the above mentioned limitations. 
Lumbar puncture has enabled us to recognize a new pathologic picture in- 
variably accompanied by greatly increased pressure of the arachnoid fluid,, and 
which Quincke has designated as serous meningitis. In this condition a rapid 
increase of the ventricular fluid is said to take place, so that it also includes 
cases of acute acquired hydrocephalus. The affection sets in primarily with 
symptoms of cerebral pressure, and if there is fever, tuberculous meningitis 
or epidemic meningitis is closely simulated; in non-febrile cases the symp- 
toms resemble those of brain tumor. Etiologically the cases vary; the dis- 
ease may arise spontaneously or after infectious diseases or intoxications; its 
occurrence in purulent otitis, particularly on account of the difficulty in diag- 
nosis, must be especially mentioned. What, however, stamps this serous men- 
ingitis charactericallv is its curability, either spontaneously or after lum- 
bar puncture; for here, as well as in some cases of chronic hydrocephalus, 
lumbar puncture is of great therapeutic value, which originally was not ex- 
pected to be the case, or at most only transitorily. In a pathogenetic and 
pathologico-anatomical respect the subject of serous meningitis still requires 
elucidation. In a symptomatologic sense, however, we not infrequently observe 



DIAGNOSIS OF DISEASES OF THE BRAIN 219 

its clinical picture. Many a case formerly obscure, many cases of tuberculous 
meningitis apparently cured, of cerebral tumor, etc., belong in this category. 
Increase of pressure is also found in certain forms of severe headache, espe- 
cially in chlorosis, in sinus thrombosis, and in spasmodic attacks, of epilepsy, 
uremia, etc. 

Increase of pressure is often accompanied by changes in the appearance 
and composition of the fluid, and these we must briefly discuss. The most 
conspicuous admixture is with blood, which may, however, be accidental. 
Otherwise, provided hemorrhage has occurred in the subarachnoid space, pure 
blood is not found but it is admixed with the subarachnoid fluid; for exam- 
ple, after trauma of the skull or vertebral column, in hemorrhages of the 
hemispheres which have ruptured into a ventricle, or in hemorrhage upon 
the surface, in ruptured basal aneurysms, and in pachymeningitis which is 
not encapsulated in the subarachnoid space. By diffusion of hemoglobin 
from the surrounding area without a direct macroscopic admixture of blood, 
the fluid may show a slight yellow discoloration, and erythrocytes may be recog- 
nized with the microscope, or hemoglobin with the spectroscope. 

The findings in various forms of meningitis are of great diagnostic signifi- 
cance; the macroscopic and microscopic composition of the fluid, its chem- 
ical alterations, and its bacteriologic investigation may furnish valuable data 
as to the etiology. The appearance of the fluid varies in different forms of 
meningitis; sometimes it appears to be absolutely clear; in tuberculous men- 
ingitis, as shown by Lichtheim. Schiff and others, the subsequent formation 
of a coagulum from this clear fluid is characteristic. Besides, as some authors 
have demonstrated, this occurs also in rare cases of brain tumor, while, accord- 
ing to Orglmeister, it may be absent in tuberculous meningitis. In other 
forms of meningitis small coagula are found even in the native fluid, some 
so large that they may occlude the cannula. Finally, the fluid may show 
turbidity from the slightest degree to actual pus. as, for instance, in some 
cases of epidemic cerebrospinal meningitis. The chemical examination of the 
fluid obtained by puncture may enable us to reach certain conclusions; for 
instance, in different forms of meningitis the amount of albumin is generally 
increased to 3 per 1,000, while a decrease in the amount of reducing sub- 
stance does not form a reliable guide. 

The microscopic examination of the fluid obtained by puncture is of espe- 
cial importance and is best made after centrifugation. Normally only iso- 
lated leukocytes are found, but in meningeal processes their number is greatly 
increased, and in acute processes the polynuclear, in subacute and chronic 
cases the mononuclear, forms predominate or are exclusively present. This 
cyto diagnosis of the fluid in lumbar puncture, as shown by many recent inves- 
tigations of French physicians, has acquainted us with a far more interesting 
fact all the details of which are not as yet clearly defined. Widal, Siccard 
and others — among German authors so far only Schoenborn. Frenkel and 
Mayer have studied the question — have shown that in syphilitic and so-called 
metasyphilitic processes of the nervous system, above all in tabes and 
paretic dementia, an increased number of lymphocytes has been found in the 
cerebrospinal fluid, occasionally also large mononuclear elements. While nor- 
mally with the microscope only three to four lymphocytes are found in the 
fluid, under the previously mentioned conditions this number is increased to 



220 DIAGNOSIS OF DISEASES OF THE BRAIN 

30, 40, or even more. Many French authors regard this symptom as an 
important guide in the diagnosis of tabes, as it may appear very early; for 
example, in cases in which there is only reflex rigidity of the pupil, where, 
therefore, the diagnosis of tabes or paralysis cannot be made with certainty 
but only with some degree of probability. They refer this increased num- 
ber of lymphocytes to an irritative state of the meninges which accompanies 
tabes and paresis, and produces a mild or severe meningitis. This lympho- 
cytosis also occurs but to a lesser degree in metasyphilis without nervous symp- 
toms, a fact which sometimes nullifies its diagnostic importance; in the cases 
of isolated reflex pupillary rigidity, above mentioned, this condition is espe- 
cially vitiated, for we know that reflex pupillary rigidity without the devel- 
opment of tabes or paralysis may occur as an isolated symptom in persons 
who have had syphilis. Lymphocytosis is also found in chronic alcoholism 
and in certain cases of herpes zoster, in which it is produced by the same 
cause as in tabes or paresis. On the other hand, it may be absent in tabes or 
paresis (Dejerine). In this lymphocytosis, therefore, we are dealing with a 
new condition which even to-day is worthy of notice, but many phases of 
which still require elucidation and explanation. 

A case published by Hartmann shows that under some circumstances lum- 
bar puncture may reveal other conditions; for instance, the discharge of a 
cysticercus permitted the diagnosis of cysticercus of the brain which otherwise 
would not have been thought of. 

There is another modern development of diagnosis: Sero-diagnosis — the 
agglutination of tubercle bacilli by the cerebrospinal fluid in tuberculous men- 
ingitis — which was attempted by Donath, but as yet has given no results. 
That sero-diagnosis may lead to errors, even when all precautions have been 
observed, is shown by two cases of tuberculous meningitis in which the fluid 
from lumbar puncture gave the Widal typhoid reaction. 

The demonstration of pathogenic microorganisms in the fluid obtained by 
lumbar puncture, either from the sediment obtained by centrifugation or 
from the coagula by staining methods, or, finally, by inoculation upon culture 
media or in animals has much greater practical value and is sometimes decisive. 
Among the organisms so far found is the tubercle bacillus — according to 
recent reports, with proper methods this is almost invariable in tuberculous 
meningitis, particularly when the coagula is employed for staining — also the 
meningococcus intracellularis, the pathogenic agent of cerebrospinal menin- 
gitis, pneumococcus, streptococcus and staphylococcus, the typhoid and coli 
bacilli, the influenza bacillus, etc. Notwithstanding the presence of micro- 
organisms the fluid may be clear, as in the case of tuberculous meningitis. 
More frequently, however, it is slightly turbid, or even appears purulent. On 
the other hand, with a distinct purulent appearance the fluid may be sterile, 
apparently because the pyogenic microorganisms have disappeared; we know, 
for example, that microorganisms directly and experimentally introduced into 
the circulation and thence passing to the spinal cord or the subarachnoid fluid 
may disappear with relative rapidity after a few days. This experience was 
gained in producing myelitis experimentally, and in part explains why micro- 
organisms are so rarely demonstrated in infectious or post-infectious forms 
of myelitis. It also explains many of the very common relations between 
acute infectious diseases and the grave nervous symptoms which accompany 



DIAGNOSIS OF DISEASES OF THE BRAIN 221 

or follow them, above all, those of a cerebral nature. We see that acute infec- 
tious diseases, whether caused by microorganisms or by a so-called secondary 
infection, perhaps by staphylococci or streptococci, may produce severe, even 
purulent inflammatory processes in the nervous system, particularly in the 
meninges, as well as in other parts of the body, while in other cases the pres- 
ence of microorganisms may cause merely an irritation without a true inflam- 
matory process. But, of course, all such cases are not directly due to micro- 
organisms; often their toxics cause the condition. 

Having briefly described the findings in lumbar puncture, we now turn 
to its differentio-diagnostic importance, i. e., let us consider how and when we 
may base our diagnosis upon the results of lumbar puncture, and how we 
can make a differential diagnosis. Lumbar puncture, we must state at the 
outset, is especially valuable in the differential diagnosis, difficult and often 
impossible from the clinical symptoms, of abscess and meningitis, especially 
purulent meningitis. This question arises in the cases with cerebral com- 
plications from disease of the ear, also in traumatic metastatic processes with 
cerebral symptoms in the course of infectious diseases, in which conditions 
encephalitis and cerebral tumor must under some circumstances be considered 
in the differential diagnosis. While in abscess which has not ruptured nor 
led to secondary purulent meningitis, also in tumor and in encephalitis, lum- 
bar puncture shows an increase of pressure, yet clear fluid is obtained, in 
purulent meningitis we find the changes mentioned above; macroscopically 
there is turbidity, an apparent coagula, upon microscopic investigation many 
cells are noted, above all polynuclear leukocytes which often furnish an eti- 
ological explanation by the demonstration of microorganisms. Tuberculous 
meningitis also must be considered, and may be thus determined. It is true 
that the results of lumbar puncture are not absolutely conclusive. There are 
cases of purulent meningitis in which the process is at first local, the collec- 
tion of pus in the meninges becomes encapsulated; in these, therefore, lum- 
bar puncture may reveal clear fluid. On the other hand, to many surgeons 
purulent meningitis is no longer a noli me tang ere, for here also favorable 
results may be expected from the early evacuation of pus. At all events, 
in these difficult cases lumbar puncture makes our diagnosis more certain, 
and indicates the proper therapy. 

The diagnosis of tuberculous meningitis becomes more positive by lumbar 
puncture, for tuberculous meningitis, especially in the adult, by no means 
always runs a typical course, showing the familiar stages of irritation and 
paralysis so frequently represented in text-books. I do not refer solely to the 
cases in which acute cerebral focal symptoms, paralysis, aphasia, etc., appear, 
or to cases with long intermissions, etc. The question, so long disputed, 
whether tuberculous meningitis is curable, has also been solved by lumbar 
puncture, for with each succeeding year the number of recoveries in cases 
in which the affection was proven by the finding of tubercle bacilli in the 
fluid steadily becomes larger; in some cases this demonstration was subse- 
quently verified by autopsy findings. 

To demonstrate the importance of lumbar puncture, I must refer to what 
was previously said in regard to serous meningitis, many an obscure case of 
which has been made clear by lumbar puncture. Hemorrhages, particularly 
of traumatic origin, or cerebral hemorrhages with external rupture or rupture 



222 DIAGNOSIS OF DISEASES OF THE BRAIN 

into the ventricles, may also be diagnosticated, and thus, under favorable cir- 
cumstances, the differential diagnosis between hemorrhage and softening, 
which sometimes can only be assumed, becomes certain. 

Much more might be said of lumbar puncture, but the foregoing is suffi- 
cient to show that it enables us to determine very interesting and even impor- 
tant facts, and that it is a method to be employed whenever possible. 

In conclusion I must devote a few words to a method frequently resorted 
to by internal clinicians in the differential diagnosis of purulent processes, a 
method which may also be of use in nervous affections when the differential 
diagnosis between purulent processes (abscess and meningitis) and non-puru- 
lent ones (encephalitis, tumor, etc.) is perplexing. This is the examination 
of the blood. It is well known that in suppuration there is an increase of 
the polynuclear leukocytes ; the iodin reaction, which depends upon the amount 
of glycogen produced by the leukocytes during suppuration, may also be of 
value. Eaymond recently diagnosticated local secondary suppuration after 
traumatic pachymeningitis — the patient was trephined — by a leukocytosis 
which subsequently occurred ; the case was cured by a second operation. 

If I were asked to state comprehensively the gist of this article, I should 
say that it set forth the methods which, in common, form physical, chemical, 
or microscopical aids, by the help of which, combined with the consideration 
of the neurologic symptoms, it is possible for us to arrive at more certain 
conclusions, and wherever possible also to define the nature of the underlying 
process. Partly new, hence varying in results, these methods have neverthe- 
less greatly facilitated the diagnosis of cerebral affections. On continued use 
we may hope that these methods may become more certain and reliable. 

We have not yet reached the goal of a clear and precise diagnosis of dis- 
eases of the brain, but we hope that this will be possible in the not far distant 
future, for the path of advance in our science is not marked out by well laid 
plans based upon what has already been acquired. What seems most distant 
may suddenly become attainable, and advancing knowledge may come with 
rapid strides. 



LUMBAR PUNCTURE 

By H. QUINCKE, Kiel 

Puncture is performed for diagnostic or therapeutic purposes when path- 
ologic collections of fluid exist, or are presumed to exist, either in pathological 
and newly formed cavities^ such as cysts and abscesses, or in anatomical cav- 
ities, such as those in the joints, the pleura, the peritoneum, etc. The cav- 
ities of this latter group normally contain only capillary layers of fluid 
between the serous layers ; when puncture reveals fluid, this indicates a patho- 
logic increase. Such cavities are similar to the spaces in the subcutaneous 
cellular tissue from which in anasarca decidedly large amounts of fluid may 
sometimes be removed by puncture and drainage. 

There is only one cavity of the body which in the normal state contains 
a considerable amount of fluid, the cerebrospinal cavity. The central nervous 
system is so suspended in the fluid that its surface nowhere touches the osseous 
capsule. Even in a healthy person some of this fluid can invariably be ob- 
tained if the cerebrospinal cavity is punctured at a suitable point in the lum- 
bar portion of the vertebral column. 

(1) For the better understanding of such puncture and its action and 
results I must refer briefly to the structure of the membranes of the central 
nervous system. 

The dura mater, the fibrous covering of the central organ, is closely adher- 
ent to the cerebral portion of the inner surface of the skull, and at the same 
time forms the periosteum of this bony surface, while in the vertebral canal 
the dura mater forms a loose sac resembling a hollow cylinder, and separated 
from the inner periosteum and the fibrous walls of the vertebral canal by a 
loose connective tissue which contains many large venous plexuses. At the 
height of the second or third sacral vertebra the sac of the dura terminates 
abruptly, narrowing in the form of a tenpin. 

The delicate covering of the central nervous system, the leptomeninx, is 
formed by two membranes designated in descriptive anatomy as the pia and 
arachnoid', in most areas these may be detached by a gross anatomical method 
from each other, but in reality they form the internal and external boundaries 
of the delicate membrane of the brain, the two layers being united by loose con- 
nective tissue. The internal, the pia, rich in vessels, the connective layer of the 
delicate membrane of the brain, covers the surface of the spinal cord just as 
it does the brain, and follows the convolutions on the upper surface of the 
latter deep into the furrows, while the arachnoid follows only the general 
surface, enclosing the brain like a loose sac, covering the fissures like a bridge, 

223 



224 LUMBAR PUNCTURE 

and being in close contact with the convolutions only at their convexities. 
These layers, the pia and arachnoid, are united by a very delicate web of 
connective tissue strands with wide communicating spaces, the subarachnoid 
spaces, between. On the surface of the convolutions the connective tissue 
strands are short, the subarachnoid spaces very shallow; in the furrows the 
latter are deep, the connective tissue strands longer and more loosely arranged. 
The latter is also true of the under surface of the brain, the arachnoid extend- 
ing over its varied convolutions as a smooth, tense membrane, so that here 
we find wide, communicating, subarachnoid spaces ( cisternal ). 

While, therefore, the pia is closely adherent to the brain and spinal cord, 
the arachnoid forms a sac-like covering separated from it by the subarachnoid 
fluid. This sac is continuous with the sac of the dura mater; its external 
surface and the internal surface of the dura lie in close contact, while in the 
cavity of the skull they are separated from each other by a thin layer of 
fluid. This space in the skull between the dura and arachnoid, the subdural 
space, is a few tenths of a millimeter in thickness, while in the cavity of the 
cord it is exceedingly thin, and becomes apparent only in consequence of 
pathologic accumulations. 

Autopsy does not accurately show what the relations are during life, since there 
is no turgescence due to the pressure of the blood and secretions. The varying relations 
of the subdural space in the cavity of the skull and in the spinal cord can be deter- 
mined with certainty only at operations and in vivisections. As Hitzig has demon- 
strated, the subdural fluid in the skull is absorbed soon after death by the substance 
of the brain, so that even a few hours after life is extinct the cerebral subdural space 
seems no longer to exist. 

The diagram on the opposite page, modified from the " Topographical Anatomy " of 
Merkel (page 78, Fig. 38), shows these relations. The subarachnoid spaces, the ventri- 
cles of the brain which communicate with them, and the aqueduct of Sylvius are blue, 
the subdural space between the dura and arachnoid is red, but in the cavity of the spine 
where the arachnoid and dura are in closest contact, this normally no longer exists. 

The subdural space may be regarded as analogous to the pleural or peri- 
toneal cavity; the fluid it contains may be estimated at a few cubic centi- 
meters. The greatest bulk of the cerebrospinal fluid ("subarachnoid") is 
found in the connective tissue meshes of the delicate membrane of the brain 
which Henle has accurately described morphologically as " the physiologic, 
water-seeking, connective tissue." The meshes of this tissue throughout the 
brain and spinal cord communicate with each other ; and this continuity which 
normally exists is such that, aside from special conditions, pathological proc- 
esses from the surface of the brain are very readily transmitted to the spinal 
cord and vice versa, not quite so smoothly as from the serosa of the thoracic 
and abdominal cavities, but much more readily and more rapidly than in 
the normal, or even in the dropsical, subcutaneous cellular tissue. 

(2) The fluid in which the central nervous system almost seems to float, 
is in many ways protective. 

Its displacement with alterations in the position of the body which might 
mechanically damage it prevents marked changes in the form of the spinal 
canal; by its deviations, by its being subjected periodically to a greater afflux 
and efflux notwithstanding the slight relaxation of the capsule of the skull, 
alterations in the amount of blood and in the circulation of the blood through 



LUMBAR PUNCTURE 



225 



the brain become possible; at the same time the narrowness of the outlets 
prevents this change from being rapid or sudden. 

The cerebrospinal cavity contains the following elements: (a) Firm tissue 
which largely forms the central nervous system; (b) Blood; (c) Cerebro- 
spinal fluid. Of these component parts the tissues undergo a change in vol- 
ume to the least extent and only after a long time. 

The amount of blood depends upon the general and local circulation. In 
this respect the most important factor is the height of the blood pressure in 




ubdural 



Fig. 127. 



the aorta and vena cava, the caliber of the arteries which enter the cerebro- 
spinal cavity, of the veins which emerge from it. and the caliber of the cere- 
bral and spinal cord vessels; the latter no doubt varies because of general 
conditions and on account of the richness of the muscular coat of the arteries 
of the brain. It is immaterial whether vasomotor activity, or the composi- 
tion of the blood or the hemic requirements of the nervous substance are the 
determining factors. It is obvious that the amount of blood in the central 
nervous system and in the cerebrospinal cavity must depend on the caliber 
and number of the afferent and efferent vessels, and that this is subject to 
great and rapid variation, provided there are no other modifying conditions. 
The third element in the cavity, the cerebrospinal fluid, serves mainly as 
compensation for the first and second. Its amount is adapted to the varia- 



226 LUMBAR PUNCTURE 

tions in volume of the brain substance ; when this is decreased it increases as 
meningeal edema or ventricular dropsy (hydrops ex vacuo) ; more rare and 
less conspicuous is its decrease in neoplasms and hemorrhages. 

The regulating and controlling effect of the cerebrospinal fluid on the 
variations in the amount of blood, especially within the skull,- is much more 
important. It is true the conditions here are complicated, for this fluid is 
itself a secretion from the blood and, moreover, is secreted from diverse areas : 
From the choroid plexus of the ventricles, from the subarachnoid spaces on 
the surface of the brain and the spinal cord and, finally, from the lymph 
tracts of the central nervous system which empty into it. 

The outlet for the cerebrospinal fluid is by means of narrow channels 
through the villi of the arachnoid to the venous sinuses, thence through the 
subdural space and the dura to the lymph vessels of the neck and throat 
(possibly also to the nasal mucous membrane and the labyrinth of the ear), 
and probably along the sheaths of the nerves of the brain and spinal cord. 

The cerebrospinal fluid, therefore, resembles a stream which receives tribu- 
taries from several sources, the importance of which, even physiologically, 
may vary, and of which pathologically sometimes one, sometimes another, 
may be paramount. As an example of this variation I may cite the action 
of ventricular dropsy in obliterating all of the subarachnoid spaces, of acute 
meningeal edema upon normal ventricles, and of diffuse edema of the brain. 
The increased exudation of fluid (whether in one of the previously mentioned 
forms or in their combination) may decidedly affect the caliber of the blood- 
vessels, so that the amount of blood they contain is decreased. 

(3) The general conditions under which the brain receives its supply 
of blood and nutrition are peculiar from the fact that the organ is enclosed 
in a bony capsule which yields but little to pressure, although it contains 
spaces and has outlets which have a wide external point of communication in 
the foramen magnum. The skull of the child, prior to complete ossification 
of the sutures, still yields to a certain extent, but the capacity of the cranial 
cavity of the adult is practically unchangeable, and consequently the amount 
of blood it contains depends upon the position of the body. If a greater 
volume of blood flows to the brain and a dilatation of its vascular tracts there- 
fore becomes necessary, this can only be brought about by a corresponding 
decrease in the amount of cerebrospinal fluid, either by its actually flowing 
into the veins and lymph vessels, or by its passing into the subarachnoid spaces 
of the yielding spinal sac; the latter mode of compensation would appear to 
be much more rapid, and therefore, in the frequent and slight functional vari- 
ations in the amount of blood in the brain, is probably the usual process. The 
capacity of the spinal dural sac is limited, although there are variations due 
to its natural firmness and the resistance of its external surfaces. Of these 
surfaces the firmness of the bony and ligamentous vertebral' canal and the vary- 
ing pressure of the peridural venous plexuses may be especially mentioned. 

While the conditions regulating the volume of blood in the cavity of the skull may 
be compared with those of the medullary cavity of a bone, in the spinal cord this bears 
a certain but imperfect analogy to the process in organs which, like the testicles or the 
kidneys, are surrounded by a firm fibrous capsule. A crude idea of these conditions is 
conveyed by an inverted glass bulb, the neck of which is turned downward and con- 
nected with a wide rubber tube closed at the end. The volume of fluid within the 



LUMBAR PUNCTURE 227 

skull (the bulb) is the same under all circumstances. The pressure which it sustains 
depends upon its fulness and the elastic tension of the dural sac (the walls of the 
tube ) . This pressure is the same at all points upon the same horizontal plane, but 
different at varying heights because hydrostatic pressure must be added to the elastic 
pressure. It varies, therefore, at a given point with the position which the body 
assumes. If the distance between the floor of the bulb and the end of the tube is 60 
cm., on placing the model in a vertical position the pressure at the lower end of the 
tube will be respectively + 60, + 80, + 40 cm., if the pressure upon the floor of the 
bulb is respectively 0, + 20, — 20. With a horizontal position of the body the pressure 
at both points is the same, and with an inverted vertical position (standing upon the 
head) it will be 60 cm. less at the end of the tube than in the bulb. 

(4) The amount of pressure in the cerebrospinal cavity depends in the 
concrete case upon the following factors : 1. The mass of tissue present (cen- 
tral nervous system + pathologic products); 2. Upon the blood-pressure; 
3. Upon the amount of cerebrospinal fluid secreted. 

The effect of blood-pressure is due to the arterial pressure in so far as 
this is not conveyed by the walls of the vessels and by the tissues, also by the 
extremely varied pressure in different veins. The result is designated by 
Pfaundler as " transmissible blood-pressure." 

The effect on the cerebrospinal fluid of the pressure of secretions is further 
modified by the rapidity of secretion and the caliber of its outlets. 

In order to understand these conditions it is absolutely necessary for us 
to bear in mind that the amount of fluid in the cavity is not invariable but 
subject to continued variations, and that the three factors previously enumer- 
ated alternate with one another in a certain rhythmic measure. By their 
combined action pressure is exerted upon the internal surface of the cerebro- 
spinal cavity, and its walls so far yield that the spinal dural sac becomes dis- 
tended and tensely elastic. We may designate pressure produced in this way 
as " elastic pressure." Because of the free communication of the subarachnoid 
spaces with each other, this elastic pressure is equal throughout the cerebro- 
spinal cavity, and, except under special conditions, alterations in pressure in 
one part of the cavity must always, even though somewhat modified, be trans- 
mitted after a time to the entire tract ; thus the increased pressure trans- 
mitted from the contents of the skull to the spinal dural sac (which as an 
elastic supplement simultaneously forms a safety valve) may be compared 
with that of the air chamber of a pressure syringe. 

(5) In man the only point at which we may gauge the pressure in the 
cerebrospinal cavity, and perhaps decrease it by evacuating some of the fluid, 
is the spinal portion of the dural sac. As the spinal cord of the adult with 
its conus medullaris extends only as far as the top of the second lumbar ver- 
tebra, below this we find only the loose bundle of descending nerve roots, 
the cauda equina, the individual filaments of which are somewhat motile and 
extend through the cerebrospinal fluid with such wide interspaces that, on 
inserting a cannula at this point, it freely enters the fluid. 

At most points above the second lumbar vertebra, the subarachnoid space is wide 
enough (1 to 3 mm.) to permit the introduction of a cannula. Practically, however, 
we might either insert it beyond the dura and readily injure the spinal cord or by 
accidentally displacing the cannula even to the extent of 1 mm. we might glide beyond 
the dural sac. Therefore, puncture of the spinal sac can be practised only in the lum- 
bar portion, and to make this clear, instead of the more general expression spinal 



228 LUMBAR PUNCTURE 

puncture, I have chosen the term lumbar puncture. Of the subarachnoid spaces of the 
brain, those which lie between the cerebellum and the medulla, on account of their 
width and position, permit the introduction of a cannula. In animal experiment this 
has been done by exposing and punctuVing the membrana atlanto-occipitalis ; in man 
such a puncture through the skin is impracticable on account of the close proximity 
of the vital centers. The pressure within the skull cannot be determined by the intro- 
duction of a cannula, even in animal experiments, owing to the narrowness of the 
subarachnoid spaces, and even if, as v. Leyden has proposed, a pressure tube is screwed 
into the skull through a trephine opening, the measure of pressure may readily be 
disturbed by a protrusion of the brain. 

Puncture of the lumbar arachnoid space (lumbar puncture) may be read- 
ily performed in man and without danger; the needle should be inserted 
through the skin and the muscles of the back into the soft parts between the 
arches of the vertebrae. I first advised lumbar puncture as a therapeutic 
measure, but it has at the same time given us an insight into the causes of 
pathologic processes which take place in the cerebrospinal cavity, and into the 
pressure condition of this cavity which, as mentioned above, embraces both 
elastic and hydrostatic pressure. 

The height of the pressure, which we should never neglect to' ascertain 
when performing lumbar puncture, is estimated as follows: The cannula, 
after insertion into the subarachnoid space, is attached to a small rubber tube 
with a glass tube at its end, and this is raised perpendicularly just as soon 
as the first drop of fluid appears. The fluid is received in this vessel, and 
its height, measured vertically above the point of puncture, indicates the 
pressure existing in the subarachnoid space. If lumbar puncture is performed 
in the horizontal lateral position, we generally estimate the " elastic pressure," 
not only as it exists at the point of puncture, but approximately also within 
the cavity of the skull, for with this position of the body the difference in 
height between the seat of the puncture and the highest point of the skull is 
very slight, and the proportion of hydrostatic pressure to the entire pressure 
estimated is also very low. The conditions are different with an erect position 
of the body; here the vertical difference between the vertex of the skull and 
the point of puncture amounts in the adult to about 60 cm. In this position 
we invariably find higher values in lumbar puncture because of the increase 
of hydrostatic pressure — of course, this is not so great as to correspond with 
the altered position and the height of the vertex of the skull. 

Pfaundler has made comparative estimations on this line; he first measured the 
pressure during lumbar puncture of children in a sitting posture, then in a recumbent 
position, and in the former position he determined the vertical difference between the 
point of puncture and the vertex of the skull. In children whose cranial bones had 
united the difference in these pressure values averaged only 21 per cent, of the ideal, 
hydrostatic components (H) ; in children whose fontanelles were open, 33.9 per cent. 
From this it is evident that the system of subarachnoid spaces does not act like a 
wide communicating tube, but that some of this pressure (certainly, some time after 
a change of position has occurred) is borne by the walls, and is, perhaps, also com- 
pensated for by the dissemination of fluids to other higher tracts. 

The hydrostatic pressure in children with hydrocephalus was somewhat greater 
(namely, 27 to 53 per cent.) ; in these cases the spinal cavity is usually more or less 
dilated. 

Kronig found the lumbar pressure in adult healthy persons who were in a hori- 
zontal position to be 125 mm., in a sitting posture (where muscular tension adds 
something to the increase in pressure) 410 mm. If the difference from the point of 



LUMBAR PUNCTURE 229 

puncture to the vertex of the skull (about 60 cm.) is expressed by H u and that from 
the foramen magnum (about 40 cm.) by H 2 , the difference (285 mm.) equals 48 per 
cent, of Hi and 71 per cent, of H 2 . 

(6) The position of the fluid in the pressure tube almost invariably 
shows variations which may be ascribed to different causes. Most distinct 
is the influence of respiration : The pressure falls with inspiration and rises 
with expiration, being greater after prolonged respiratory movements; the 
difference amounts to 20 mm. More slight (1-4 mm.) and less invariable 
are the variations in pressure due to pulsations of the heart. In some cases 
there is a rise which is synchronous with the systole of the heart, and in others 
a fall of the fluid; I am unable to state the amount of this variation. In 
addition to these respiratory and arterial variations others are sometimes 
observed which amount to from 10 to 30 mm., and vary in duration from 10 
to 30 seconds. The cardiac systolic and the respiratory variations in the 
pressure of the arteries and veins are transmitted throughout the entire course 
of the cerebrospinal cavity to the subarachnoid fluid; in lumbar puncture the 
narrowness of the spaces scarcely permits those generated in the cavity of the 
skull to be noticeable in the pressure tube; the variations in pressure which 
are here visible are probably produced mainly from the spinal cavity, and 
especially by the varying fulness of the voluminous, peridural, venous plexuses. 
Great increase in pressure (several hundred millimeters) is caused by forced 
expirations as in cough, straining, or screaming; the pressure is still further 
increased by increasing muscular resistance, particularly in epileptic spasms. 
Xawratski and Arndt measured the pressure in the tonic stage and found it 
to amount to 750-870 mm., while previously and subsequently it was normal. 
Muscular action for the development of this brief but marked increase in 
pressure may be directly transmitted through the ligaments and other tissues 
to the spinal dural sac. 

If the continuity of the spinal subarachnoid space be interrupted at any point, the 
cardiac and respiratory variations will probably be lessened, since they are only trans- 
mitted* to the lower portion of the dural sac. Henneberg believes that this may be 
utilized in the differentiation of myelitis and compression myelitis. 

In practice it is, of course, necessary to determine the height of the cere- 
brospinal pressure while excluding all of these disturbing factors which may 
increase it, but the numberless influences which must here be considered 
probably account for the variations in the height of the normal pressure in 
lumbar puncture. In our experience, normal pressure may vary between 40 
and 130 mm. water; 150 mm. is the limit, and 200 must always be regarded 
as abnormal. 

With cerebrospinal fluid of a low specific gravity, 1.007-1.010, the height of the 
column of fluid may be read off accurately as water pressure; it is much easier to use 
these figures directly than to calculate them as mercury pressure (dividing by 13.6). 
The average blood-pressure in man (120 mm. mercury) amounts to 1,620 mm. water. 

(7) Under pathologic conditions the cerebrospinal pressure frequently 
rises, and this is of importance for the function and nutrition of the brain. 
The conditions within the skull are decisive for the height of the pressure, 
since here are the principal secretions and here also their chief outlets. The 



230 LUMBAR PUNCTURE 

spinal cavity, so far as we know, is as little concerned actively in the increase 
of pressure as the spinal cord appears to be susceptible to it. 

Pathologic processes which may produce pressure within the brain are the 
following : 

(a) Proliferation of tissue which decreases space, tumors of the brain 
substance or those in the soft or hard membranes which compress the organ, 
inflammatory swelling of different areas of the brain, and fragments of bone 
which mechanically compress it; 

(b) Effusions of blood in or between the membranes of the brain or in 
the brain substance; 

(c) Purulent and serous exudates, generally of inflammatory origin. The 
more liquid, hence the more displaceable these are the more likely they are to 
produce general pressure or local symptoms; they are frequently combined 
with the processes enumerated under 1 and 2, which at the same time increase 
the normal secretion of the cerebrospinal fluid. The principal source of the 
latter is evidently the venous plexuses of the ventricles of the brain; thence 
the fluid finds its way through the aqueduct of Sylvius and the foramen of 
Magendie also through spaces in the tissue, along Galen's veins, and laterally 
past the tonsils of the cerebellum into the subarachnoid spaces. Hypersecre- 
tion of the venous plexuses is generally to be regarded as inflammatory. Some- 
times microorganisms are found in this secretion, for example, tubercle bacilli, 
but more frequently they are absent and a chemico-toxic action must be as- 
sumed. Accordingly we may separate ventricular meningitis into simple and 
bacterial forms. Serous exudates sometimes form acutely but are so transi- 
tory that they cannot be regarded as inflammatory but of angio-neurotic 
origin. 

In compression of the vena magna Galeni (by tumors, etc.) stasis hyper- 
emia arises in the venous plexuses of the three anterior ventricles, and this 
causes increased transudation. 

When the aqueduct of Sylvius or the foramen of Magendie is obliterated, 
even though the secretion be normal in amount, the ventricles are dilated be- 
cause of the stasis of the secretion and hindrance to its outlet. 

The effect of profuse serous transudation is most obvious in the simple 
hydrocephalus of children; here the sutures and fontanelles which have not 
yet closed permit us to recognize the pressure existing; if the exudation is 
permanently increased, these appear tense and stiff, but again become flaccid 
after the pressure is relieved. 

In internal hydrocephalus the lateral ventricles are most markedly, and 
the fourth ventricle the least, distended; when the aqueduct of Sylvius is 
closed the distention may be limited entirely to the three anterior ventricles. 

In internal hydrocephalus the contents of the ventricles of the brain, 
which may normally be estimated at from 20 to 30 cc, may amount to sev- 
eral hundred or even to more than a 1,000 cc. The ventricular dropsy due 
to hypersecretion presses upon the brain, and forces it to the inner surface 
of the skull. The convolutions are flattened and the fissures obliterated. 

The subarachnoid spaces on the surface of the brain are implicated in 
the bacterial inflammations of the pia, especially so in the purulent ones, 
and here serous exudations are found in the form of meningeal edema; but 
the number of observations proving the frequency with which these " edemas " 



LUMBAR PUNCTURE 231 

accompany an increase in intracranial pressure is insufficient. Some cases, 
for example in uremia, in plague, and in influenza, may be of toxic or angio- 
neurotic origin; and in the transitory, rapidly cured cases in which transuda- 
tion causes an increase in pressure, we cannot differentiate those of subarach- 
noid exudation from those chiefly of ventricular origin. 

The absorption of the fluid into the general circulation might just as read- 
ily take place by diffusion into the blood-vessels of the cerebral and spinal 
cord membranes as by the previously mentioned outlets. In how far the 
former vent is to be at all considered we do not know; the preformed outlets 
are found particularly in the cavity of the skull just as in the organs of 
secretion. 

Whether occlusion (other than occlusion of the ventricles themselves) may 
alone produce an increase in pressure is unknown to us. 

On the other hand, in every case of dropsy of the ventricles with disten- 
tion, from pressure of the cerebral convolutions against the skull, the subdural 
space, the subarachnoid spaces, the path to the Pacchionian granulations, and 
the remaining outlets are narrowed. Thus a new course of increase in pressure 
arises, and the spinal sac perhaps absorbs fluid to a greater extent than under 
normal conditions. 

According to Bonninghaus (1. c, pages 345 et seq.), ventricular dilatation 
in itself ("automatically") brings about the transference of pressure from 
the foramen of Magendie to the occipital bone, or an acute exudation by 
kinking of the aqueduct of Sylvius. 

Finally, as will be more minutely explained later, in enlargement of the 
brain by internal hydrocephalus or other causes, the foramen magnum is often 
closed as by a plug, and thus communication between the spinal and cerebral 
subarachnoid spaces is cut off. Not only the complementary spinal outlets 
for the liquor cerebralis, but the cavity of the skull becomes an absolutely 
rigid capsule which no longer acts by its yielding and elasticity as a safety 
valve for the spinal dural sac. 

Intracranial increase in pressure, therefore, leads to a number of sequelae 
which, the higher the pressure rises, make compensation increasingly diffi- 
cult, and thus produce a true vicious circle. The process may be compared 
to the kinking and incarceration of an intestinal coil, perhaps even better 
to acute glaucoma, in these conditions at one time one, at other times another, 
factor is predominantly active. 

We might extend the use of the expression a automatic closure " still fur- 
ther and include all of these active factors. 

In the pathogenesis of these conditions the observations made by Nolke in the 
Clinic at Kiel are especially interesting and are as follows : In four cases in which, 
on account of a more or less complete closure at the foramen magnum, the cerebral 
pressure could not be reduced by lumbar puncture the ventricles of the brain were 
directly punctured by a small opening through the fontanelles into the skull. The 
fluid thus obtained invariably showed much less albumin than that previously obtained 
in the same case by lumbar puncture, the amount of albumin per thousand being: 

t In Case I. ^ 

In the lumbar fluid 2.7 3.0 4.7 

In the ventricular fluid A trace. A trace. 

There are, so far as I can see, only two possible explanations of these findings: 
Either communication between the skull and the cavity of the cord had been perma- 



n. 


in. 


IV. 


2 


1 


1 


1 


0.2 


A trace. 



232 . LUMBAR PUNCTURE 

nently cut off, in which case a fluid much richer in albumin would have been secreted 
in the spinal subarachnoid spaces than in the ventricles — or communication existed but 
was incomplete, in which case the fluid secreted in the ventricles during its passage to 
the lumbar portion would have become richer in albumin by absorption. The latter 
condition is similar to that in the kidney where, in the vascular nodules of the 
glomeruli, a very thin fluid is excreted which develops into urine from absorption of 
solid constituents in the uriniferous tubules ; only additional comparative investigations 
of the spinal and ventricular fluids in cases where there is open communication will 
decide this question. It is certain that the cerebrospinal fluid is not uniform, but is 
a mixture the origin and composition of which varies. 

(8) The rises in pressure observed in lumbar puncture frequently equal 
300 mm. of watqr; 500 mm. may be designated as high, 700 as extremely 
high pressure and is not frequently seen; 1,000 mm. is very exceptional. In 
children the normal as well as the pathologic figures are upon the average 
somewhat less than in adults — about three-quarters. 

Lumbar pressure is generally in proportion to the clinical symptoms of 
brain pressure (headache, slowing of the pulse, vomiting, etc.), but, of course, 
to this there are exceptions. We know that characteristic pressure symptoms 
in severe and even in fatal cases may be slight or entirely absent, and in such 
cases the estimation of the pressure is supplementary and corrective, and 
of more significance in deciding upon the condition than the functional symp- 
toms. Generally in acute diseases there is a moderate increase in pressure with 
severe clinical symptoms, in chronic diseases markedly increased pressure 
with slight pressure symptoms ; in the latter there is also frequently a grad- 
ual compensation of the brain. 

A series of estimations of pressure permits more certain conlusions than 
a single puncture, because extraneous influences are less potent, and we obtain 
some idea of the progress or decline of the disease. 

While the sutures of the skull are open (open communication of the ven- 
tricles with the spinal subarachnoid spaces) the visible and palpable tension 
of the fontanelles is a measure of the height of the lumbar pressure; and we 
may convince ourselves that the latter is increased by mere external palpation. 

(9) Lumbar pressure probably becomes subnormal with lowered blood- 
pressure in collapse and chronic debility, but this is of no practical utility, 
and has therefore not been investigated in man. But the finding of low lum- 
bar pressure with an undoubted existing intracranial increase in pressure is 
significant. Except in those cases in which the cannula is occluded by nerve 
roots, by pus flocculi or blood coagula, and the observation is consequently 
erroneous, when the cannula directly enters the spinal subarachnoid space, 
and shows low pressure the passage to the upper part of the cerebrospinal cav- 
ity is wholly or partly obliterated. For example, this occurs when the sub- 
arachnoid space in the upper part of the spinal cord is obliterated by a gelati- 
nous exudate or cicatricial connective tissue, when the foramen Magendie is 
impermeable, or when the foramen magnum is occluded by the brain itself '; 
the latter may be the case in hydrocephalus of all of the four ventricles or 
when the brain mass itself is enlarged; the brain then lies in the cavity of 
the skull like a tense bladder. This occlusion may happen in acute serous 
effusions and in edema of the brain, as well as in chronic conditions (for 
example, in tumors, especially of the cerebellum) ; in these cases the tonsils 
are frequently seen at either side of the medulla oblongata, where with the 



LUMBAR PUNCTURE 



233 



neighboring convolutions of the cerebellum which are elongated and in shape 
more or less like a cone they occlude the foramen magnum like a conical plug 
(Fig. 128 ). 1 Owing to these conditions, the subarachnoid communication 
between the cavity of the skull and the spinal cord is at first narrowed, but 
with an increase of intracranial pressure it may be periodically or permanently 
obliterated. 

Under such circumstances, and in spite of high intracranial increase in 
pressure, lumbar puncture gives only slight or normal pressure values. It 
sometimes happens that when 
we begin to measure, the 
pressure is high, but after 
the evacuation of one or sev- 
eral cubic centimetres it falls 
very rapidly to normal or be- 
low; this is because there was 
at first only limited com- 
munication with the cavity 
of the skull, but after the 
evacuation of some of the 
fluid the spinal pressure de- 
clined so markedly because 
of intracranial pressure com- 
pletely occluding the fora- 
men magnum. Attention is 
drawn to this threatening oc- 
clusion hj two circumstances: (a) When conspicuous differences in the 
height of pressure are revealed upon repeated punctures in spite of the other 
symptoms remaining the same, (b) When there is an extremely rapid de- 
cline in pressure during puncture. If we suspect a tumor of the posterior 
cavity of the skull, this observation may confirm the diagnosis. It is prac- 
tically important because it shows how very cautious we should be in per- 
forming puncture, for, in those cases in which puncture has been followed by 
severe cerebral symptoms or even a fatal result, the occlusion spoken of was 
probably the cause. 

Under such circumstances the fluid must be evacuated with great care. 
When there is a rapid decline of the pressure, or if functional brain symp- 
toms appear, the withdrawal of fluid must be interrupted (perhaps the fluid 
should be returned ) . In these cases lumbar puncture should not be attempted, 
and it would perhaps be wiser to puncture the ventricles of the brain directly 
(compare Xblke, 1. c). 

(10) Composition of the Fluid Obtained by Puncture. — The quantity of 
fluid obtained by puncture varies from a few drops to several hundred cubic 




Fig. 128. — Two-thirds Natural Size. 



i Chiari has described in congenital hydrocephalus frequent and conspicuous anoma- 
lies in the form of these parts, and has illustrated these without discussing the 
mechanical consequences mentioned above. The same deformities, however, also occur 
in adults and, without being so marked, may produce complete occlusion. My colleague, 
Heller, has made a full report of these. 

The illustration is from the brain of a girl, aged eighteen, in whom meningitis 
developed from otitis media, and subsequently diffuse inflammatory edema of the brain. 



234 LUMBAR PUNCTURE 

centimeters; large quantities are found when the effusion is clear and purely 
serous. 

The normal cerebral spinal fluid is perfectly transparent and as colorless 
as water ; it has the same appearance but is more profuse in serous meningitis 
and hydrocephalus. There are all transitional stages from slight turbidity 
up to a sero-purulent state ; as a rule, pus is uniformly distributed ; occasion- 
ally there are small flocculi in an almost perfectly clear fluid. On standing, 
the suspended cells form a sediment even though the fluid at first appeared 
perfectly clear; occasionally a clear fluid precipitates fibrin in the form of 
small flocculi, more rarely as an extensive network. In the fibrin flocculi, the 
deposit of which may be increased by the introduction of a small piece of 
cotton, scant cellular elements and microbes accumulate in large numbers. 

Braunstein alone mentions a purely molecular turbidity without morphotic 
constituents which could be determined microscopically. 

Microscopically the normal fluid shows no cells, but sometimes isolated 
endothelia; turbidity is usually due to pus corpuscles. In acute inflamma- 
tions only polynuclear elements are found; in subacute or more prolonged 
processes we may find these, or even mononuclear lymphocytes (Bendix) 
exclusively. 

The fluid obtained by puncture frequently contains small admixtures of 
blood, which, without distinctly coloring it, produce the faintest turbidity, and 
are only recognizable when deposited. These small admixtures of blood are 
due to the introduction of the needle, and generally appear with the first few 
cubic centimeters. This permits a differentiation from blood which is a con- 
stituent of the fluid itself, which is usually admixed in larger quantities, and 
distinctly colors the fluid. We observe this in hemorrhage from the sub- 
arachnoid space ; for example, after injuries of the skull or of the vertebral col- 
umn, in ruptured aneurysm of the cerebral arteries, and when intracerebral 
blood foci rupture into the ventricles. 

In intermeningeal (subdural) hemorrhages (therefore in pachymeningitis) 
no blood is found in the fluid unless the arachnoid is simultaneously injured. 

Sometimes blood flows from the cannula when its point is before or behind 
the dural sac in the venous plexus. It differs from the hemorrhagically 
stained cerebrospinal fluid by its slower flow and greater discoloration. Upon 
sedimenting, the cerebrospinal fluid admixed with blood is but slightly stained, 
on standing in a tube it deposits blood, but usually shows no, or very slow, 
coagulation. If only a few drops of blood are exuded, we can generally decide 
whether the fluid previously contained blood or it was admixed during the 
puncture. 

Often the fluid contains pathogenic microbes, and up to the present time 
the following have been found : Tubercle bacilli, the meningococcus intracellu- 
laris, pneumococci, streptococci, staphylococci, the pneumobacillus, typhoid, 
coli, and influenza bacilli, bacterium aerogenes meningitidis, thick movable 
rods (Stadelmann), the bacillus tetragenes, and actinomyces (Sicard). Since 
microbes produce inflammation in the cerebral membranes, the fluid which 
contains them is usually more or less rich in cells, sometimes of a distinctly 
purulent character; but pneumococci, streptococci, and typhoid, coli and 
influenza bacilli may also be present in purely serous exudates. The microbes 
are found in the sediment, after centrifugation, and in the fibrin coagula, 



LUMBAR PUNCTURE 235 

Microscopic investigation and cultures do not always give like results. 
Where nothing is revealed microscopically, culture may sometimes give a posi- 
tive result; on the other hand, microbes that have perished may be found in 
stained preparations but it is impossible to develop these in culture; this is 
particularly true of the meningococcus. 

Purulent fluids are not rarely free from microbes; and, moreover, a clear 
fluid may be obtained by puncture, yet, further up, the pia may show puru- 
lent infiltration. 

The specific gravity of the cerebrospinal fluid is normally 1.006 to 1.007, 
in pathologic cases it is sometimes higher, 1.008 to 1.009, exceptionally it may 
be 1.013, but these figures are inconstant and therefore of slight diagnostic 
importance; in the estimation of small quantities at hand, and because the 
temperature must be considered, the use of the scales or the areopycnometer 
is necessary, and the process thereby becomes laborious. 

The normal cerebrospinal fluid contains albumin, and in chronic hydro- 
cephalus this is 0.2-0.5 per thousand; sometimes there may be merely traces. 
According to Halliburton, it consists exclusively of globulin, and only when 
there is inflammation is serum albumin found. When pus and blood are 
present, the amount of albumin increases to 8 per thousand and more, and 
even when the fluid is clear it may amount to 0.5-2.0 per thousand, which 
usually indicates inflammation; in a case of hydrocephalus due to venous 
stasis I saw 7 per thousand of albumin. 

A reducing substance which most authors regard as sugar is found in 
the normal fluid and in chronic hydrocephalus. It is inconstant in tumors; 
it is absent in the fluid of meningitis, but may be found in slight inflamma- 
tory conditions; therefore, the diagnostic importance of its absence is uncer- 
tain. When only a small quantity of fluid is at our disposal this test in par- 
ticular is often omitted. 

Fibrin coagula point with great likelihood to existing inflammation, but 
may also sometimes be produced secondarily by a tumor. 

Hemoglobin from a transudate may give to the fluid a yellow color (in extradural 
effusions of blood, Rendu and Geraudel ) . 

The cerebrospinal fluid also contains normally a diastatic ferment, cholin (Cavaz- 
zani, Grober), which is increased in paralytics and in meningitis (Gumprecht). 

The virus of hydrophobia was found by Deniges and Sabrazes, and the tetanus 
toxin by Gumprecht, in the puncture fluid, but other authors failed to find these; the 
agglutinins of blood serum (for example, of typhoid) are either not found or exist 
only in very small quantities in the cerebrospinal fluid ; the hemolysins and alexins 
are also absent. 

The secretion of the fluid is composed of selected materials, as is proven by the 
fact that it contains more potassium salts than the blood serum (A. Schmidt), and 
that ferrocyanid does not pass from the blood, and iodin only very slowly, into the 
cerebrospinal fluid ( Cavazzani ) . Sicard never saw iodin and methylene-blue pass into 
the normal cerebrospinal fluid. According to Widal and Monod the same condition 
occurs in tabes, paralysis, and inflammation due to the meningococci, while in tuber- 
culous meningitis iodin passes from the blood to the fluid. 

In uremia, in tuberculous meningitis, and in enteric fever, the fluid may have a 
toxic effect ( Dircksen ) . 

As a guide in diagnosis the pressure and the amount of albumin in the 
fluid should be measured with each lumbar puncture, and it should be micro- 
scopically examined for cellular elements and microbes. 



236 



LUMBAR PUNCTURE 




Fig. 129 a. 



(11) Method of Performing Lumbar Puncture. — The patient should be 

placed upon his left side near the edge of the bed, the body inclined a little 

forward, the chin 
as nearly as possi- 
ble approaching the 
knees, the operator 
sitting beside the 
bed ; the needle 
should be intro- 
duced between the 
third and fifth 
lumbar vertebral 
arches. When the 
dural sac is reached 
and the fluid be- 
gins to flow from 
the cannula, the 
mandrin should be 
removed and a hol- 
low metal cone 
should be closely 
attached to the 

needle and this again to a narrow rubber tube with a glass tube at its end. 

As soon as fluid appears, the glass tube is held to such a height that the fluid 

column is in equilibrium, when its 

vertical position from the point of 

opening will show the existing pres- 
sure; after this has been noted 

(naturally taking into account the 

previously mentioned secondary in- 
fluences) the glass tube is lowered 

(10 to 20 mm.) below the height of 

pressure so that the fluid may slowly 

drop into a graduated cylinder. At 

the conclusion of the operation, the 

needle is withdrawn, and the fluid 

which is seen to exude from it is 

allowed to trickle into the vessel con- 
taining the rest of the fluid, and the 

point of puncture is closed. 

I shall now describe the details. 
A hollow needle, 4 to 10 cm. long 

and 0.8 to 1.6 mm. in thickness, serves 

as the instrument for puncture; all of 

my needles except the smallest sizes 

have a steel mandrin which fits them 

exactly and extends to the point, being 

ground so as to match the slanting surface of the needle. I believe the 

mandrin very useful because, if the needle does not at once enter the dural 




Fig. 129 b. 



LUMBAR PUNCTURE 



237 



sac and while we are moving its point about, particles of tissue may easily 
slip into the opening and occlude it. After passing the interarcual space 
the mandrin may be removed, and the position of the needle may be shifted 
a little until fluid appears. The handle is attached to a well fitting cone 
with a rubber tube and a glass tube; both should be of narrow lumen (1.5-2.0 
mm.) so that too much fluid shall not escape before the pressure is read. 
The adjoining illustration shows the proper shape of the glass tube for the 
outlet of the fluid; this should be from 10 to 15 cm. long, the length of the 
rubber tube being 20 to 40 cm. Its length should correspond with the expected 
pressure, and if insufficiently long it may be extended. 

To measure the pressure we use a metallic rule half a meter in length, 
this being held vertically with the zero figure at the point of puncture; or a 
metallic tape-measure may be used. 

To prevent the outpouring fluid from filling the tube, "Wilms attaches the cannula 
to a small mercury manometer by a short connecting piece. Kronig has a stop-cock 
on his puncture needle, attaches this by a short piece of rubber tubing to a T-shaped 
glass tube, one of the parts serving for the outflow, the other for measuring the pres- 
sure, and this may be lengthened if necessarj\ I found no special advantage in these 
modifications, and prefer my own more simple method, especially as it permits the 
reading of the pressure at any moment during the evacuation. 

Needles of varying length and thickness must be chosen according to the 
size of the body and the thickness of the soft parts; but, as a rule, three 
sizes only are used. Of course, the instrument 
must be absolutely clean and sterilized. To 
prevent rust, the needle and mandrin after 
cleansing should be finally rinsed with alcohol 
and placed in glass tubes, the mandrin not 
being inserted in the needle. The needles of 
iridium are very satisfactory and durable. 

The left horizontal lateral position described 
above is the best position for lumbar puncture. 
This permits the greatest possible curvature of 
the spinal column anteriorly, and produces a 
separation of the arches of the lumbar verte- 
brae. The patient's condition may make it de- 
sirable to choose the right lateral posture, but 
this is always less convenient for the operator. 
A sitting posture would afford us a better view 
of the anatomical landmarks and help to main- 
tain the mid-plane, but this is for many patients 
very exhausting and even impracticable. With 
this position the pressure figure read off indi- 
cates the hydrostatic pressure as well as the 
elastic, but does not accurately show the amount 
of the former ; besides, if the outflow is not very 
carefully regulated, a sudden fall in pressure may easily occur, and serious or 
even dangerous consequences ensue because the fluid is then actually drawn 
out of the cavity of the skull. For these reasons I never perform lumbar 
puncture in the sitting posture. 




Fig 



One-half Natural 
Size. 



238 



LUMBAR PUNCTURE 



Careful disinfection of the skin is of course necessary, especially in uncon- 
scious or uncleanly patients. 

Anesthesia is not necessary in lumbar puncture except in quite rare cases 
of mania, severe agitation, or spasm; even local anesthesia I rarely employ. 
The pain from the cutaneous wound is but momentary, and needling the mus- 
culature is absolutely painless if the patient is quiet; in passing through the 
periosteum and into the dura itself there is some pain; the latter factor is, 
under some circumstances, a valuable guide. 

Anatomical Relations. — As the conus medullaris in the adult terminates 
between the first and second lumbar vertebrae, the four lower interarcual 
spaces of the lumbar vertebrae are best adapted to lumbar puncture. The 
needle should enter the dura exactly in the middle line; the dura (includ- 
ing the adjacent arachnoid) is readily penetrated on account of its tension. 
The point of the needle rests in the median plane between the nerve 
trunks of the cauda equina, which, 
united into two strands upon the 
right and left side, leave a space in 

the middle about 2 to 5 mm. in WrwS^ fi 

width; this space is generally wider 

and better developed in children, in ^il'KR'Mf/ 

whom the cauda equina is also much 
more lax. 







Fig. 131 a. — Cauda Equina of the New-born. 
(Natural size.) 



Fig. 131 b. — Cauda Equina of the Adult. 

(One-half of natural size.) 



LUMBAR PUNCTURE 



239 



The median space is more distinct near the conus at the height of the second 
and third lumbar vertebra than further downward. 

Figs. 131 a and 131 b show these relations in the new-born and the adult 
(but they vary also in individuals). 1 Lumbar puncture is very simple in 
children in whom the large vertebral arch shows wide rhomboid spaces be- 
tween the vertebrae. Here where the spinous 
processes, the ligaments and muscles are but 
slightly developed, we pass directly to the 
median line in the plane of a horizontal 
transverse trunk section, therefore directly 
from before backward, and readily penetrate 
the dural sac to the depth of 1-2 cm. In 
adults the conditions are more complicated, 
since with growth and muscular development 
(besides varying racial and individual peculi- 
arities) the bones also enlarge, and the inter- 
vertebral arches change in width and form ; 
the possibility of reaching them also depends 
upon the shape of the spinous processes; 
these it is true usually extend horizontally ; 
occasionally, however, they incline downward, 
or have at their point a hook-shaped process 
so that the posterior free interarcual space is 
partially covered. 2 If, in the first case, we 
penetrate to the middle line somewhat below 

the lower border of the spinous process with the needle directed horizontally, the 
bone will be encountered, while, in the latter case, the needle must be slightly 
inclined upward toward the head. In muscular persons the ligamenta inter- 
spinalia are frequently so strong and tough that a needle punctures them only 
with great difficulty; in such cases I believe it better to make the puncture 
through the skin 0.5-1 cm. to the right of the median line, and to give to 
the needle such a direction that it enters the dura in the median line. The 
rule for puncture, therefore, is the following : Hold the needle horizontally or 
slightly inclined toward the head and insert it immediately below the spinous 
process in the median line; if the muscles are firm and the ligaments tough, 
the puncture should be made through the skin 0.5-1 cm. to the right of the 
median line, and after inserting the needle deeply we should give it a slight 
inclination toward the median plane — depending upon the presumable thick- 




Fig. 132. — Lumbar Vertebral Col- 
umn of a Child One Year Old. 



i These preparations have been made in the following manner : After removal of 
the brain, the upper part of the cadaver is raised, and inclined forward upon the abdo- 
men ; the sacral canal is then opened and at the apex of the dural sac a 10 per cent, 
lime solution is injected into the subarachnoid spaces of the spinal cord. When the 
body cools and rigidity has set in, the vertebral column from the sacrum up to the mid- 
dle thoracic vertebra is removed and placed in a solution of formalin. After several 
days and when the lime has hardened, the vertebral arches are removed and the dura 
opened. In Fig 131 5 the vertebrae are not illustrated. 

2 These relations may be perceived in Figs. 133 and 134, which have been copied 
after Braun by permission. In a geometric drawing they show the relations of the 
lumbar vertebrae in a lateral and posterior aspect. Fig. 133 is a preparation with a 
horizontal, Fig. 134 with a downward, direction of the spinous processes. 




Fig. 133 a. 



Fig. 1336. 





Fig. 134 a. 



Fig. 134 6. 



240 



LUMBAR PUNCTURE 



241 



ness of the muscles. In following this rule the dural sac will not be pene- 
trated immediately, but the needle will meet the bone or tough periosteum. 
It must then be withdrawn and carefully inserted in another direction. We 
can often feel the needle enter the dural sac, and sometimes the patient is 
conscious of this by the pain he feels. To determine the location of the point 
of the needle, we must notice the depth to which it has entered; if correctly 
inserted, in adults it must penetrate from 4 to 6 cm. before fluid exudes, the 
slighter depth being in delicate, weak persons or women, the greater depth in 
cases the reverse of these. In large, muscular men the depth to which the 
needle penetrates may be from 7 to 8 cm., in a stout person even to 10 cm. 
In the rare cases in which there is cutaneous edema, this must be considered 
and an effort made to remove it by pressure before the puncture. 

It is advisable always to note the depth to which the needle penetrates, so 
that on repeating the operation, this may serve as a guide. 

In regard to the question which interarcual space is to be used for punc- 
ture, there has been much unnecessary discussion. In my investigation of 
the skeletons of 30 adults 
and 12 children, I have 
generally found the first 
and second lumbar inter- 
vertebral spaces to be 
larger than the third or 
fourth. The fifth (also 
known as the hiatus lum- 
bosacralis) is usually lower 
but somewhat broader; 
transversely the interver- 
tebral spaces measure from 
18 to 20 mm., and vertic- 
ally 10 to 15 mm. The 
third intervertebral space 
is generally the best for 
puncture since it is broad, 
is sufficiently distant from 
the conus medullaris, and 
the space between the bi- 
lateral bundles of the 
cauda equina is here suffi- 
ciently large; but the 
fourth and fifth inter- 
vertebral spaces, also in 
adults the second, may be used for puncture. Hence, in the individual case, 
the choice must be made according to the palpable form of the bones, par- 
ticularly of the spinous processes. With repeated punctures it is generally 
better to vary the point of insertion of the needle. 

In the cadavers of eight children I found the end of the conus to be at the height 
of the third lumbar vertebra up to the end of the first year; at the end of the third 
year, at the height of the second lumbar vertebra, and only once, in a child four years 
old, at the height of the fourth lumbar vertebra. 
17 




Fig. 135. — Transverse Section through the Lumbar 
Vertebral, Column, the Puncture Needle in situ. 
(Two-thirds of natural size.) 



242 LUMBAR PUNCTURE 

In thin people the lumbar spinous processes may be easily counted. They 
are broader and may be outlined more easily than the dorsal which some- 
times deviate laterally (although the twelfth dorsal vertebra frequently re- 
sembles those of the lumbar region). It must be remembered that the twelfth 
rib is joined to the upper margin of the twelfth thoracic vertebra. This 
is difficult to find in stout people, in whom also it is difficult to feel the ribs. 
The fourth lumbar spinous process is in these cases a good landmark; it is 
on the same horizontal plane as the upper border of the iliac crest. After 
determining the height of the five spinous processes by palpation I have found 
it of great assistance to outline these by five horizontal lines on the skin 
(compare Fig. 129 a.). 

In estimating the pressure, the behavior of the patient must be considered ; 
sometimes, on account of pain, a change in respiration, or muscular tension, 
the fluid will at first rise, often decidedly (up to 100 mm.) above the true 
average. This should be read off only after the patient becomes perfectly 
quiet. The respiratory variations depend greatly on the type of respiration; 
those due to pulsation, as in the skull itself, are greater with high pressure. 
The fluid must be evacuated slowly and carefully while constantly observing 
all the symptoms. The operator by raising or lowering the glass tube can 
control the rapidity of the flow, and even instantaneously stop it; small quan- 
tities can even be returned to the sac. 

How much is to be withdrawn depends upon the individual case ; and the 
pressure and other symptoms, not the quantity of fluid discharged, form the 
indication for its cessation; this, however, must be read from time to time 
during the outflow; it should not be allowed to decline below the normal, 
therefore it must equal 100 mm. of water ; if the pressure is high at the start, 
we must stop the sooner; if after a little waiting we feel assured that the 
figure first read shows the existing pressure in the cranial cavity, this is cer- 
tainly not true of the subsequent figures, because the intracranial pressure 
accommodates itself slowly to that at the point of puncture, and secretion 
is constantly going on in the ventricles. In fact, when low pressure is finally 
attained by the evacuation, we see that the fluid in the glass tube tends to 
rise more or less rapidly. 

A comparison between the amount evacuated and the change in pressure at 
the beginning of puncture is of special significance considering the previously 
discussed possibility of existing or threatening " automatic " closure of the 
cranial cavity; in this condition there is no addition from the skull to the 
small contents of the spinal dural sac; hence, after a few cubic centimeters 
have been drawn off, the spinal pressure falls several hundred millimeters. 
If the outflow is at first profuse with only a moderate fall in pressure, we 
cannot absolutely exclude such an automatic closure, but it is not very likely. 

The rapidity of the outflow is, of course, increased with the height of the pressure. 
If, prior to attaching the tube, the fluid pours from the cannula in a stream this 
naturally shows high pressure, but never reveals its degree ; the rapidity of outflow 
depends upon other important factors as well as the height of pressure, namely, the 
caliber of the cannula, and the condition of the subarachnoid spaces. 

On attempting to remove the cannula at the conclusion of lumbar punc- 
ture, a palpable resistance is sometimes met with as if it were retained by 



LUMBAR PUNCTURE 243 

tense ligaments. If blood or clear fluid flows from the point 01 puncture this 
should be immediately controlled by compression. The opening should be 
closed by the application of a little iodoform, or, preferably, by collodion 
and a cotton bandage; in uncleanly patients several layers should be em- 
ployed. I have never seen complications from the puncture of the canal. 

When the condition of the patient does not make it impossible, he should 
always remain in bed for at least twenty-four hours after lumbar puncture, 
so that the disturbances of equilibrium in the cerebrospinal cavity may be 
adjusted. 

With an open f ontanelle the tension ceases during puncture ; this indicates 
a fall in the lumbar pressure. 

Otherwise, no noteworthy symptoms appear in the patient during or after 
puncture, the pulse and respiration remaining unchanged. 

Sometimes during the operation, usually a short time afterward, head- 
ache is lessened, the mind becomes clearer; on the other hand, in isolated 
cases headache appears or increases, or general malaise sets in; in this case 
the puncture should at once be interrupted, the patient should be kept quiet, 
and an ice-bag be applied to the head. Only very exceptionally do these symp- 
toms portend danger; as stated, they may be due to automatic closure of the 
cavity of the skull, but usually are attributable to distention of the brain with 
blood on account of the decreased pressure; in twenty-four hours at most 
these symptoms generally disappear. In very rare cases an elevation of tem- 
perature for a day or two follows puncture. 

Difficulties in Lumbar Puncture. — The pain in lumbar puncture is 
usually no greater than in pleural aspiration, since in this condition also it 
varies according to the individual susceptibility of the patient; pain is some- 
what greater if he is restless, or if the space between the bones is not imme- 
diately found; it is sometimes due to the peculiar hyperesthesia which exists 
in many patients with disease of the brain. 

In rare cases they complain of pain or painful tension in one leg during 
the puncture, occasionally the latter condition becomes objectively noticeable; 
usually this disappears at the termination of puncture, at all events in the 
course of the next day; the sequels never persist. In such cases a nerve 
root of the cauda equina has probably been touched or disturbed by the 
needle. This explains the occasional anatomical finding of a fine, thread-like 
coagulum of blood along the nerve root (in cases in which none of the pre- 
viously mentioned irritative phenomena had before appeared). 

If, on withdrawing the mandrin, blood exudes from the needle in drops 
the point of the needle has probably penetrated the anterior or posterior 
venous plexus, and we must try to find the proper position by pushing the 
needle forward or by withdrawing and reinserting it. If no fluid appears, 
the same maneuver is repeated, or rotary or lateral movements are made with 
the needle to free the opening from occluding nerve roots, coagula of blood, 
or pus flocculi. In fact, during the entire operation of puncture, it is advis- 
able to keep the needle in the position which is most favorable for the exuda- 
tion of the fluid. If we believe that we are in the dural sac yet no fluid 
appears, we should not be afraid to draw off a few drops of fluid with a 
syringe; if the opening is occluded merely by pus or blood coagula, this 
maneuver will clear the outlet ; if not, the drop which has been aspirated may 



244 LUMBAR PUNCTURE 

give us points in the diagnosis. To aspirate more than a few drops is nnwise 
since there is danger if the pressure is low; and if nerve roots occlude the 
opening or the stoppage is due to the gelatinous composition of the exudate 
aspiration is useless. Thick fluid pus is not found in the dural sac. There- 
fore, when for these reasons or because the opening between the bones cannot 
be found, there is no result from puncture, we should withdraw the needle 
at once, and on the following day we may try to puncture another interverte- 
bral space. 

(12) Lumbar Puncture is Performed in the Following Conditions: 

(a) When there is brain pressure with threatening symptoms which render 
the decrease of this pressure imperative; 

(b) When there is moderate, but long-continued brain pressure which we 
may hope to relieve by puncture; 

(c) When an increase in pressure is assumed or suspected, and a gauge 
of this pressure is desirable; 

(d) In cases in which, aside from the question of brain pressure, we wish 
to examine the fluid, or to determine the existence of meningitis, or to decide 
its nature; 

(e) In cases in which the fluid is diagnostically examined for possible 
admixtures (blood, toxins, agglutinins, etc.) ; 

(/) When we wish to inject fluids into the spinal canal for therapeutic 
purposes. 

Like other punctures, for example, in the pleural cavity, lumbar puncture 
is resorted to both for diagnostic and therapeutic purposes. In this descrip- 
tion, just as in practice and in the concrete case, these two indications cannot 
be distinctly separated from each other — less so in puncture of the spinal canal 
than in puncture of the pleura. As the symptoms, especially the objective 
signs of meningitis, are by no means so positive as the signs of exudative 
pleurisy, puncture of the spinal canal is frequently practised as an aid to 
diagnosis; its results, however, are more important, because the pressure of 
the exudate may at this point be much increased and is of greater signifi- 
cance. Spinal puncture is less serious than pleural because, in passing through 
the serosa, even under normal conditions, the needle encounters fluid, while 
in the pleura, if there is no exudate, the lungs or other organ may be injured. 

In the diagnostic puncture of the pleura the needle inserted is usually 
withdrawn and replaced by a larger one for the evacuation, but in spinal 
puncture the same needle serves for both purposes. 

(13) Lumbar Puncture in Various Diseases. — (a) Serous effusions are 
most readily influenced by puncture. The cerebrospinal effusions termed 
hydrocephalus are of less significance, even if of the same volume, for the 
pressure which the brain suffers, not the amount of fluid, is the important 
point. 

This is most evident in the hydrocephalus of children ; here we have cases 
with only a slightly enlarged cranium yet severe brain symptoms, and, on 
the other hand, enormous heads with but slight disturbance in function. If 
the sutures of the skull are still open the first group will show decided, and 
the second group but slight, tension of the fontanelles. But the condition 
may change; in the first group the brain symptoms may lessen (although the 
skull may perhaps increase in size) if the tension of the fontanelles is de- 



LUMBAR PUNCTURE 



245 



In order to lose none of the data obtained by lumbar puncture, we should note the 
findings on a printed diagram (such as is here depicted). This enables us to compare very 
readily the results of several punctures in the same patient. 



Name 




Disease (Duratic 


m, Symptoms) 


Age 


Sex 




Admission to Hospital 


LUMBAR 


PUNCTURE 




Date ' 








Place 














Degree 
















Ed 

CO J 

co "S 
fH 

03 


At the begininng 
of puncture 








At the end of 
puncture 










Variations 








.. 


a 
< 

Q 

5 


r 

Amount 










Appearance 








Specific gravity 








Albumin per 1,000. . 








Sugar 










Cells 










Microorganisms 








Remarks: 

S . ... . 







246 LUMBAR PUNCTURE 

creased, while in the second group symptoms of cerebral irritation and pressure 
may sometimes persist for weeks or longer if the tension is increased. 

" Chronic hydrocephalus " of children often begins acutely with meningeal 
symptoms, and not rarely may subsequently show acute or subacute exacerba- 
tions, both anatomic as well as symptomatic. With the closed skull these 
processes are not marked by increase of tension and circumference, but only 
by functional disturbances, which, however, are readily produced because 
with the same volume the exudate exerts a much stronger pressure. The 
volume of the effusion and the symptoms more clearly explain the varying 
picture of hydrocephalus (except hydrocephalus ex vacuo) if we regard the 
underlying process as an inflammatory increase in exudation. The clinical 
picture varies because the affection sometimes appears acutely, sometimes 
insidiously, and then again after long intervals in exacerbations; also because 
with an open skull this is difficult to demonstrate by palpation with the hands, 
and in the closed skull without lumbar puncture; to this we may add that 
unlike the condition in other serous cavities, after removal of the inflamma- 
tory exudate the former anatomical relations are restored; but the rigidity of 
the walls of the skull and deficient elasticity of the brain often cause the ven- 
tricles to be distended with fluid although the secretion is only of normal 
amount. 

In addition to acute serous forms of meningitis we meet the symptom- 
picture of acute and transitory exudation which I have designated as angio- 
neurotic hydrocephalus. Other pathologic processes — neoplasms, parasites, 
effusions of blood — may cause increased transudation and subsequently hydro- 
cephalus. 

As in other serous cavities the restoration of the equilibrium by the out- 
flow of the secretion is of decisive importance; if the outlets become too nar- 
row or too large, the absolute quantity of the fluid increases and the pressure 
rises; the latter circumstance often at first increases the outflow, the tracts 
widen, and compensation is brought about; but a certain increased internal 
pressure may also occlude the outlets, most readily in the cavity of the skull, 
where distention of the ventricles presses the surface of the brain against the 
bone, and the subarachnoid and subdural spaces, also the channels to the 
Pacchionian granulations, are narrowed. These are the conditions in which 
spinal puncture beneficially decreases the pressure; it clears the outlet of 
obstacles and thus breaks up a vicious circle. Whether this benefit is perma- 
nent, depends upon other factors. 

When the secretion continues to be profuse because of the return of the 
old condition, repeated punctures are necessary, and these may possibly retard 
the damage to the brain from pressure until the disease is on the decline. 
Spinal puncture, therefore, may be beneficial, especially in acute and subacute 
serous meningitis and in the acute exacerbations of chronic cases, but this 
will be only transitory or very slight when the exudation, although perhaps 
not great, is uniformly persistent ; success, therefore, depends upon the nature 
and study of the pathologic process — just as in that of the pleural cavity and 
of the joints. In these regions puncture is merely a remedy which, com- 
bined with other treatment, we use in certain stages and for certain indica- 
tions. On account of the importance of the organ, the danger of an increase 
in pressure in the cerebrospinal cavity is greater than anywhere else. At the 



LUMBAR PUNCTURE 247 

present time, we can often determine the cause of an existing pressure in the 
brain, but we can only guess at the nature and stage of the exudative process ; 
hence, the conditions being to a great extent unknown and varying, the bene- 
fits from lumbar puncture in serous effusion also vary; in adults as in chil- 
dren the results of one puncture have been even more surprising than in 
pleural aspiration, and have suddenly changed the course of the disease. This 
is particularly evident in the acute, inflammatory, and angio-neurotic forms 
where it is only necessary to free the outlets as soon as the exudation has 
reached a high mark. 

The cases in which the initial improvement did not continue, and in which 
repeated punctures were necessary, are more numerous. Here the exudation 
decreased only after the lapse of weeks or months, either " spontaneously/' 
under other treatment, or because puncture produced a different condition, 
perhaps because irritating products in the fluid had been removed. In such 
cases many punctures (10 to 25) have been made at intervals of days 
(Grober). 

When the secretion is continuous and profuse, puncture is of little benefit 
or only transiently. It is useless also in those cases of chronic hydrocephalus 
in which we must assume that larger or smaller areas of the brain have been 
changed or atrophied by long continued pressure. This statement must be 
modified in regard to the moderate hydrocephalus of small children, in which, 
even under such circumstances, repeated punctures are sometimes followed 
by a marked improvement in mental and other nervous functions, probably 
because the diminution in pressure permits a greater development of the brain. 

As the development and the stages of serous effusion differ, so the result 
also varies. 

In addition to other data, the puncture throws" light upon certain condi- 
tions: High pressure denotes a still existing hypersecretion ; the removal of 
large quantities of fluid indicates a decided dilatation of the cerebrospinal 
cavity, therefore probably a somewhat longer existence of the hydrocephalus; 
an amount of albumin below 0.5 per thousand generally denotes a favorable 
prognosis. 

It may also be stated that an absolutely clear fluid may contain a small 
number of morphotic elements which can be recognized only upon sedimenta- 
tion, centrif ligation, or coagulation. The microscopic examination for cells 
and microbes should never be omitted; serous exudates may show a series of 
conditions from that truly and morphotically free through all grades of tur- 
bidity up to a purulent one. 

Pneumococci and streptococci, the bacilli of typhoid, influenza and tuber- 
cle, and coli bacilli have been found in the serous fluid, as well as in other 
serous exudates, i. e., those poor in cells, as in the pleural cavity. 

(b) Purulent Meningitis. — In purulent meningitis puncture is rarely 
followed by the evacuation of thick pus, but of a cerebrospinal fluid with 
more or less purulent admixture, and this varies from slight turbidity to a 
distinctly yellow and purulent discoloration. On standing, the deposit of pus 
is slight and only exceptionally is it coagulated by excreted fibrin; macro- 
scopically (and this is diagnostically important) the fluid may sometimes be 
perfectly clear, while, further upward, the meshes of the pia are filled with 
purulent coagula. 



248 LUMBAR PUNCTURE 

Microscopically the cells of fresh purulent exudates contain leukocytes 
with divided nuclei, while in chronic exudates, in which the fluid is usually 
less turbid, lymphocytes also are found; in very late stages of protracted 
purulent or tuberculous meningitis these cells may be found alone. 

The microbes most frequently noted are meningococci, pneumococci, and 
streptococci, as well as tubercle bacilli; in stained preparations the meningo- 
coccus and pneumococcus, although recognizable, are sometimes attenuated, 
and no longer grow in the culture. If they are absent from the fluid, particu- 
larly the meningococcus intracellularis, they may be found in the adherent 
purulent exudate. 

But there are also actually sterile purulent inflammations of the membranes 
of the brain (in syphilis) in the exudate of which no microbes can be demon- 
strated, in which, therefore, a dissolved virus from the blood or from the 
cerebrospinal fluid has perhaps produced the inflammation. 

Purulent meningitis is always a dangerous disease, and the results of 
therapy are necessarily less satisfactory than in the serous form. Since the 
malady is a purulent inflammation of the cell tissue, we can expect but little 
from puncture, even from repeated punctures; nevertheless these sometimes 
produce most certain results which of course differ according to the genesis 
of the disease. The greatest benefit appears to follow puncture in the forms 
due to meningococci, next those due to pneumococci, and after one puncture, 
especially in the former, we occasionally observe improvement both in regard 
to the fever and the cerebral symptoms. In other cases several punctures 
are necessary (in a case of Netter's, eleven) ; in such a series of punctures 
the turbidity of the fluid gradually decreases. In this form of meningitis 
success probably depends upon the fact that the virility of the meningococcus 
suffers, the disease is thus checked, and it is then only necessary to form an 
outlet for the escape of the toxins with the dead bacilli. 

When thick, fluid, flocculent pus is found, puncture is followed by irriga- 
tion with a sterile normal salt solution (Jakob, Kronig; see page 42). 

Even when therapeutic success cannot be expected from puncture, either 
because the symptoms are too mild or too severe, by determining the nature 
of the meningitis this operation may give us indications for the diagnosis as 
well as for treatment. It has often been resorted to in diseases of the train 
in order to decide whether operation on the skull was justifiable or not. After 
injury to the skull, in brain abscess, and in otitis media, cerebral pressure 
symptoms may be due to purulent meningitis and may be either circumscribed 
or diffuse, but they may also be due to serous meningitis and of varying 
importance in the prognosis and treatment. Here lumbar puncture clears the 
situation. If it shows serous fluid, operation upon the skull is likely to be 
successful, although it by no means excludes circumscribed purulent inflam- 
mation. 

Surgeons as well as otologists have practised lumbar punture on these principles. 
Braunstein (Schwartze) excludes purulent cerebral meningitis only when a clear fluid 
flows off so profusely that we may suppose it to originate from the cranial cavity. 
Aside from the fact that this is not an infallible guide, such a profuse evacuation 
appears to me not devoid of danger, because it may mobilize a circumscribed pus focus 
and diffuse the inflammation. Nor does an operation upon the skull appear to me to 
be absolutely contraindicated because purulent fluid is obtained by puncture; certainly 
there is less hope of success, but other circumstances also must be taken into considera- 



LUMBAR PUNCTURE 249 

tion. It is quite conceivable that a purulent meningitis limited to the cerebellum and 
spinal cord might heal if the abscess in the cerebellum, or suppuration of the bone 
from which it originated, were given an outlet. 

(c) Tuberculous Meningitis. — Although tuberculous meningitis is really 
included in the previous description, its frequency and its clinical picture 
merit special consideration. As a rule, the fluid obtained by puncture is only 
slightly turbid. It may be perfectly clear with small floating flocculi. On 
standing it forms fibrin coagula, and lymphocytes are microscopically present 
(either with pus corpuscles or alone). At the stage of onset the fluid may be 
perfectly clear, in rare cases it is markedly purulent (for example, in mixed 
infections with meningococci or pneumococci). 

The amount of albumin is usually greater than normal, and varies be- 
tween 0.5-2 per thousand. The fluid, therefore, according to the individual 
case and stage, may show all the transitional stages between a purely serous 
and a purulent meningitis. Lumbar pressure, corresponding with the dis- 
tinct pressure symptoms, is usually high, over 500 mm. water. 

Pfaundler once observed it to be 1,500 mm. (=110 mm. Hg) ; he found in his 
cases in the stage of irritation an average of from 650 mm. to 700 mm. of pressure, 
and in the stage of paralysis 320 mm. pressure. The " pressure curve " resulting from 
this corresponds also with the other pressure symptoms. It can scarcely be called 
typical, but varies greatly in the individual case. 

The finding of tubercle bacilli in the fluid is noteworthy and often an 
important aid to the diagnosis; these increase in the terminal stages of the 
disease, and are invariably found in the fluid post mortem. Some investi- 
gators found them in more than 75 per cent, of the cases. The greater fluidity 
of, and sparsity of cells in, the liquor compared with other serous exudates 
may favor their dispersion; and the shape of the dural sac promotes their 
sedimentation in the lumbar portion. 

In addition to other methods, tubercle bacilli may be demonstrated by inoculating 
guinea-pigs either subcutaneously, intra-peritoneally, or intra-durally ; Martin i pro- 
duced tuberculous meningitis by occipital injection, Hellendahl induced by spinal injec- 
tion not this disease but general miliary tuberculosis, and more readily than by any 
other means. 

Langer used the fluid itself for culture; 4 to 5 cm. were dropped into sterilized 
culture tubes and placed in the incubation oven; after five or six days the sediment 
was profuse, and after several weeks the culture showed more tubercle bacilli, granule 
and nodule forms. Langer was thus enabled to demonstrate bacilli when none could 
be found in the fresh specimen. 

In some cases of tuberculous meningitis this demonstration of tubercle bacilli may 
be of great significance ; I will merely mention that Freyhan, Henkel and Gross proved 
that the disease does not always terminate fatally. 

Although only in the rarest cases does recovery from tuberculous menin- 
gitis follow lumbar puncture, there is often a transitory improvement, con- 
sciousness returns at least for a time, and restlessness, moaning and spasms 
cease; repeated punctures bring about the same amelioration. 

(d) Tumoes. — Brain tumors at first press upon the surrounding areas, 
subsequently by displacing the brain substance they flatten the subarachnoid 

i Soc. de biologie, 5. Mars, 1898. 
18 



250 LUMBAR PUNCTURE 

spaces; this impedes the passage of the cerebral spinal fluid toward its out- 
lets, and lymph stasis may produce hydrocephalus. The secretion of the 
venous plexuses may be increased by the influence of the tumor; this may 
also be caused by venous hyperemia from pressure upon Galen's veins. Besides 
the lessening of space from the existence of the tumor, other factors may 
increase the pressure. 

These complicating conditions vary according to the size, the seat, and 
the nature of the tumor ; hence lumbar puncture in brain tumor reveals vary- 
ing heights of pressure. 

The evacuated fluid is usually clear and without cellular admixture; the 
amount of albumin varies from 0.5-8.0 per thousand. 

Lumbar puncture can be therapeutically successful only in those cases in 
which hydrocephalus is marked besides producing pressure on the brain. 
It is obvious that this result can be only palliative, as in the case of neoplasms 
of the pleura. 

I have stated that in brain tumor an automatic closure of the cranial 
cavity readily occurs, and for this reason puncture must be most carefully 
performed. This is particularly true when the seat of the tumor is presum- 
ably the cerebellum. Here the rapid decline of lumbar pressure after the 
removal of but a small quantity of fluid is an aid to the diagnosis. 

(e) Abscesses. — Abscesses like tumors act mechanically upon the con- 
tents of the skull. Here lumbar puncture aids but little in the diagnosis 
except in determining the existing cerebral pressure. In abscess it may help 
us to decide the question whether rupture has taken place and produced men- 
ingitis. 

In cases of abscess, lumbar puncture is as little beneficial as in tumor; 
at most it may temporarily lessen the pressure on the brain, which is due to 
an accompanying ventricular effusion. 

That free outlet may favor a threatening rupture of the abscess is obvious, 
but no case of this has as yet been reported. 

(/) Varying Conditions. — Lumbar puncture proves that the cerebro- 
spinal pressure in sinus thrombosis (Braunstein) and compression of the 
superior vena cava (up to 420 mm., Noelke) may be increased by stasis transu- 
dation into the subarachnoid spaces and the ventricles. This may also be 
presumed, although to a less extent, in valvular lesions with general venous 
stasis. 

The same circumstance is the chief cause of the temporary but marked 
increases of pressure in the spasmodic conditions of epilepsy and hysteria, in 
the spasms of paralytics, and also in attacks of mere screaming with pressure 
amounting to from 300 to 800 mm. (Navratzki and Arndt, Stadelmann, 
Quincke). Except during the attacks, pressure has been found normal in 
epileptics. In two epileptics who had severe attacks, Chipault saw improve- 
ment after several punctures. 

Vidal and Le Sourd found lymphocytes in the spinal fluid in progressive 
paralysis; probably these were the expression of subacute inflammation. 

The serous transudations in uremia, lead colic, and in some infectious dis- 
eases are of somewhat doubtful pathogenesis. 

In uremia the spinal pressure is sometimes unchanged and, even when 
there are no convulsions, it is sometimes increased. In these cases, as in 



LUMBAR PUNCTURE 251 

arachnoid and cerebral edema, there is increased transudation, and to this 
as well as to toxic influences do we attribute the varying symptomatology of 
uremia. After spinal puncture there is sometimes a decrease in the cerebral 
symptoms. In a case of lead colic, and during the coma following eclampsia, 
Segelken and Brasch saw the pressure amount to 310 mm. and after 
evacuating 50 c.c. of fluid, partly clear and partly turbid, there was im- 
provement. 

In some infectious diseases, such as enteric fever, scarlatina, pneumonia, 
a moderate increase of pressure has occasionally been found on lumbar punc- 
ture (Stadelmann, Salomon and others), no true meningeal symptoms being 
present. As the diseases in question are sometimes intercurrent with microbic 
meningitis, it is possible that there may have been a very mild grade of this 
affection, or perhaps only a toxic irritation of the meninges. The same con- 
ditions were found in influenza and in acute leukemia (Stadelmann), perhaps 
also in plague, for marked meningeal edema has been described in the cadavers 
from this disease. 

In chlorosis with severe headache and meningeal symptoms lumbar punc- 
ture frequently shows an increase of spinal pressure, and the symptoms are 
often favorably influenced by puncture. Although sinus thrombosis may in 
some cases be the cause of the condition (Lenhartz), yet, on account of the 
brief duration of most of the symptoms, I am inclined to assume an angio- 
neurotic increase in secretion. Moreover, lumbar puncture sometimes pro- 
duces the same results and is just as beneficial in the severe migraine of those 
who are not chlorotic. 

The meningeal affection of syphilis is most frequently gummatous or in- 
durative and circumscribed; when localized in the spinal cord the latter form 
may produce such a condition that a normal discharge of fluid does not follow 
spinal puncture. In the secondary as well as the tertiary stage of syphilis 
we have the picture of serous meningitis; here, under high pressure, a clear 
or slightly turbid fluid flows profusely, and improvement follows (Quincke; 
Chipault, a typical case). I have no knowledge of the anatomical cause of 
these forms. 

(g) Hemorrhage. — Copious effusions of blood into the cranial cavity 
are produced by spontaneous cerebral hemorrhage, by aneurysms from the 
large basal cerebral arteries, or by trauma; the latter are usually meningeal. 
Traumatic hemorrhages in the skull as well as in the spinal canal often, but 
not necessarily, accompany fractures. Hemorrhages into the cranial cavity 
may increase not only the intracranial, but also the spinal, pressure; as in 
tumors the disturbance is caused primarily by an impediment to the out- 
flow of lymph, but often it may be due to an inflammatory increase in secre- 
tion. 

If there has been a rupture into the ventricles or the subarachnoid spaces 
blood alone is mixed with the fluid from puncture ; but the blood may coagu- 
late further up and the spinal fluid remain clear. The blood usually coagu- 
lates when it is effused into the almost empty subdural space. Partly for 
this reason and partly because the physiologic current sets in the opposite 
direction, blood effused subdurally does not, as a rule, reach the subarachnoid 
spaces and subsequently the fluid obtained by puncture; the conditions are 
different if the arachnoid has been injured. When the seat of the hemor- 



252 LUMBAR PUNCTURE 

rhage is extradural we may still less expect to find blood in the aspirated fluid, 
but by diffusion hemoglobin may transude and give to it a yellow color. 

In obscure cases, after accidents, and in unconscious patients who can 
give no history, lumbar puncture from positive as well as from negative 
findings may be valuable in diagnosis. But we must be cautious, for if the 
hemorrhage is recent a marked decrease in pressure may increase it or start 
it anew, especially in the skull. After a few days this danger is lessened, 
and if some of the blood is evacuated, perhaps simultaneously the transudate 
also, the remainder may possibly be more rapidly absorbed. After traumatic 
hemorrhage between the membranes of the cord, puncture and evacuation of 
the effused blood has frequently had a very beneficial effect (Kiliani, Jacoby, 
quoted by Braun, Bugge). 

(h) Subdural Effusions. — Between the dura and arachnoid, just as may 
occasionally happen between the layers of the pleura or of the peritoneum, pus 
forms in a thin layer; for example, in bone suppuration which may extend 
from the ear to the dura, the pus may flow toward the spinal sac and sepa- 
rate the arachnoid from the dura; the subarachnoid spaces are then com- 
pressed and the arachnoid is forced against the pia. In lumbar puncture, 
after passing through the dura, we reach a subdural space which normally 
does not exist. 

In a case of this kind I found 500 mm. of pressure and evacuated 20 c.c. of foul, 
creamy pus. At the autopsy the pia-arachnoid of the spinal cord was merely hyperemic, 
and showed no purulent infiltration; the surface of the cerebellum was covered by pus 
which could be washed away, and the subarachnoid tissue in the area of the pons and 
the chiasm was already pus stained. 

Serous effusions into the subdural space do not appear to occur ; a secretion 
into the ventricles and subarachnoid spaces seems to predominate, normally 
as well as pathologically; for this reason the arachnoid becomes tense and 
separates from the pia; in the spinal canal hydrostatic pressure is added to 
this, and the arachnoid is closely pressed against the dura. 

(14) In conclusion, in puncture of the spinal sac the diagnostic and 
therapeutic conditions are analogous to those of puncture of the pleura, 
of the joints, and of other serous cavities. The fluid in these cavities is not 
a fixed and constant amount, but an ever varying one and ever forming; 
it cannot be compared with the contents of a bottle which may be emptied, 
but to a natural reservoir with an inlet and outlet ; by pumping the level may 
be reduced for a short time, but this will be permanent only when the afflux 
is decreased or the outlets are enlarged. So the immediate results of a punc- 
ture will be permanent only when the conditions which have promoted the 
accumulation of fluid are changed, either by evacuation, by the natural course 
of the disease, or by other means ; this is also true of lumbar puncture. The 
peculiarity and vital importance of the enclosed organ, the narrowness of the 
cavity, and its distended shape are important. The first two factors give to 
each of its diseases greater significance; the third enables us to differentiate 
from each other the distant cranial cavity and the lumbar region, as well as 
to attempt therapeutic procedures. 

(15) Slitting the Dura. — When, after the puncture of serous effusions, 
the cerebrospinal pressure persistently rises, it is often advisable to evacuate 



LUMBAR PUNCTURE 253 

the fluid continuously for some time. This has been done in hydrocephalus 
by inserting a drain of catgut between the lateral ventricle and the subdural 
space, or a gold cannula between the ventricle and the subcutaneous tissue. In 
the lumbar region the needle (Lenhartz) or cannula (Sahli) is permitted to 
remain for some time, but on account of the danger of displacement and infec- 
tion this cannot be permitted for a long period. I have, therefore, tried to 
dissipate the fluid into other tissues and fluid tracts by slitting the dura; 
this is accomplished by a long-bladed lancet, the tip having a breadth of 3 to 
6 mm., which is held in the median position and introduced exactly like a 
needle; the passage through the dura is distinctly felt, and the depth of 
the longitudinal section may be gauged by the further introduction. The 
incision may be enlarged by a lever movement. Sometimes fluid exudes from 
the wound and along the handle of the knife, but shortly after the removal 
of the knife this flow ceases, the wound is packed with iodoform gauze, and 
collodion and a bandage are applied. The cerebrospinal fluid now flows into 
the canal which has been artificially made and into the tissue spaces; this 
is sometimes recognizable from an edematous swelling of the subcutaneous 
cellular tissue and of the deeper soft parts in the lumbar region. This swell- 
ing may continue for one, two, or even eight days ; but even when it is invis- 
ible the drainage may continue, especially into the loose, 
peridural cellular tissue. 



o 



In this process there must necessarily be some injury 
to the veins and the peridural plexus; but, under the 
pressure on the exuding fluid, hemorrhage soon ceases; I 
have never seen serious consequences from this, nor from 
an injury to the bundles of the cauda equina. When 
a necropsy was subsequently performed, the slit was 
found in the desired area or very near it in the median 
line, and always shorter than I expected (1 to 3 mm.) 
probably because of the elasticity of the dura; hence 
there appear to be neither danger nor sequela?; as a rule, 
the slit closes more rapidly than is desirable, probably 
because of a slight displacement of the arachnoid. Lately 
I have several times attempted to produce a transverse 
slit by withdrawing the knife from between the arches, 
turning it at an angle of 90°. and then reinserting it. FlG i 36 ._one-haef 
But with this method it is probably wiser to make the the Natural Size. 
transverse slit at first. 

Slitting should be performed only after a preceding puncture when we are 
familiar with the conditions, and also have some idea of the depth to which 
we wish to penetrate. It may happen that the narrow, intervertebral space 
will admit the needle but not the lancet; then the adjoining space will be 
chosen. While we should puncture and gauge the pressure, as a rule, imme- 
diately before slitting, it is not wise to diminish this by an outlet because 
the tense dura permits the knife to enter more freely. To facilitate the 
insertion of the knife, I have had a furrow made in the side of the lancet, 
and the pedicle bent like a bayonet, so that the knife may be inserted along- 
side the needle which is already in the dural sac ; but this is no great improve- 
ment, and it necessitates that the blade of the lancet be somewhat thicker. 



254 LUMBAR PUNCTURE 

I have performed slitting about twenty times, and my opinion is that it 
should be further tested; it is beneficial in chronic and subacute cases of 
serous exudation when the pressure is permanently increased; I have not 
refrained from employing it in tuberculous meningitis, because the bacilli 
which are evacuated are manifestly less dangerous outside than within the 
spinal canal. 

(16) Lumbar Puncture has also been employed therapeutically, namely, 
to introduce fluid into the spinal sac. Jacob and Kronig, 1 after performing 
puncture in purulent meningitis, injected a physiologic salt solution which 
diluted the pus, and then evacuated it. The much smaller dimensions of the 
subarachnoid space naturally make this less suitable for such irrigations than 
the cavity of a joint or serous sac, and the benefit from this procedure will 
of course be limited to merely a few segments of the spinal cord; successive 
trials will show whether by this means we cannot occasionally obtain results. 
From 5 to 25 c.c. of fluid were repeatedly injected, and allowed to flow out. 

Drugs, too, have been injected into the spinal canal on account of their 
direct action on the spinal cord and nerves. 

Injections of cocain, first made by Bier, show to-day the best results and 
the most manifold application of this process. Within four to eight minutes 
after 0.01-0.02 of cocain has been injected into the subarachnoid sac, there 
is decreased sensation (for tactile and temperature impressions) and complete 
analgesia in the lower half of the body from the navel down, so that operations 
may be performed upon the lower extremities and pelvis without the patient 
feeling pain. 

The analgesia lasts from an hour to an hour and a half; motility is com- 
pletely retained but the muscles are flaccid (in 5 per cent, of the cases flatus 
and feces were involuntarily passed, but never urine). In the majority of 
cases (80 per cent., Tuffler), there are sequels: Headache, general discomfort, 
paresthesia of the legs, difficulty in respiration, a sense of heat, sweating, 
nausea or vomiting (in 40 per cent.). After a few hours there is sometimes 
a brief rise in temperature (up to 102.2° F.). 

At the expiration of an hour cocain can no longer be demonstrated in the 
cerebrospinal fluid (Tuffler). 

There is some uncertainty in regard to the degree of analgesia and its 
relation to the dose, as well as concerning the appearance and duration of the 
secondary effects. 

Bier injected 0.01-0.02 of cocain in a one or two per cent, solution; lately he has 
preferred to inject 5 c.c. of a corresponding dilute solution after having previously 
evacuated the same amount of fluid (5 c.c). Jacob considers the most suitable men- 
struum a 0.1 per cent, salt solution. Guinard regards the headache which often appears 
after Bier's process as a slight aseptic meningitis, since lumbar puncture diminishes 
the pain, and the lymphocytes discharged in the fluid are in proportion to the intensity 
of the pain. Guinard succeeded in averting the headache by adding cocain to the cere- 
brospinal fluid previously obtained by puncture, and then reinjecting it. This is very 
easily done; instead of using a glass tube for lumbar puncture a small burette with a 
capacity of 5 to 10 c.c. may be employed by attaching it to a rubber tube (see Fig. 
129 & ) ; after about 5 c.c. of the cerebrospinal fluid have drained into this, we add 
the cocain with a graduated syringe or pipette, and then by raising the burette the 
fluid passes back into the spinal sac. Beta-eucain (0.02-0.06) or tropa-cocain (0.05- 

i Berl. klin. Wochenschr., 1902, Nr. 8, p. 174. 



LUMBAR PUNCTURE 255 

0.06) have been employed instead of cocain. Of course, the solutions must be sterilized 
by boiling. 

Besides in operations upon the lower extremities and the pelvis, subarach- 
noid injections of cocain have been used to diminish pain in sciatica, in herpes 
zoster, in epididymitis and in gastric crises, also other pain eccentrically pro- 
jected as in priapism due to myelitis. They had no efficacy in lead colic. 

The question as to where the effects of cocain are produced brings into 
consideration the posterior roots and the sensory ganglion cells of the spinal 
cord; probably the latter are chiefly affected. Contrary to the usual course 
of the current, the cocain must reach them by way of the lymph tracts. With 
MssPs stain, Carini found changes in their chromatin and cell nuclei 8 to 24 
hours after the injection. 

Xeugebauer investigated very minutely the time and place in which anal- 
gesia developed; as a rule, he found the first sensory area to be in the fourth 
sacral segment (perineum, etc.), thence gradually rising to the third, second 
and first sacral segments; above this the trace was lost. In 50 per cent, of 
the cases a certain diminution in the sensation of pain was perceptible also 
in the upper extremities and the head, including the mucous membrane. 

The differences in the action and the sequels of the same dose may be 
attributed either to the fact that the cocain solution in a relatively concen- 
trated form acts only upon the lower portion of the spinal cord, or that higher 
up it is more admixed with the cerebrospinal fluid. 

Tetanus antitoxin, as well as cocain, has frequently been injected into the 
subarachnoid space, apparently with success. 1 Here the active substance is 
injected directly into the diseased spinal cord; after a few hours there is a 
decided rise in temperature. 

The marked effect of this direct introduction appears to depend not only 
upon the short distance to be traversed but also upon the fact that some sub- 
stances very slowly reach the nerves by way of the blood and through the walls 
of the vessels; for instance, Lewandowsky observed that strychnin introduced 
into the subarachnoid of animals was effective in one-tenth of the subcutaneous 
dose, and that sodium ferrocyanid in doses of but a few centigrams pro- 
duced motor irritative symptoms which could not be attained through the 
circulation by doses one hundred times as great. 

Sicard made similar observations concerning morphin. 

In regard to further absorption by way of the subarachnoid spaces, reports are 
contradictory. While E. and A. Cavazzani, after a few attempts, declared the absorp- 
tion of combinations of iodin and ferrocyanid to be slow, Lewandowsky found the 
absorption of ferrocyanid to be but little slower than through the subcutaneous con- 
nective tissue, the substance appearing in the urine after the expiration of 15 to 35 
minutes. After the injection into dogs of one gram of KI in a 4 per cent, solution, 
Jacob found the iodin in the urine six hours later; fulminant symptoms, * however, 
showed that the meninges were markedly irritated by the salt, for even a 0.7 per cent, 
salt solution has an irritating effect both in dogs and man, while a 0.1 per cent, solu- 
tion does not irritate. Of iodin (sodium iodid?, Quincke) a solution of 0.04 per cent. 
is most suitable. In three cases of cerebrospinal syphilis Jacob injected 25 c.c. of this 
solution (therefore 0.01 of the salt) once or twice into the cerebrospinal sac, and 
believes that the results were good. 

i Sicard, Schultze, Leyden, Blumenthal and others. See also volume on " Infectious 
Diseases," pp. 842 fit seq. 



256 LUMBAR PUNCTURE 



Literature 

R. Bandel (Merkel, Niirnberg), "Die therapeutische Wirksamkeit d. L. P." 
Mitth. aus. d. allg. stddt. Krankenhause zu Niirnberg, 1902. — Bendix, "Die Cytodiag- 
nose d. Meningitis." D. med. Woch., 1901, Nr. 43. — G. Bonninghaus, "Die Menin- 
gitis serosa acuta." Wiesbaden, 1897. — Brasch (Goldscheider), "Erfolge d. L. P. bei 
Hydrocephalus chron. d. Erwachsenen und bei Men. serosa." Zeitschr. /. klin. Med., 
Bd. 36, H. 1. — H. Braun, "Ueber d. L. P. u. ihre Bedeutung fiir die Chirurgic" 
Arch. /. klin. Chir., 1897, Bd. 54. — A. u. E. Cavazzani, "Ueber Cerebrospinalflussig- 
keit." Centralbl. /. Physiol, 1892, Bd. VI, Nr. 14 u. 18.— A. Chipault, "Sur la 
ponction lombaire simple." La Medecine moderne, 25. Dec, 1901. — Deniges et 
Sabrazes, "Valeur diagnostic de la ponction lombaire." 777. Congres franc, de med. 
int. La semaine med., 1896. — M. Dircksen, "Etude sur la composition chimique 
et la concentration moleculaire du liq. cephalo-rachidien." These, Paris, 1901. — 
Fleischmann (Lichtheim), "Die Ergebnisse d. Lumbalpunction." D. Zeitschr. /. 
Nervenheilk., Bd. 10, H. 5-6. — Grober, "Ueber d. Wirksamkeit der Spinalpunction, 
etc., bei chron. Hydrocephalus." Milnchener med. Wochenschr., 1900, Nr. 8. — Gum- 
precht, "Gefahren der Lumbalpunction, plotzl. Todesfalle danach." Deutsche med. 
Wochenschr., 1900, Nr. 24. 15 Falle zus. gestellt (2 eigene). — Henneberg, "Ueber 
Spinalpunction bei Hirnblutungen." Sitzung d. Bed. psychiatr. Ver., v. 16, Juni, 
1900. Aerztl. Sachverstandigen-Ztg., v. 1, Oct., 1900. — Hitzig, "Ueber den Ort der 
extraventricularen Cerebralflussigkeit," Reichert u. DuBois-Reymond's Arch., 1874, 
p. 263. — P. Jacob, "Ueber Duralinfusion." Bed. klin. Wochenschr., 1898, Nr. 21, 
1900. Nr. 3-4. — Th. Kocher, "Hirnerschutterung," Hirndruck, etc., in NothnageVs 
Handb. d. spec. Path. u. Ther., Bd. IX, 3, 2, 1901. — O. Kohts, "Meningitis d. Kinder 
u. Hydrocephalus." Deutsche Klinik, 1902, Bd. VII, Vorles. 8, p. 150.— Kronig, 
D. med. Wochenschr., Vereinsbeil., 1897, p. 229. — Lewandowsky, "Zur Lehre v. d. 
Cerebrospinalflussigkeit." Zeitschr. f. klin. Med., Bd. 40, 1900, p. 480. — Langer, 
Zeitschr. f. Heilk., 1899, XX, 327. — Levi ("Serose Entziind. bei Pneumo- u. Strep- 
tococcus, Typhus-, Influenza- und Colibacillen "), Arch, de med. exper., IX, 1897. — 
Naioratzki, "Zur Kenntniss d. Cerebrospinalflussigkeit." Hoppe-Seyler's Zeitschr., 
1897, Bd. 23, H. 6. — Neurath, "Referat uber Lumbalpunction." Centralbl. /. d. 
Grenzgebiete der Med. u. Chir., I, 1898. — Nolke, " Beobachtungen zur Pathologie des 
Hirndrucks." D. med. Wochenschr., 1897, Nr. 39. — Pfaundler, "Ueber Lumbalpunc- 
tionen bei Kindern." Beitr. zur klin. Med. und Chir., H. 20. Wein, Braumiiller, 
1899.— A. Pilcz, "Referat uber L. P. bei Hydrocephalus." Ibid., 1899, pp. 685, 754. 
— H. Quincke, "Zur Physiol, d. Cerebrospinalflussigkeit." Arch. j. Anat. u. Physiol., 
1872. "Ueber d. Druck in Transsudates" D. Arch. /. klin. Med., Bd. 21, 1878. 
"Ueber Hydrocephalus." X. Congress f. innere Medicin, 1891. "Die Lumbalpunc- 
tion d. Hydrocephalus." Bed. klin. Wochenschr., 1891. "Ueber Meningitis serosa." 
Volkmann's Samml. klin. Vortr., 1893. "Ueber Lumbalpunction." Berl. klin. 
Wochenschr., 1895. "Ueber Meningitis serosa und verwandte Zustande." D. Zeit- 
schr. /. Nervenheilk., IX, 1896. — H. Salomon, "Ueber Hirndrucksymptome beim 
Typhus." Berl. klin. Wochenschr., 1900, Nr. 6. — F. Schultze, "Die Krankheiten der 
Hirnhaute und die Hydrocephalic" NothnageVs Handb. d. spec. Path., Bd. IX, 3, 
1, 1901. — Seegelken (Stintzig), "L. P. bei Encephalopathia saturnina." Milnchener 
med. Wochenschr., 1896, p. 1161. — Seiffer, "Beitr. zur Frage der serosen Meningitis." 
Charite-Ann., XXIV, 1900. — Sicard, "De la forme ambulatoire des meningites bac- 
teriennes." " Cytodiagnostic du liquide rachidien." La Presse med., 1901, 69. 
Zeitschr. /. prakt. Aerzte, 10. Dec, 1901. — Stadelmann, Centralbl. f. d. ges. Therapie, 



LUMBAR PUNCTURE 257 

1898. — Wilms (Leichtenstern), " Diagnostischer und therapeutischer Werth d. Lum- 
balpunction," u. s. w. Miinchener med. Wochenschr., 1897, p. 53. 

Ueber Cocainisirung des Ruckenmarks. — Bier, D. Zeitschr. /. Chir., Bd. 51, 
p. 361. — Guinard, Miinchener med. Wochenschr., 1901, Nr. 46. — Neugebauer, Miinch- 
ener med. Wochenschr., 1902, Nr. 18, p. 741. — Hahn, "Referat iiber subarachnoidal 
Cocaininjectionen nach Bier." Centralbl. j. d. Grenzgebiete, 1900, III, p. 337; 1901, 
IV, pp. 305 u. 750. — A. Sicard, "Les injections sousarachnoidiennes et le liquide 
cephalo-rachidien." Paris, Carre, 1900. — Tuffier, "L'analgesie chirurgicale par voie 
rachidienne." Paris, Masson, 1901. 

Very complete literature up to 1899 is embraced in Neuratri's reference. 
In the preceding we have mentioned only the more recent articles and those 
of the earlier ones which were especially referred to in the text. 






CEREBRAL HEMORRHAGE AND EMBOLISM 

By R. GEIGEL, Wurzburg 

When a person is seized by apoplexy, in by far the great majority of cases 
the attack is due either to hemorrhage from, or embolism of, an artery of 
the brain. These affections differ in many features, yet they have also much 
in common, and it is worth our while to make a few comparisons in their 
description. 

In the first place both are typical focal affections of the brain. In one 
case we are dealing with an effusion of blood, a hemorrhagic focus, in another 
with the consequences of embolism, softening of the brain substance, an 
encephalomalacic focus, both occurring at the same points of preference and 
both being markedly circumscribed local affections. Common to both is a 
series of diffuse brain symptoms culminating in a more or less rapid disturb- 
ance of consciousness which may terminate in complete coma, symptoms which 
have been included under the name of the " apoplectic insult." It is impos- 
sible to decide from the symptoms alone whether rupture of the vessels or 
an occlusion has taken place. The statement is frequently made that in the 
first case redness, and in the other pallor, of the face is present — an unreliable 
differentiation upon which we should never depend in practice — and it must 
be confessed that without a general examination, without a history, the differ- 
ential diagnosis is absolutely impossible from the objective cerebral phenom- 
ena ; even including everything diagnostically valuable it is exceedingly decep- 
tive, and in many cases cannot be accurately made. 

These initial symptoms which introduce cerebral apoplexy as well as em- 
bolism may be referred to a disturbance of the entire brain function, and 
since these are of mechanical origin they must also be explained mechanically, 
and this we shall now attempt to do. 

It must be remembered that after ossification of the sutures of the human 
skull and the closure of the fontanelles, the skull resembles a capsule in which 
two fluids, the blood and the liquor cerebralis, also the semi-solid brain mass, 
are hermetically enclosed. These three constituent parts, as well as the cap- 
sule of the skull, are insusceptible to pressure, and in a physical sense may 
be regarded as absolutely rigid. We know that there is pressure — the intra- 
cerebral pressure — within the cavity of the skull, that this may vary even in 
normal conditions, and that it usually attains a height of 100 mm. water 
pressure. If the blood-vessels, the arteries of the brain, are assumed to empty 
freely into the cavity of the skull, necessarily in the interior of the skull the 
same pressure — the arterial pressure — must be present. The theory of the 
simple propagation of this pressure is opposed by the vascular tension; there- 
258 



CEREBRAL HEMORRHAGE AND EMBOLISM 259 

fore we may state it as a law that the intracerebral pressure at all times equals 
the difference between the arterial pressure and the vascular tension. When 
the arterial pressure remains the same, an increase of vascular tension leads 
to a diminution, and a cessation of vascular tension to an increase of intra- 
cerebral pressure. This process is of vital importance for the blood supply 
of the brain, therefore also for the normal processes of life. Not the quan- 
tity of blood constantly present in the cavity of the skull, but the quantity 
propelled in a unit of time through the capillaries of the brain determines 
the nutrition and proper function of the living cerebral substance. 

When the intracerebral pressure increases, the thin walled portions of 
the vascular system — the veins and capillaries — must necessarily be compressed, 
thus producing a slowing of the circulation which we designate by the term 
adiaemorrhysis cerebri — deficient circulation in the brain. Hence, from what 
has been stated, even a decrease of arterial tension while the arterial pressure 
remains the same represents a causative factor in the production of this con- 
dition. 

Any sudden arrest of the circulation in the brain may produce the symp- 
toms which we observe during an attack of apoplexy — vertigo, nausea, vomit- 
ing and loss of consciousness — and also the clinical symptoms which we desig- 
nate by the term " preeminent pressure symptoms " ; rigid pupils, retraction 
of the abdomen, and slowing of the pulse are merely the consequences of a 
greatly increased intracellular pressure, a sudden, acute adiaemorrhysis cerebri. 

How this occurs in apoplexy may be easily understood. If the arterial 
wall ruptures at any point, the arterial pressure may irresistibly be trans- 
mitted to the cavity of the skull, the intracerebral pressure increases, the veins 
and capillaries are compressed, general adiaemorrhysis occurs, the apoplectic 
insult takes place, and only gradually, after the blood and lymph have had 
time to flow off, is the equilibrium within the skull reestablished, the rupture 
in the vessels becomes occluded, the brain begins to recuperate and to function 
normally. 

The description of the process in embolism is not quite so simple. When 
a plug produces occlusion of a branch of a cerebral artery, the propagation 
of the arterial pressure in the vessel to the periphery ceases, and the vascular 
tension brings about a constriction by contraction of the walls. 

Since there is never a vacuum in the normal skull the effects of this 
constriction must be conveyed to the entire brain substance, and cause a sudden 
dilatation of all of the cerebral arteries in which no embolism has occurred. 

Therefore, at the moment when a vessel of the brain is occluded by em- 
bolism, the blood coming from the heart pours into the widened vessels of 
all the other vascular areas and dilates them, not, however, flowing from the 
arteries through the capillaries into the veins, and thus supplying the brain 
substance with freshly oxidized blood. This theory demonstrates that even 
in the veins a regurgitating stream must arise, and that in the capillaries 
— as we must infer — the circulation is for an instant arrested; they are 
filled with blood from the veins, and this acute disturbance of the circulation 
is nothing more than a sudden impairment of the circulation of the blood 
in the brain, an adiaemorrhysis cerebri acuta. 

In comparing these affections it at once becomes obvious that no differen- 
tial diagnosis between the apoplectic attack in hemorrhage and that in em- 



260 CEREBRAL HEMORRHAGE AND EMBOLISM 

bolism can be made, since in both, notwithstanding the difference in cause, 
the same mechanical factors are operative — an acute, general impoverishment 
of the supply of blood to the brain. 

While the same general and grave cerebral symptoms may be observed in 
other conditions, in intoxication, in cardiac asthenia, in uremia, in diabetic 
coma, they are distinguishing features of the apoplectic insult in that, in 
spite of the similarity of the brain symptoms, each represents a focal affection 
of the brain, and even during the continuance of the severe general picture 
produced by the phenomena of the attack, we must search for symptoms which 
will furnish a clue to the localization in the brain. 

Of course there is as yet no sign of the circumscribed symptoms of paral- 
ysis which subsequently appear when the patient has regained consciousness. 

The four extremities are apparently flaccid, and uniformly show that they 
are no longer under the control of the patient. The expression of the face 
and the very rapid drop of an extremity which has been lifted scarcely reveal 
the severe implication of one side as compared with the other. 

Very soon, even while profound unconsciousness continues, peculiar changes 
in the reflexes occur, and among these none is more important than the well 
known cremaster or — more aptly speaking, since it can be tested also in the 
female — the inguinal and oblique reflex. If we stroke the skin upon the 
internal side of the thighs toward the pudenda with a pointed instrument, 
the fibers of the internal oblique muscles contract toward Poupart's ligament ; 
this reaction, is perceptible in females, while the contraction of the sluggish 
cremaster muscle, which also takes place, is peculiar to males. 

On testing this reflex in a case of apoplexy we find it present, as a rule, 
on one side of the body and absent on the other. Upon the latter side paral- 
ysis of the extremities is to be expected, and crossed with it upon the other 
side we must assume the hemorrhagic or encephalomalacic focus in the brain. 

The position of the eyes may aid us in the diagnosis; we refer to their 
conjugate deviation. Both are turned toward one side and upward, and in 
this position may oscillate so as to simulate nystagmus. In by far the great 
majority of cases this conjugate deviation is very transitory, and disappears 
with the symptoms of the attack. The lesion in the brain may be assumed 
to be on that side to which the eyes have turned. 

Before discussing the further course of these diseases we must consider the 
etiology^ the prognosis, and the treatment of the attack. 

Here, besides many points of resemblance, we find also many differences. 

Apoplexy is a disease of advancing age; atheroma of the arteries of the 
brain forms a predisposing cause by the development of miliary aneurysms 
of the finest branches. It is also favored by cardiac hypertrophy — for instance, 
by a valvular lesion of the aorta — and by a contracted kidney; but it may 
also be the consequence of the hemorrhagic diathesis, as in purpura or scurvy, 
or may arise during the course of a severe blood affection such as leukemia 
or pernicious anemia. In embolism, under some circumstances, degeneration 
of the aortic valves may permit an embolus to be detached and subsequently 
to reach an artery of the brain. This accident occurs much more frequently 
in the young than hemorrhage, and we may therefore state the general law 
to be as follows: When an attack of apoplexy occurs in a youthful person 
who is not suffering from a disease of the blood (and this can be readily 



CEREBRAL HEMORRHAGE AND EMBOLISM 261 

determined), and who is also non-syphilitic, embolism is very likely, particu- 
larly if there is coexisting cardiac disease. 

The suddenness of the apoplectic attack and the rapidity with which 
loss of consciousness ensues is usually greater in embolism; a protracted form 
is, as a rule, common to cerebral hemorrhage, in which form, under low 
pressure, instead of streaming out the blood oozes from a narrow slit. The 
more profound the unconsciousness and the longer it continues the less hope 
is there usually that the patient will survive the attack. If in cases of hem- 
orrhage consciousness does not return within the first twenty-four hours — 
although it may be but momentary and incomplete — the prognosis may be 
declared hopeless. It is also very unfavorable when tracheal rales or Cheyne- 
Stokes respiration appear, when the pupils are rigid, when there is decided 
slowing of the pulse or this becomes very rapid, when the abdomen is re- 
tracted and the initial vomiting recurs ; when, therefore, those signs are added 
which we have learned to regard as special symptoms of pressure on the brain. 
Recovery is possible in embolism even when unconsciousness lasts for twenty- 
four or forty-eight hours or longer. On the other hand it is a favorable sign 
if consciousness is not completely lost, or if it soon returns; if intellection 
is clear from the first day, even though for a few moments only, we are justi- 
fied in hoping that consciousness -will return after a short or long period, 
that it will constantly become more clear and, finally, that regular alternations 
of waking and sleeping may be established, the sleep at the onset being still 
very profound. 

L T nfortunately, during the apoplectic attack little can be done therapeu- 
tically. It has taken place, the lesion has been effected. But a mechanical 
consideration of the injury furnishes at least an indication as to the treatment. 

When the blood has ruptured through the wall of the vessel, nothing 
prevents its further distribution except the coherence of the semisolid brain 
mass and the intracerebral pressure. "When the former is broken, and the 
blood finds its way to the free surface of the brain or to a ventricle, a massive 
hemorrhage always follows and to this the patient invariably succumbs in a 
very brief time. It is the increase of the intracerebral pressure which pro- 
duces the symptoms of the attack, and it is the increase of the intracerebral 
pressure which — apart from the mechanical resistance of the brain substance 
itself — prevents the further extent of the hemorrhagic focus; and from the 
fact that these conditions combined — resistance of the brain and increased 
cerebral pressure — equalize the arterial pressure, not a drop of blood can leave 
the vessels. 

Thus we see how incorrect it is to attempt to decrease intracerebral pressure 
during the acute attack, as most physicians do, by the application of an ice- 
bag to the head. This should never be employed until the time when severe 
brain symptoms show that an improvement of the circulation to relieve the 
greatly compressed brain is urgently necessary. If we are called to the bed- 
side of a patient and find that an ice-bag has already been applied to the 
head, we may permit it to remain in order to keep in check the symptoms of 
the attack. 

Venesection which was formerly so generally employed has fallen almost 
into disuse, and perhaps not quite justly. When the carotids pulsate, when 
the pulse is full and tense, when the face is flushed and congested, and other 



262 CEREBRAL HEMORRHAGE AND EMBOLISM 

severe symptoms appear, venesection may be performed as a last resource ; but 
it should be thorough so as to give immediate relief to the circulation, dimin- 
ish the venous pressure, and in this way secure a better circulation in the 
brain. Of course, with this procedure there is always danger that the power 
of the heart may be severely damaged,* and this cannot fail to lessen the 
recuperative power of the cerebral activities. 

In most cases, therefore, we merely loosen any clothing that may con- 
strict the body, raise the head moderately high if the patient is in bed, empty 
the rectum by an enema, and await further developments. 

Attention must be given to the bladder; many semi-unconscious patients 
cease moaning only when the paralyzed bladder which is distended to the 
utmost is emptied by catheterization. After the first twenty-four hours the 
patient may be cautiously raised to a sitting posture so that the lungs may 
be examined, and we then note whether hypostatic pneumonia, which so fre- 
quently sets in on the side opposite to the lesion of the brain, is present. If 
this has occurred the patient must be placed upon the healthy side; therefore, 
with a right-sided pneumonia, upon the left side. At the same time we must 
take the utmost precautions to prevent the formation of bed-sores upon the 
sacrum; in many cases these develop very rapidly and severely, and if prac- 
ticable it is always wise to put a patient who has been attacked by apoplexy 
upon an air or water bed. 

In embolism, theoretically and apart from general measures, entirely dif- 
ferent methods are necessary. 

It has been demonstrated that in embolism of the cerebral artery the circu- 
latory disturbance is less when the venous pressure is increased; therefore a 
low position of the head, besides stimulating its power, is always indicated 
in embolism of an artery of the brain. 

It is unfortunate that in the rarer cases of these diseases it is impossible 
at first sight to make a differential diagnosis with the slightest degree of 
certainty ! 

It has already been stated that hemorrhages and encephalomalacic foci 
have their preferred seats. These are found in the areas of the artery of the 
Sylvian fossa which supplies the internal capsule and the basal ganglia, and 
in accordance with well known laws, destruction in this region brings about 
crossed hemiplegia, a paralysis of the arm and leg and the lower branches of 
the facial nerve, probably also of the hypoglossal nerve. 

Embolism manifests a peculiar preference for the right side. Occasionally 
the paralysis may rapidly and totally disappear in days or weeks, and it is 
this fleeting character of the focal symptoms which preeminently distinguishes 
it. On the other hand, paralysis due to hemorrhage may often markedly 
improve, even late in its course. 

The hemorrhagic focus not only destroys and displaces everything in its 
immediate area but also presses upon its surroundings, and as it decreases 
in size the pressure diminishes still more, and those tracts may then recu- 
perate which — lying in the internal capsule — resupply the arm and leg which 
have been paralyzed for weeks. This distant action does not arise from the 
encephalomalacic focus; only the regions which it immediately occupies are 
destroyed, and this damage is absolute, permanent, and irreparable if, within 
the first forty-eight hours, a collateral circulation does not bring a sufficient 



CEREBRAL HEMORRHAGE AND EMBOLISM 263 

amount of blood to the brain substance. It may be remarked in passing that 
this absence of distant action is the reason why the encephalomalacic focus 
is such an excellent object for investigation in topical diagnoses of diseases 
of the brain. 

The subsequent treatment of apoplexy, the treatment of the remaining 
paralysis, should be about as follows: After from two to three months an 
attempt may be made by galvanization of the brain to repair the damaged 
tracts and to decrease the hemorrhagic focus. Only large, well moistened elec- 
trodes should be employed. The anode is usually applied upon the side where 
the hemorrhagic focus exists, the cathode upon the opposite side, so that the 
focus is in any case within the current. The current must be very weak; 
the physician himself should test its strength by placing the two well moistened 
electrodes upon his temples while his eyes are closed, and the current is then 
slowly increased until a distinct effect of light is produced in his eyes. Even 
this current is much too strong, and should not be at once applied to the dis- 
eased brain. On the contrary, we should apply electrodes without any cur- 
rent, and then use a rheostat so that the previously determined strength may 
be increased or diminished at will. The duration of the treatment should be 
from two to three minutes, and this is to be repeated at first twice, subse- 
quently four times a week. Systematic exercises in walking and in movements 
of the arm are about the only measures by which we can hope to restore motion 
in the paralyzed limbs ; exercises in speech may also be practised. The atrophy 
of inactivity may be combated by faradization of the muscles which may be 
begun about four weeks after the attack ; above all, we must endeavor to pre- 
vent what proves in most cases a great hindrance to the restoration of motion ; 
namely, secondary contracture of the paralyzed limbs. 

After a few weeks rigidity appears in the paralyzed extremities of the 
hemiplegic and the arm is adducted, rotated inwardly, flexed at the elbow, 
the forearm being pronated, the fingers and thumb flexed. 

In the same manner the leg becomes stiff, flexed at the knee, the foot 
assuming the equinovarus position. 

Heinrich Munk recently conferred a great service upon mankind in prov- 
ing by experiments upon monkeys that this contracture is chiefly the conse- 
quence of inactivity, and that it may be prevented by systematic passive move- 
ments. If, as soon as stiffness in the muscles becomes noticeable in an animal 
that has been operated upon, the affected parts are extended twice daily for 
about five minutes, but slight resistance is afterward present, and contracture 
does not follow. If we suspend these extensions, the stiffness at once increases, 
and subsequently passive movements have no effect in producing the former 
slight relaxation. Therefore in man, soon after the attack, say in about two 
to three weeks, we begin systematically to extend those muscles which are 
liable to contract, stretching them twice daily for from five to ten minutes, 
and we persist in these endeavors for months because, fortunately, it has been 
proven, of course in only a few instances, that contractures which according 
to experience would have certainly followed may in this way be actually pre- 
vented. 

A person who has suffered an attack of apoplexy is, as a rule, prone to be 
anxious, and the advice which we give him to avoid all excitement, all bodily 
exertion, all errors in diet, constipation, any extreme effect of heat to the head, 



264 CEREBRAL HEMORRHAGE AND EMBOLISM 

particularly the use of alcohol in concentrated form, is probably followed in 
most cases as carefully as possible. Although relapses occur so frequently, and 
the patients succumb in the second or third attack, this is not due to the indis- 
cretion of the patient or the physician but rather to the nature of the case, 
to the atheroma, to the valvular lesions, to those conditions which produced 
the first attack, and are just as likely to cause a second and third, and the 
final, fatal one. 



THE SYMPTOM-COMPLEX OF APHASIA 

By C. WERNICKE, Breslau 
I. DISTURBANCES OF PHONETIC SPEECH 

A woman, aged 46, the wife of a tailor, presented on examination a remarkable 
defect. She had received the average education of people of her class; at least, it was 
certain that she had been able to read fluently and to write. All signs indicated a 
focal disease of the left half of the cerebrum. There was right-sided hemiplegia 
combined with severe sensory disturbance of the entire right half of the body. In the 
course of nine months, at first slowly developing, an increasing weakness of the right 
hand had appeared, followed by three acute exacerbations of the disease, the second 
being followed by a periodical loss of the power of speech. After the third and last 
exacerbation her condition remained unchanged. Only a slight disturbance of speech 
was found, namely, some difficulty in articulation, which did not affect the distinctness 
of expression. This condition of moderate anarthria could not be left entirely out of 
consideration, yet at the present time it can be disregarded in the investigation of her 
case. The woman was thoughtful, attentive, and apparently of normal mental grasp. 
She answered all questions intelligently, a proof that she had understood what was 
spoken and had followed a definite train of thought. She read aloud fluently, and it 
was evident that she understood what she read. Every letter, every figure, was read 
correctly without the least hesitation, and she recognized and immediately understood 
all pictures, diagrams, and outlines. On the other hand she had completely lost the 
ability to write. She was under prolonged observation which gave her abundant oppor- 
tunity for practice of this kind ; the only result was that once, under urgent persua- 
sions and with visible subjective difficulty, she wrote upon a slate at dictation the 
small letter a and the figures 2, 3 and 4. The art of writing spontaneously without 
dictation was wholly lost. This was, therefore, a case of quite isolated loss of a 
previously possessed power of writing, a classical case of so-called pure or isolated 
agraphia.! 

Here we must naturally consider that the right hand, although not wholly para- 
lyzed, was generally incapacitated. There w r as complete tactile paralysis as far as all 
the finer movements were concerned, so that only gross massive movements could be 
performed. Nothing further was noted. But the art of writing, as we know from 
experience, is by no means entirely confined to the right hand. Normally every person 
who has learned to Avrite can also learn to write with the left hand; less easily of 
course than with the right, much more slowly, and in a more painstaking way, as if 
drawing. In this writing, as we must admit, there is a different technic of individual 
movements, but it may be done without extreme difficulty, and each letter and w r ord 
may be legible. Usually awkwardness is noted only upon the first attempt, and com- 
paratively good results follow a little practice. In the case of this woman, however, 
there was an almost absolute lack of power to write syllables, words, or even letters 
alone. As the power of massive movements of the right hand had been retained she w r as 
persuaded to grasp with the right hand a small block of wood to w T hich chalk was 
attached. Standing before a blackboard with this block of wood in her hand the patient 
could write nothing, although she was able to lift the right hand and guide it with 
the left. 

i Monatsschr. f. Psych, u, tf enrol., Aprilheft, 1903. 

265 



266 THE SYMPTOM-€OMPLEX OF APHASIA 

The peculiar psychical condition of the patient on any attempt to write deserves 
mention. Her expression and posture denoted extreme embarrassment and helplessness. 
She seemed lost in thought for a few minutes, was coaxed, made several efforts, and 
finally exclaimed that she was tired and could try no more. 

The apparently perfect analogy of this peculiarly circumscribed mental 
defect with the more frequent and familiar pathological picture of aphasia is 
so remarkable that the case is especially suitable as an introduction to the 
questions concerning this realm of disease which are still so much discussed. 
That in this case the faculty of writing was lost in consequence of a local 
disease of the brain is at least no more noteworthy than the familiar experi- 
ence that the power of speech may be similarly lost; if we adhere to the 
definition given by Broca (the discoverer of a circumscribed center in the brain 
for human speech) of the resulting speech disturbance, the analogy appears 
complete, for in his patient the power of articulate speech, the faculty of 
speaking, which we know must be acquired like the art of writing, had been 
lost, and this isolated defect could no more be explained as a paralysis of a 
group of muscles than in our case. Therefore, if we may assume that these 
defects are of kindred nature, we are also forced to assume a writing center 
as well as a special speech center in Broca's convolution. As a matter of 
fact many authors have drawn this conclusion from their clinical experience; 
among them we find no less a one than Charcot — the man who, as long as 
he lived, was regarded as the intellectual leader in Medicine in France. 

Nevertheless, a closer study of the entire symptom-complex of aphasia as 
well as of the symptoms of the case under discussion shows that if we admit an 
actual writing center we have already passed beyond the most obvious assump- 
tion, and that this assumption is by no means borne out by autopsy findings. 
We should be particularly cautious in the study of aphasia, for a too hasty and 
erroneous generalization of facts in themselves strictly correct and comprehen- 
sive is dangerous, and has caused every attempt at localization in aphasia to be 
regarded as Utopian. I must call to remembrance the fact that the discovery 
of Broca (1861) occurred at a time when the principles of cerebral pathology 
taught by clinicians such as Bouillaud and Andral were directly opposed to 
those of physiology as taught by Flourens. Where the first exponents pointed 
to facts which apparently favored the varying importance of different parts 
of the brain, the latter was inclined to ascribe these to the effect of Gall's 
teachings of phrenology which have quite properly been discredited, and ex- 
periments in animals were referred to as proof of the resemblance of the animal 
cerebrum in all of its parts to that of man. Hence a clinician who disputed 
the truth of Broca's discovery found a soil well prepared by skepticism. When 
Trousseau expressed his doubts, he found scientific opinion easily swayed to 
his side. The celebrated discussion of this subject which arose in 1864 in the 
French Academy of Medicine is an instructive illustration of this. But it was 
at the same time evident that in the defect which Broca called aphemia he 
meant something very different from what his opponent, Trousseau, under- 
stood. Pure cases of Broca's aphemia, as Broca expressly maintained, are 
characterized only by the " impossibilite de parler " without other disturbance. 
This patient, according to Broca, lost " la f aculte d'articuler les mots," " le 
souvenir du procede, qu'il faut suivre pour articuler les mots." Broca ex- 
pressed himself still more clearly when he characterized the process by which 



DISTURBANCES OF PHONETIC SPEECH 267 

the child learns to speak, as follows : " Par le developpement d'une espece 
particuliere de menioire, qui n'est pas la memoire des mots, mais celle des 
mouvements necessaifes pour articuler les mots." Xow there was no reason 
why Trousseau should not develop a picture of aphasia differing entirely from 
that characterized as aphemia, which is very rarely seen in a pure type; and 
he pointed out the many remarkable and perfectly incomprehensible phenomena, 
at that time observed in these patients. But these cases were of entirely unlike 
character; it is true they seemed to belong to the same aphasic symptom-com- 
plex, as we understand it to-day, but they were vastly different from the con- 
dition described by Broca as aphemia. If the necropsy findings in many 
of these cases showed Broca's convolution to be uninjured, we to-day are 
inclined to regard this as favoring rather than opposing Broca's localization. 
The presumable proof of the contrary, which Trousseau evolved from the vary- 
ing necropsy findings, therefore related to an entirely different clinical picture. 
In addition, numerous cases in which Broca's symptom-complex was observed 
as an indirect focal symptom could not at that time be satisfactorily explained, 
and these also appeared to contradict Broca's discovery. This led to the 
inevitable and undesirable result that the symptomatology of aphasia was in- 
creased by the addition of many individual points; but these, although a 
fundamental gain, and although at first welcomed with great enthusiasm 
as marking an epoch of advance in the knowledge of cerebral functions, were 
finally considered to be unproven and were discredited. Xot until an enor- 
mous impetus was given to the stud}' of the anatomy of the brain by Meynert, 
and to the understanding of the experimental physiology of the brain by 
Fritsch and Hitzig, was a more profound interest awakened in the clinical 
picture of aphasia in Trousseau's sense, as well as a correct appreciation of 
Broca's discovery. Even the name aphemia, employed by Broca in its re- 
stricted sense for the previously described clinical picture, had been relegated 
to oblivion. 

If we recall that it was chiefly the hope of discovering a localization for 
cerebral functions that introduced Broca's discovery, which was vitiated by 
Trousseau's influence, we will be inclined to ascribe a paramount role to the 
experimental proofs furnished b} r Fritsch and Hitzig that there are motor 
points in definite areas of the animal brain which may be stimulated, and 
that this led to the consideration of aphasia in the sense of cerebral localiza- 
tion. But even Hitzig's experiments would have proven, incomprehensible, 
and he would probably have found no adherents, if Meynert had not given 
us such ingenious and convincing descriptions of the anatomical structure of 
the human and animal brain as to enable us clearly to understand the func- 
tion of the cerebral cortex. We are indebted to Meynert for our conception 
of "the projection systems" which in a physiologic sequence embrace, on 
the one hand, all of the sensory and motor tracts leading from the organs 
of special sense to the musculature, and, on the other hand, also to the cortex 
of the cerebrum. While stimulations from the body can be conveyed to the 
" sensory sphere " of the cerebrum only through this tract, the impulses which 
arise in the latter are projected to the muscles. According to Meynert. a trans- 
verse section of the cerebral peduncles would include the entire organism, 
which " would be devoid of the senses of smell and sight." The ganglion cells 
of the cortex of the cerebrum have everywhere the same elementary function, 



268 THE SYMPTOM-COMPLEX OF APHASIA 

and only from the variation of their combinations with the periphery of the 
body by means of the projection systems are their specific qualities arising 
from different localities of the cortex manifested. From anatomical investi- 
gations, Meynert came to the conclusion that the cerebrum may be divided 
into two large territories, an anterior one of motor, and a posterior of sensory, 
importance. Memory pictures produced by this function, and which fill the 
cerebral cortex, are motor in the anterior structures and sensory in the pos- 
terior. 

Viewed from this standpoint, the movements which Fritseh and Hitzig 
produced by galvanic irritation of the anterior portion of the brain of a dog 
appeared in a new light. They did not resemble those evoked by irritation of 
special peripheral nerves, but involved several muscle groups, and produced 
distinct movements simulating those which are voluntary. Both of Hitzig's 
extirpation experiments, which were at once published, permitted the inter- 
pretation that the resulting disturbance of movement depended upon loss of 
motor memory pictures or conceptions of movements, and Broca's aphemia 
also appeared to be a loss of motor memory pictures, particularly for the 
movements of speech which had been acquired. The time at which a child 
learns to speak is especially the time for this functional acquirement of motor 
memory pictures, or, more definitely stated, " du developpement d'une espece 
particuliere de memoire, celle des mouvements necessaires pour articuler les 
mots/' It will be observed that Broca, with the remarkable acuity of great 
discoverers, had taken into consideration only those functions of definite parts 
of the brain which (in Meynert's opinion this was subsequently fully con- 
firmed) more nearly corresponded to the motor area of the cerebral cortex. 

Nevertheless, the remarkable variety of the clinical forms of aphasia con- 
stituted a fact which demanded consideration and explanation. Here also the 
ground was broken by Meynert, for this author believed he had found in this 
peculiar structure (in his opinion the claustrum was entirely composed of 
association cells, its area of distribution being in the island and its cortical 
convolutions) the central termination of the auditory nerve, and at the same 
time a sound area for speech. Although the auditory nerve could not be 
traced up to the island of Eeil, nevertheless, the significance of this nerve and 
of the island for the function of speech called attention to their previously 
ignored connection. 

It is evident that, after Meynert's investigations, we had all the prerequi- 
sites for the full and comprehensive understanding of aphasia. My work upon 
" The Aphasic Symptom-Complex " which appeared in 1874, was merely a 
minute explanation of Meynert's theories and their special application to 
human speech. In the anterior region of the brain, Broca's convolution, we 
possess a motor speech center which we regard as the region of motor memory 
pictures of speech, or conceptions of speech movements, and the origin of 
speech impulses; in a posterior region we assume a sensory speech center, 
the region for pictures of speech sounds, and at the same time the terminal 
point of the auditory nerve. The child's brain receives and stores up memory 
pictures of the sounds of speech which it has heard and gradually imitates 
these; thus speech becomes the functional acquirement of every individual. 
Combined fiber masses, " association tracts," between the sensory and motor 
speech centers, transmit this act of imitation, and also the power of speech. 



DISTURBANCES OF PHONETIC SPEECH 269 

From general anatomical considerations which permit us to regard the entire 
island covered by the first embryological convolution arches as an anatomical 
entity, I believe the sensory speech center to be situated in the first temporal 
convolution, and the postulated association tract in the association organ de- 
scribed by Meynert as located in the fiber system extending through the cortex 
of the island, the deepest layer of which loses itself in the claustrum. Fortu- 
nately the findings at two autopsies confirmed this presumption that the sensory 
speech center was in the first convolution. In both cases the posterior half 
of the longitudinal course of the first temporal convolution and a closely 
adjacent area of the second temporal convolution were destroyed by a focus 
of softening. This, of course, permitted the differentiation of three clinical 
forms of aphasia, a motor, a sensory, and a third form which I designate 
conduction aphasia. The motor form had already been clearly described by 
Broca; it was the aphemia of this author. Sensory aphasia was chiefly char- 
acterized by the fact that the sounds of speech were heard, and the power of 
articulate speech was retained, but the speech sounds were not understood. 
This symptom, the confounding of words (the paraphasia of Ivussmaul), was 
explained by the hypothesis that the sound pictures of phonetic speech pre- 
sumably regulated the speech. The clinical existence of conduction aphasia 
appeared to be proven by cases of paraphasia in which both the power of 
speech and its understanding were retained. The article published in 1874 
explained the seeming contradiction, which, according to Kussmaul, still 
existed in a description regarded as classical, and which was opposed to any 
localization of speech. After the opinion had been expressed that Broca's 
convolution was the only speech center, and this was assigned its proper 
position as being exclusively the center for motor speech, skepticism, which 
had opposed further efforts at localization, at once gave way. With the 
appearance of Kussmaul's book, which in a clinico-symptomatologic sense 
is still valuable, skepticism was routed. And as Meynertfs point of view in 
regard to one realm of the pathology of the brain was accepted, that one 
which had heretofore been regarded as especially difficult and impenetrable, 
this opened the way to further revelations of the secrets of the brain, and to 
expectations which have not failed of realization. 

The clinical picture of sensory aphasia with its localization in the first 
temporal convolution and the adjacent parts of the second temporal con- 
volution was soon generally recognized. Kahler and Pick, subsequently 
Lichtheim, also French, Italian and English authors, helped to bring about 
this recognition, and we maintain to-day that the clinical picture of this 
special form of aphasia and the necropsy findings are just as surely based 
upon facts as is Broca's motor aphasia. It, therefore, becomes my duty to 
emphasize that the chief symptom of sensory aphasia, arrest of the under- 
standing of speech with retention of the power of hearing, had already been 
recognized by two authors, by Schmidt x upon the basis of his own observa- 
tions, and by Bastian 2 after reports of strange cases. These authors did 
not ascribe defective speech to a definite locality of the brain; hence we are 
constrained to admire their acuity which enabled them to grasp the true 

lAllg. Zcitschr. f. Psychiatr., 1871, XXVII. p. 304. 

2 On the various forms of loss of speech in cerebral disease. British and Foreign 
Med. Chir. Review, April 1869. 



270 THE SYMPTOM-COMPLEX OF APHASIA 

condition while ignorant of Meynert's point of view and the process of its 
development. 

As already stated, the most important point is proof (which I attempted 
to demonstrate) of the fact that the aphasic symptom-complex embraces the 
conflicting clinical pictures of motor and sensory aphasia, and the anatomical 
localization of these contrasting conditions has been proven to be in two 
entirely different areas of the brain. Historic retrospect here teaches us but 
little, and is therefore unnecessary. But an essential and very important 
advance was made in this direotion when we arrived at a clear understanding 
of the relation which must be assumed between the diametrically opposite 
functions of these two centers, and upon this standpoint we base the follow- 
ing description. 

The sensory speech center is the region in which, corresponding to the 
central termination of the auditory nerve, the memory pictures of speech 
sounds that have been heard, sound pictures (Helmholtz), have their ana- 
tomical substratum in the ganglion cells of the cerebral cortex, " cortical 
units" (H. Sachs). 

Hence this transmits the understanding of word sounds (Liepmann), the 
recognition or the " identification " of the perception of sounds which form 
the word, a function which must be strictly differentiated from the under- 
standing of the " sense of the word." The mere thought of a foreign lan- 
guage enables us to understand the necessity of this differentiation, for we 
learn to comprehend our mother tongue in the same way in which we learn 
a foreign language. Most children understand spoken words, and even their 
meaning long, even years, before they acquire the power to articulate these 
words. From this, as we shall soon see, a certain independence of the sen- 
sory speech centers arises, in comparison with the much more dependent 
motor center. But we must admit individual variations in this duplex rela- 
tion, since undoubtedly there are children whose power of articulation keeps 
pace with their understanding of word sounds. The words which the child 
learns to speak have at first nothing to do with the artificial sound forma- 
tion of written language. There are complicated words the sound picture 
of which, as well as the speech movement conception thereof, must be slowly 
acquired by practice. The possession of both of these memory pictures, inti- 
mately combined for each word, is what the French authors have designated 
as " internal speech" which suggests also a mental power, a kind of memory 
— and this may be likened to Broca's definition given above. We will subse- 
quently see that a firm combination of associated memory pictures consti- 
tutes for us the nature of conception. For this reason I proposed the desig- 
nation word conception, recognizing the acquirement of word conceptions to 
be the most important process in learning to speak. Dejerine has accepted 
this view and employs the phrase " notion du mot." The expression " speech 
conception of words" (Caro) appears to me to be happily chosen, for the 
correct articulation of words, the intactness of both components of word 
conception, the sensory as well as the motor, is absolutely necessary. Hence, 
if only the method of learning to speak is unquestionable, it is nevertheless 
very doubtful whether subsequently the impulses for movements of speech 
are not referable to the tract through which speech was originally learned 
by imitation. A number of authors hold this to be so self-evident (I shall 



DISTURBANCES OF PHONETIC SPEECH 271 

mention only Kussmaul and Bastian) that they scarcely deign to discuss 
the question. Yet clinical experience up to the present time is overwhelm- 
ingly opposed to this view. As we shall soon see, these are chiefly experi- 
ences of sensory aphasia attributable to destruction of the sensory speech 
centers. We must therefore assume that the main impulses of speech are 
directly transmitted from the remaining cerebral cortex to the word concep- 
tion, and when this is deranged by an absence of the sensory center the 
impulse is transmitted to the speech movement conceptions; hence the power 
of articulate speech is retained, but is disturbed only because of the lack 
of a regulating influence of the sensory speech center which determines the 
choice of the correct conceptions of movement. 

This description enables us clearly to understand the clinical picture of 
sensory aphasia as well as its principal characteristics. 

(1) The comprehension of the sound of words is defective, and as this 
comprehension by way of the hearing is a prerequisite to the understanding 
of the sense of words the comprehension of spoken words is also defective. 
In any case the defect is purely acoustic, and the investigator is very liable 
to fall into error if he attempts to facilitate the understanding of the sense 
of the word by signs, looks or gestures. If, however, by refraining from 
this, it becomes evident that none of the speech sounds heard by the patient 
have been understood, he may prove by means of these signs and gestures that 
the hearing is still intact or, if defective, that the degree of deafness present 
by no means explains the defective comprehension of the sense of words. As 
is well known, even with extreme peripheral deafness the ability to under- 
stand the sound of words is usually unimpaired. 

(2) The power of articulate speech is retained, the patients' speech even 
being remarkably voluble, perhaps in consequence of the frequent misunder- 
standings to which they are exposed. For, although they speak rapidly, use 
a comparatively rich vocabulary, and form their phrases properly, they fre- 
quently make mistakes in the choice of an expression, or employ incorrect or 
distorted words without observing it. Under excitement speech is sometimes 
much better than at others, and entire sentences may be spoken perfectly. 
Objects shown them may be incorrectly named, or distorted words are fre- 
quently used in naming them. Their confusion of words in spontaneous 
speech may so increase that it is impossible to understand them, and we 
then designate their speech as jargon aphasia. On attempting to answer 
questions these patients never use words in the same sense in which they 
have heard them. Their inability to comprehend the sounds of words appar- 
ently also makes them incapable of imitating the sounds they have heard. 

(3) I shall not here discuss written language, since it is not yet the 
common property of mankind, and consequently it often fails to throw light 
upon the clinical picture. We shall see later that this ability to write is 
combined with an intact power of internal speech, what we have called the 
conception of words, and therefore is invariably and severely damaged in sen- 
sory aphasia. 

Sensory aphasia in the overwhelming majority of cases is due to the occlu- 
sion of vessels, therefore, usually has an acute beginning. As a rule the 
symptoms of the attack are slight, sometimes entirely lacking, and there may 
be no sign of unilateral paralysis. As to the prognosis of the disease, views 



272 THE SYMPTOM-COMPLEX OF APHASIA 

differ. Dejerine concluded from his observations that there is usually a 
permanent defect; the majority of authors, with whom my experience leads 
me to coincide, are of the opposite opinion, and believe that a comparatively 
rapid compensation of the defect is the rule. In fact the difficulty appears 
to remain at its acme only for a few weeks, when, provided no intercurrent 
condition appears, it gradually disappears in the course of a few months. 
Thus, by means of a returning sense of hearing, pictures of word sounds 
are newly acquired, and those sound pictures are permanently lost which the 
daily intercourse of the patient gives him no opportunity of using. I have 
repeatedly succeeded in demonstrating traces of the preceding disease by prov- 
ing the patient's absolute inability to understand words or phrases in rare 
use, such as vertebral column, popliteal space, axilla, and the like. The 
inability to name correctly any objects presented to view appears to persist 
for a long time. The conditions in regard to the restitution of written lan- 
guage have not been thoroughly studied. 

No less easy is the description of motor aphasia, the aphemia of Broca. 
In adhering to the preceding train of thought the following symptoms are 
prominently revealed, so that the clinical picture is easy of recognition. 

(1) The power of articulate speech is wanting. The patients have for- 
gotten the process, the mechanism, which they formerly called into action 
to produce its sounds. They are therefore mute — at least, they have only 
a minimal power of speech. In some typical cases even this is lacking, and 
nothing remains but the power to make and repeat a few inarticulate sounds. 
The defective speech most frequently observed consists of senseless syllables, 
perhaps of a few words or phrases, or it may be some profane or emotional 
expressions. 

These few words, monotonously repeated, to which speech is limited are 
not articulated voluntarily and exclusively to express what they mean, but 
they appear as an invariable reaction to all the demands which are made 
upon the patient's power of speech. It may happen after some exceptional 
emotion that these patients, who are usually mute, will give utterance to a 
natural expression, as a rule merely an interjection, yet subsequently they 
are unable to do this voluntarily. It has also been observed that during 
sleep words and phrases are uttered which the patient at other times is in- 
capable of enunciating. As a rule, the faculty of speech is entirely lost. 
This loss of speech cannot be ascribed to any paralysis of the muscles, for 
a test of their functions excludes all forms of bulbar paralysis. 

Often we find as an accompanying phenomenon a hemiplegia or hemi- 
paresthesia, a unilateral weakness in the right lower facial or lingual region, 
sometimes only in the region of the right lower facial or right hypoglossal 
branches. A clinical symptom of the latter is the deviation of the tongue 
to the right whenever it is protruded. As these signs of paralysis also accom- 
pany articulation which is really unimpaired, they have merely the signifi- 
cance of an accompanying symptom, as has been sufficiently demonstrated 
by clinical experience. 

A certain impairment of the mechanism of speech which we must assume 
to be analogous to defective speech is so common as to demand attention. 
This is manifested by the impossibility of producing complicated movements 
in the region of speech muscles. Thus, some of these patients cannot put 



DISTURBANCES OF PHONETIC SPEECH 273 

out their tongues, cannot inflate their cheeks, cannot gnash their teeth, or 
they are unable to open their mouths upon being bidden to do so, without 
simultaneously protruding the tongue, etc. These futile attempts at speech 
movements, which could be previously performed with ease, permit us to 
recognize most distinctly the patient's inability to perform the required 
maneuvers. According to Broca's excellent definition they lack for this " le 
souvenir du procede qu'il faut suivre." Sometimes these symptoms occur 
only during the early and acute stages of the disease. 

(2) In the main, the power of understanding speech is retained; at least 
this appears to be the case on ordinary tests. The faulty reaction may in part 
be referred to an impossibility of movement, analogous to that in the case 
just described and of the same significance. Therefore all orders given may 
be promptly carried out, objects asked for may be produced, questions, to 
judge from the expression of the face, are clearly understood, and the like. 

There is almost invariably a certain inability to understand complicated 
constructions and the finer differentiations of speech, as was first pointed out 
by Dejerine, and illustrated by numerous convincing examples. Here the 
contrast to sensory aphasia is so marked that a sharped differentiation is 
justifiable, and the sensory defect is amply compensated for as soon as we 
furnish the necessary mental stimulus and corresponding surroundings. I no 
longer am of the opinion that in pure motor aphasia the ability to under- 
stand speech always remains unimpaired, because I consider the acquirement 
of word conceptions an important phase in the process of learning to speak. 
Since, in this respect, we must admit a decided individual difference, proper 
allowance must be made for this in examining the patient and in estimating 
his case. 

(3) What is true of sensory aphasia is also true of written language. If 
the individual in question had previously possessed this faculty it is lost 
simultaneously with articulate speech. 

Motor aphasia like sensory is a pathologic picture which is most frequently 
of acute development, usually with the symptoms of apoplexy and more or 
less profound right-sided hemiplegia. 

The prognosis quoad restitutionem is generally unfavorable unless the 
affection is transitory and functional, i. e., unless there is an embolism which 
is compensated for by the immediate production of a collateral circulation 
or is an indirect focal symptom in which motor aphasia is only the secondary 
effect of a focus in the vicinity of Broca's convolution. This is a frequent 
occurrence; we sometimes even observe that motor aphasia appears indirectly 
as a focal symptom with left-sided hemiplegia if extremely severe symptoms 
of apoplexy accompany it. If there is an improvement in motor aphasia 
without the previously mentioned exceptional conditions, and this is rare, 
speech is permanently impaired. I had under observation a patient who, 
by very painstaking efforts and by methods similar to those used with deaf- 
mutes, was in the course of years taught to speak and, in the main, could 
be understood. But the force and exaggeration of his speech processes, the 
constrained movements like grimaces which involved the entire face, and his 
slowness of speech, were remarkable. In my experience this helplessness, 
this recognizable and, to the expert, extremely characteristic want of control 
19 



274 THE SYMPTOM-COMPLEX OF APHASIA 

of the " pro-cede qu'il f aut suivre pour articuler " is permanent even in the 
most favorable cases. On repetition the speech continues to be almost as 
faulty as when spontaneous. But Dejerine and Thomas have recently re- 
ported that under the systematic optical instruction usually practised with 
deaf-mutes we may often achieve remarkable results even in long-standing 
cases (fifteen years!) in a surprisingly short time (six weeks!). I shall 
revert later to this theoretically important point. In comparatively rare 
cases motor aphasia is not so complete, or after some time it becomes evident 
that considerable power of speech remains. In such cases it is possible to 
induce the patient to repeat easy, simple words, although always with evident 
effort, but he never succeeds with longer or more difficult words or* with com- 
plicated sentences, and he becomes greatly confused so that, for example, 
instead of saying " I " he says " you," instead of saying " good " he pro- 
nounces his own name, no matter how dissimilar; therefore the most unlike 
conceptions of movement, if retained in any appreciable degree, are con- 
founded on attempting to speak. An unexpected interjection such as " 
God ! " may sometimes be ejaculated by these patients. I admit such occur- 
rences, but the clinical resemblance to motor aphasia extends no further, and 
the contrast between sensory and motor aphasia is evident even to the tyro 
in medicine. 

Of conduction aphasia, the third clinical form which I attempted to 
differentiate from the many aspects of the aphasic symptom-complex, there 
are as yet but few reports, and even these fail to coincide, hence it is impos- 
sible to describe a uniform clinical picture on empiric foundation. I was 
at first inclined to regard the majority of the cases which did not show the 
Broca clinical picture but only the symptom of paraphasia or confusion of 
words as sensory aphasia, because in these forms the power to comprehend 
speech was retained. This opinion was shattered by finding that I had 
much over-estimated the possibilities in these cases, and the view was shown 
to be untenable. Of course certain positive characteristics are necessary, par- 
ticularly the loss or impairment of word conceptions, the clinical signs of 
which I shall subsequently minutely consider, as well as the impossibility of 
repeating words; according to the views developed above, the tract which 
serves for the repetition of acoustic word sounds is especially implicated. 
In both directions positive reports are wholly lacking, and I therefore limit 
myself to one important observation. If, under some circumstances, the mere 
sound of a word is sufficient for its full understanding — and, as shown above, 
this is true of the majority of words frequently used — and if, on the other 
hand, words can be spontaneously spoken without the previous stimulation 
of the sound picture, after interruption of the association tracts between the 
sensory and motor speech centers it should still be possible to repeat at com- 
mand words whose meaning is understood. But this repetition will not be 
so infallibly successful as in the tract developed in childhood while learn- 
ing to speak; it will be partially correct and partially paraphasic. When 
paraphasic, the patient will notice his error and endeavor to correct it. It 
is positive proof that this earlier tract is unimpaired if the patient can imme- 
diately repeat on request, so-called echolalia, which is purely automatic and 
often evoked without command at the moment of speaking, and also if he 
is able to repeat incomprehensible, meaningless words or phrases, for exam- 



DISTURBANCES OF PHONETIC SPEECH 275 

pie, words from a foreign language. If this particular form of repetition 
is impossible, yet on the other hand the power of speech and its understand- 
ing be retained, also paraphasia and the ability to criticise errors that have 
been made, I consider the clinical requirements to have been sufficiently ful- 
filled. I cannot, however, refrain from emphasizing that the autopsy findings 
(for example, exclusive or predominating destruction of the island) are not 
calculated to support the view of conduction aphasia postulated by me. 

If, as I believe I have demonstrated, the assumption of a sensory and 
motor speech center has been corroborated by anatomical findings, as well 
as by clinical observations, it follows in logical sequence that still other 
clinical pictures must be embraced in the rich aphasic symptom-complex. 
Lichtheim was the first to recognize clearly this logical principle, and he 
also deduced the necessary corollary. In rare cases, and the pure cases are 
always rare, it seems possible that the medullary substance which contains 
the projection fibers of both centers is alone interrupted by a focus, while 
the centers themselves remain intact. If these centers still exist in com- 
bination, a condition which of course can only rarely be possible, a new clin- 
ical type must result bearing some resemblance to the previously described 
ones inasmuch as it presents the fundamental symptoms, loss of power to 
understand spoken words and the power of articulate speech ; they have, how- 
ever, the differentiating factor of retained " internal speech," the intact 
" word conception." As a matter of fact the corresponding clinical picture 
in the motor region is not so infrequently met with, especially if there is 
simultaneously hemiplegia. The counterpart, sensory aphasia, is much rarer 
but has unquestionably been observed. In the light of anatomical researches 
the designation of subcortical motor or sensory aphasia would be applica- 
ble to these two types of disease. According to clinical criteria the desig- 
nations "pure word mutism" and "pure word deafness," are more wisely 
chosen. 

Pure word mutism differs from cortical word mutism due to destruction 
of Broca's convolution in the fact that efforts to speak, although futile, per- 
mit us to recognize the undamaged " internal speech " or " word conception " 
even though merely from the sound or rhythm. This is most apparent when 
the power to produce sounds is to some extent still present. Xever do such 
absolutely dissimilar reactions occur on trying to repeat words as in cortical 
motor aphasia. What is spoken is perfectly understood, even when compli- 
cated demands are made upon the faculties. Moreover, the ability to write 
is unimpaired, the patient reading and writing without effort, the latter, 
in spite of the almost invariable accompaniment of right-sided hemiplegia, 
being somewhat clumsily done with the left hand. The mechanical power of 
translating the intact word conception into sounds has simply been abol- 
ished. Similar conditions prevail with pure word deafness; here also the 
entire internal apparatus of speech, both the cortical centers, and their simul- 
taneous action, so that the word conception remains clear, and spontaneous 
speech is absolutely free and coherent, the power of written expression does 
not suffer, and that of reading is unaltered. The understanding of the word 
sounds, of what has been spoken, is alone lacking, an adequate power of 
hearing being evidently retained. 

We must now investigate somewhat more minutely both of these clinical 



276 THE SYMPTOM-COMPLEX OF APHASIA 

pictures with which we have become familiar, not only on account of their 
theoretic interest but on account of the diversity of opinions concerning their 
recognition and correct appreciation. Pure word mutism, or subcortical motor 
aphasia, is undoubtedly of vast importance in both of these disturbances. 
Long recognized as a clinical picture, just as Trousseau recognized the re- 
markable fact that aphasic mutes could occasionally still read and write, these 
rare cases were considered to be the purest examples of motor aphasia, since, 
according to Charcot, there was a special center for reading and writing. We 
are indebted particularly to Dejerine, who was the first to obtain autopsy 
findings which, contrary to the opinion of Charcot, absolutely proved the 
true nature of these cases and the existence of a subcortical form of motor 
aphasia, that they were brought into general recognition. 

In regard to the anatomical location exclusively involved in the subcorti- 
cal interruption of the corona radiata fibers coming from Broca's convolu- 
tion, authors are quite unanimous. Only the medullary layers of Broca's 
convolution itself or its point of entrance at the centrum ovale can be con- 
sidered; if the projection fibers lower down and within the trunk of the 
brain are affected, the picture of motor aphasia is no longer produced but 
disturbances of articulation prevail, while there is still a distinctly recogniz- 
able use of words; this is probably due to the position of the fibers, but of 
this we have as yet no exact knowledge. The uncommonly frequent right- 
sided hemiplegias from focal disease of the corpus striatum or the internal 
capsule are usually unaccompanied by motor aphasia, or at most it is of 
exceedingly brief duration and must be regarded as an indirect focal symp- 
tom. The facts which led me to assume a special motor speech tract extend- 
ing continuously from Broca's convolution to the nuclei of the nerves of speech 
movement in the medulla oblongata and skipping the internal capsule, I shall 
refer to subsequently. Here I limit myself to the mere mention of the ques- 
tion of an exclusively unilateral innervation of the bulbar apparatus in speech ; 
this question must later be discussed, but now it would lead us too far afield. 
The intactness of word conception is a sign which permits us to recognize this 
form also in those who cannot read or write. Lichtheim proposed a method 
by which the patient informs us of the number of syllables in a word which 
he understands but cannot speak because of his infirmity; for instance, on 
being shown an article to be named, the patients are told to squeeze the 
hand of the physician as many times as there are syllables in the word. The 
objection may be raised to this that those who do not know how to spell are 
often ignorant of the number of syllables in a word. For this reason the 
ability to count has been accepted as evidence of retained internal speech 
(Frankel and Onuf). A far more reliable guide it appears to me is that the 
sounds which are indistinctly articulated permit us at least by their rhythm 
to guess the word intended. The same aids must be resorted to if, in advanced 
cases of progressive bulbar paralysis, we desire to demonstrate the intactness 
of " internal speech." 

A somewhat more elaborate description must be given of pure word deaf- 
ness, or subcortical sensory aphasia. That this in its perfect purity is not 
rare in the clinical picture above described is acknowledged by all. The 
objections to it which have been made refer, on the one hand, to the postulated 
unilateral position and the subcortical seat of lesion, and, on the other hand, 



DISTURBANCES OF PHONETIC SPEECH 277 

to the possibility which has been maintained of the peripheral development 
of the pathologic picture from disease of the terminal distribution of the 
cochlear nerve in the labyrinth of the organ of hearing. The question of this 
unilateral position will be more minutely discussed later. But there can be 
no doubt that the motor as well as the sensory speech center is generally 
unilateral and situated in the left hemisphere. For the motor speech center 
— and this has been universally done — acting on the principle of a saving 
of labor, we may assume that a facility in learning to speak, being combined 
with a symmetrically acting musculature, stimulated from each hemisphere, 
is also derived from only one hemisphere. We must also assume anatomically 
similar conditions and relations in the projection system and the cortex for 
both auditory nerves, in that each hemisphere contains a central projection 
field for these. But it is difficult to believe that this should occur in the act of 
sensory projection in only one and that the left hemisphere, and that only 
in this are there memory pictures of speech sounds that have been heard, 
as well as a center for the understanding of word sounds. The facts of corti- 
cal sensory aphasia permit no other explanation, and since we must be con- 
tent with this plausible principle of a saving of labor, recent investigations 
have shown that for the acoustic picture memory, and even the simplest per- 
ceptions of tone, a motor component is admixed from the start so that the 
labor saving principle, or, more accurately expressed, the conservation of 
motor nerve activity, must also be operative in acquiring acoustic memory 
pictures. At all events, if a center for the understanding of word sounds 
is unilaterally located, it also seems possible that the projection fibers extend- 
ing to it may be unilaterally interrupted, and the conduction path to the 
center for the understanding of word sounds be blocked, while the intactness 
of the same projection fields of the other hemisphere may permit other tones 
and sounds to be perceived and correctly appreciated. 

A few anatomical considerations are justifiable. The clinically indisputa- 
ble connection of each auditory nerve with the hemispheres must have its ana- 
tomical substratum in a semi-decussation of the auditory nerves. As such 
area, only the posterior field of the medulla oblongata can be considered. 
Later the posterior corpora quadrigemina and the internal geniculate body 
form primary centers of hearing (analogous to the primary centers of sight 
in the anterior corpora quadrigemina and the external geniculate body), 
hence the posterior superior peduncle, that is, the pedicle of the internal 
geniculate body from the first temporal convolution is certainly the portion 
of the tract here under discussion. Besides this peduncle of the internal 
geniculate body, it is possible that a special portion of the optic thalamus 
from the temporal lobes also belongs to the central auditory tract. This can 
be situated onty in the immediate vicinity of that previously mentioned. It 
is certain that portions of both acoustic nerves are within these areas of the 
corona radiata fibers of the temporal lobes. That a unilateral focus in this 
region of the corona radiata fibers of the temporal lobe may produce pure 
word deafness as a permanent symptom has been proven by Liepmann's 
undoubted case. 

S. Freund has maintained that pure word deafness must be assumed to 
be the consequence of peripheral disease of the auditory nerve in the laby- 
rinth. He bases this assumption upon his observation of a case in my Clinic, 



278 THE SYMPTOM-COMPLEX OF APHASIA 

which he has, however, incorrectly reported 1 since he ignored the positive 
symptoms of cerebral disease which are mentioned in the history. Even less 
conclusive are his two other cases. However, S. Freund has done us a great 
service in having shown the necessity in such cases of minute examination 
of the ear by a specialist. Bezold 2 has proven that the majority of the fibers 
of the auditory nerve are not involved in the comprehension of sounds of 
speech, and that " in the entire musical scale there remains only the short dis- 
tance from b'-g" inclusive, therefore a large sixth, the perception of which is 
absolutely necessary for the understanding of speech." Liepmann 3 showed 
that a few tones besides these, either above or below and amounting to an 
octave, are also involved in the understanding of human speech, so that from 
the entire series of tones less than two octaves are necessary. In the case 
from my Clinic which Freund utilized it was deemed advisable to investigate 
with a continuous series of tones. This was subsequently done, and it was 
demonstrated that in this patient these two octaves in particular were well 
retained. In H. Liepmann's case in my Clinic, on which a necropsy was 
held, the same examination was made and proof established. 

The human faculty of hearing appreciates a series of tones ranging 
through more than eight octaves. Since merely two of these are absolutely 
necessary for the understanding of speech, and all of the parts of the auditory 
nerve in the cochlear are uniformly distributed, we come to the conclusion that 
only the fourth or the fifth part of the projection fibers of the auditory nerve 
need find its central end in the sensory speech center. This circumstance may 
possibly explain the relatively slight extension of the sensory speech center; 
this, according to numerous autopsy findings, extends only to the posterior 
third or the posterior half of the first temporal convolution and the adjacent 
area of the second temporal convolution; hence from anatomical and 
physiological considerations there can be no doubt that the remaining part 
of the temporal lobe also serves in part as the termination of the auditory 
nerve. 

These newly determined facts force us to assume that the sensory speech 
center coincides with the termination of those projection fibers which include 
the series of tones from b'-g". From this results the postulate that in cases 
of subcortical sensory aphasia the left temporal convolution would always 
be actually deaf to the aforesaid tones — if its function could be separately 
examined, which of course is impossible. In other words, that word deaf- 
ness, and not an actual although partial deafness for certain tones, results 
from unilateral lesions, and is explained by the fact that the affected portion 
of the fibers of the auditory also reach the right temporal lobes, and thus 
permit the perception of the same tones. 

This view also explains the above mentioned possibility of acquiring new 
acoustic word pictures, and thus compensating for the defect. If the corre- 
sponding cortical areas of the second right temporal lobe are subsequently 
affected, permanent sensory aphasia supervenes, and can no longer be com- 

i Compare H. Liepmann, " Ein Fall von reiner Sprachtaubheit." Psychiatr. Abhand- 
lungen, Hefte 7-8, Breslau, 1898. 

2 " Das Horvermogen der Taubstummen und Nachtrage dazu." Wiesbaden, 1895- 
1896. Zeitschr. f. Ohrenheilkunde, XXXVI. 

sL. c. 



DISTURBANCES OF PHONETIC SPEECH 279 

pensated for, as 0. Berger has proven by one of the earliest observations 
bearing on this affection. 

If we extend our researches still further, we arrive at the postulate that 
cases of sensory aphasia from bilateral disease of the temporal lobes run their 
course with actual tone deafness in the area of the previously mentioned tone 
heights. The future will reveal whether this supposition is actually correct. 
But, up to the present time, such investigations in cortical sensory aphasia, 
the difficulty of which we do not deny, have not been systematically at- 
tempted. 

On the other hand it becomes quite clear that with distributed bilateral 
disease of the temporal lobes the picture of word deafness is less prominent 
than that of general deafness. In the majority of cases the nearest point 
of termination of the auditory fibers is then destroyed, and this makes possi- 
ble the development of central cortical deafness. A. Pick has described two 
such cases in which the patients were so deaf that the almost total inability 
to understand words was only explained by the fact that extreme deafness 
from peripheral causes leads to absolute inability to comprehend words. At 
all events these cases do not show the clinical factors of subcortical sensory 
aphasia, or pure word deafness, and do not justify Pick's conclusion that pure 
word deafness is due to cortical destruction extending throughout both tem- 
poral lobes. A third case of Pick's x which presented besides extreme deaf- 
ness the essential characteristics of pure word deafness may be readily ex- 
plained as a combination of both these defects, which opinion was borne out 
by the autopsy findings. 

While we have so far especially considered those forms of aphasia which 
are generally recognized, either anatomically or clinically, such as cortical 
sensory and motor aphasia, or those which, as a rule, are regarded as special 
clinical types, such as the subcortical forms of aphasia, we now come to debata- 
ble forms which still require the support of additional observations and ana- 
tomical findings. From a purely logical standpoint we cannot reject the con- 
clusion that a transcortical motor and a transcortical sensory aphasia are 
possible ; i. e., an aphasia caused by an interruption of the tracts which bring 
about the combination of the two previously assumed speech centers with 
the so-called region of conception, therefore the greater part of the general 
surface of the cerebrum. The discussion of these clinical varieties is justi- 
fied by the fact that there are abundant clinical examples corresponding to 
the theoretically constructed picture. And although I attach great weight 
to the opinion of Dejerine, who declares these to be erected on a purely theo- 
retical foundation, I cannot recognize as conclusive the proofs he advances. 
I shall soon revert to this point. We shall first attempt to develop theoreti- 
cally the principal criteria of the clinical picture as has been done with the 
other forms. 

We must clearly understand what is meant by the conception of an object 
or "the concrete conception" (Ziehen), for, as already emphasized, the con- 
ception of an object must be awakened by a sensory speech or word sound 
center so that the word sense may be understood. Therefore a conception 

i Arch. f. Psych., XXIII, p. 909. 



280 THE SYMPTOM-COMPLEX OF APHASIA 

center has been spoken of; this would be identical with our center for the 
understanding of the word sense. Naturally it is pure fiction, and merely an 
artifice which diagrammatically simplifies the subject, to speak of such a 
center. In fact, in these processes which are extensively distributed through- 
out the regions of the cerebral cortex, we are dealing with conditions such 
as appear in the following considerations : If we take Meynert's point of view 
described at the beginning of this article in regard to the regions of special 
sense, we assume the conception of concrete objects to be the function of 
different projection fields at the cortex; for example, the conception of a rose 
is composed of " a tactile memory picture " or " a tactile picture " of the 
rose in the central projection field of the palpating surface of the hand, of 
an optical memory picture in the optical projection field, and a smell mem- 
ory picture in the olfactory projection field of the cerebral cortex. By the 
constant repetition of the same impressions of special sense so firm an asso- 
ciation of these memory pictures is formed that even the stimulation of one 
sense by the object is sufficient to remind us of the sum of its essential prop- 
erties; in other words, to awaken the conception of the object. Sometimes 
there are more, occasionally less, memory pictures of various region^ of special 
sense which correspond to a conception, but it is always a definite and firm 
grouping due to the nature of the object which forms the anatomical sub- 
stratum of every conception. This definite sum (which must always be de- 
termined) consisting of associated memory pictures, must " enter into con- 
sciousness," provided not only the sound of the corresponding word is 
appreciated but also that its sense is understood. In accordance with ana- 
tomical views we postulate also for this act an anatomical tract of fiber 
communication or an association tract between the sensory speech center, 
or center for the understanding of word sounds, and the projection fields 
in which the conception is produced. These tracts we may call transcortical, 
inasmuch as they extend beyond the nearest cortical termination of the audi- 
tory nerve in contrast with the generally recognized subcortical tract areas. 
As is obvious they can be considered only as a radiating bundle that fuses 
into one terminal point, the sensory speech centers, and only in this vicinity 
can it be focally separated. 

The condition must be quite similar if, spontaneously, i. e., in consequence 
of internal cerebral processes, the word " rose " is spoken. First the con- 
ception must appear, then the impulse of the movement conception corre- 
sponding to the word " rose " must reach the cortex of Broca's convolution. 
For this purpose the analogous " transcortical " tracts are necessary which 
can only be conceived in the form of a converging radiating bundle. The 
following diagram represents the relations of a " concrete conception " to both 
speech centers, 1 and also possesses acoustic factors as, for instance, a bell and 
a dog. 

i The definition of a concrete conception here given as the definite grouping of 
associated memory pictures with each other necessitates only the most necessary assump- 
tions. In my opinion it would be superfluous to assume anything more than this : 
That for association between the memory pictures of various projection fields special 
association centers are necessary which therefore might anatomically be considered as 
collective points of association tracts from the various projection fields of the organs 
of special sense. Flechsig claims that he has proven the existence of such association 



DISTURBANCES OF PHONETIC SPEECH 



281 



According to the preceding we have a perfectly clear definition of the 
clinical picture of transcortical sensory aphasia: An abolition of the under- 
standing of the word sense with a retained understanding of the word sound. 
The speech sounds as such are perfectly understood. This is shown by the 
retention of the ability to repeat them, but the accompanying conception is not 
awakened. The power of speech is actually retained but is slightly impaired 
in that spoken language is not understood and consequently its correct appre- 
ciation cannot be tested. xV paraphasia extending beyond this with an admix- 
ture of incorrect or distorted words or syllables is usually only suggested. 
The repetition may consist of entire sentences or may be limited to the last 
few words according to the degree of the retention of memory, and sometimes 
discloses no error; at other times there are paraphasic distortions. The 
repetition may be in obedience to command, sometimes it is involuntary. It 
may be combined with what has been heard in the form of a question, some- 
times it is spasmodic, sometimes almost reflex. In the last case we have 
echolalia, a symptom which usually indicates a weakening of the sensorium 
or general intellectual decay. The unintelligible repetition of a question is 
characteristic of this. 

The contrast in the motor realm is transcortical motor aphasia, consisting 
in the arrest or very decided impairment of spontaneous speech, while on repe- 
tition it is fluent, perfect, 
and there is no lack of 
understanding of lan- 
guage. The speech still 
at command is not made 
up of a few words or syl- 
lables constantly repeat- 
ed, as is the case in cor- 
tical motor aphasia, but 
there are occasional ex- 
pressions of discontent, 
of anger, of helplessness, 
therefore emotional ex- 
pressions ; the command 
of language, as may be 
proven by repetition, is 
unlimited, automatic. 
Series of sentences 
learned by rote, such as G ' 

the Lord's Prayer, the multiplication table, and the like, may be smoothly 
recited by the patient, either at command or after being repeated to him. 1 




centers or "coagitation centers" as he calls them. They are said to be characterized 
anatomically by the fact that no projection fibers or corona radiata fibers enter them. 
Since then it has been proven that corona radiata fibers extend to all coagitation cen- 
ters. I can only assume that the anatomical substratum for centers of this kind is in 
one cortical territory — that is, in the insula with its claustrum which Meynert regarded 
as an associated organ. 

i Compare Heilbronner, " Ueber die transcortical motorische Aphasie und die als 
■ Amnesie ' bezeichnete Sprachstorung." Arch. f. Psych., XXXIV, p. 341. 
20 



282 



THE SYMPTOM-COMPLEX OF APHASIA 



But the simplest communication, conversation, or difference of opinion, is 
impossible through speech; only in the rarest cases can questions be answered 
except by gestures; once in a while it is possible to obtain a single short 
answer. Articulation is absolutely faultless. 

These are the clinical types. They are complemented in some cases, i. e., 
in those with a fair degree of education, by the no less characteristic impair- 
ment of the power to write. Here also there is a striking contrast between 
the perception of speech and its expression: In sensory transcortical aphasia 
there is no comprehension of what is read, but, owing to the integrity of 
word conception, reading may be fluent and without visible effort; in other 
cases it is more or less paraphasia In motor transcortical aphasia spontaneous 
writing is impossible, while, in contrast to this, at dictation it is sometimes 
correct, at other times it is accompanied by many paraphasic distortions. 
These patients read silently with full understanding, but on reading aloud 
we perceive the influence of paraphasic admixtures. 

In the first case the reading may be compared to the reading of a lan- 
guage which is not understood but is composed of the same sounds, and the 
same comparison can be made with the difficulty on writing at dictation, 
for, while not faultless, it is relatively good. On the other hand, spon- 
taneous writing shows to an increased extent the disturbance (paraphasic) 
of active speech, therefore paraphasic distortions may render the speech utterly 
incomprehensible. It will have been observed that in, proceeding from the 
consideration of simple and associated facts to more complicated pictures, the 
significance of which may still be considered as disputed, we have left far 
behind us what Trousseau and Kussmaul in their time had considered to 
be strange and entirely incomprehensible observations. A more minute study 
of the impairment of the power to write may even throw light upon other 

related points, which as yet have been merely 
touched upon, and enable us to complete our 
clinical sketches. Before proceeding it is advis- 
able as an aid to the diagnosis briefly to recapit- 
ulate the essential factors in the various forms 
of aphasia which have been described. Here I 
follow Lichtheim who, in connecting a supposed 
conception center with the two speech centers, 
the motor and sensory, which I separated, was 
the first to rear a framework for the diagram- 
matic presentation of all aphasic symptoms. In 
the subjoined diagram (Fig. 138) B indicates 
the conception center, i. e., the localization of 
important memory pictures of a concrete object 
which, in fact, occupy very different cortical 
regions; but, as has been shown by their firm 
functional connection with a psychological unit, they produce harmony in the 
concrete conception. The sensory speech center is indicated by a, the motor 
speech center by b. 

The tract from a to b represents the association tract which serves for 
the repetition of speech sounds. The tracts a B and b B are those association 
tracts which serve to connect the concrete conception with both speech centers. 




Fig. 138. 



DISTURBANCES OF PHONETIC SPEECH 283 

In the center, a, b, is a centripetal and centrifugal tract; in other words, 
here the sensory and motor tracts enter parts of the projection system. The 
sensory speech tract contains at least a certain number of fibers of the audi- 
tory nerve of the same tone heights which were previously mentioned, and 
the motor speech tract contains the fibers running to the internal capsules 
(intended for the nuclei of the bulbar nerve) which function in speech. The 
diagram enables us to understand why the transcortical forms of aphasia are 
very rarely observed in their pure form; for a B and b B are, in fact, radiat- 
ing bundles only the terminal parts of which, a, b, are identical. The sepa- 
ration of these two tracts by a focus can therefore only occur in the most 
favorable eases in the immediate vicinity of both centers, a, b. If the numer- 
als in the diagram are chosen for illustration, 1-3 are the sensory forms, 4—6 
the motor forms of aphasia. According to the position of the cortical centers, 
a and b, we find upon each side a cortical, subcortical and transcortical form. 
If, for reasons previously mentioned, we disregard the form of conduction 
aphasia designated as 7, we must with Lichtheim admit that the mere occur- 
rence of the various forms of aphasia indicated by the diagram is a certain 
proof that these conditions actually occur in nature. But I can accept this 
proof only as positive in the case of the cortical and subcortical forms, 1 and 2 
and 4 and 5; in the case of the transcortical forms, 3 and 6, there is only 
some degree of probability. In fact the mere coincidence of such peculiar 
symptoms as mark the transcortical forms is difficult to explain. 

Objection has been made to the name chosen by me, especially for the 
transcortical forms, and, I must admit, with some degree of justice, for I 
find that its anatomical foundation is insufficient. But, in support of this 
nomenclature, I may state that I am aware of no other name which expresses 
the peculiarity of these cases. For the subcortical forms we possess very apt 
designations in the names pure word deafness and pure word mutism. But 
I see no particular reason for dropping an anatomical designation when, in 
my opinion, it has been deemed fitting. As to the designation transcortical, 
there are theoretic reasons for its retention to which I shall refer at the con- 
clusion of this article. 

The question of localization, that is, of the pathologico-anatomical find- 
ings, will later be considered in detail, but here I wish to call attention to 
certain decisive points of view. One of these has already been emphasized, 
for I have repeatedly referred to the general experience that pure cases are 
exceedingly rare. We cannot, therefore, expect that every case of aphasia will 
show that grouping of symptoms which is typical of the pure form which has 
been described. The second point, the possibility of grouping the correspond- 
ing anatomical findings of different clinical pictures, is no less important. 
Two such combinations must be mentioned, both of which appear as total 
aphasia, the one with inability to understand language, the other with loss 
of the power of speech, therefore as sensory plus motor aphasia; but they 
are unlike in their different response to the word conception, or so-called 
internal speech. The more common condition is associated with loss of inter- 
nal speech, therefore is the sum of the above described cortical forms. It 
is always associated with hemiplegia. The other form in which the power 
of internal speech is more or less perfectly retained is rare, but, nevertheless, 
has been observed; it is the combination of both transcortical forms of 



284 THE SYMPTOM-COMPLEX OF APHASIA 

aphasia, and occurs without hemiplegia. Furthermore, a glance at the dia- 
gram shows that combinations of subcortical and transcortical forms of both 
motor and sensory aphasia are favored by anatomical conditions, and are more 
readily produced by foci in Broca's convolution or the first temporal convolu- 
tion than by individual forms. Other combinations which must be regarded 
as natural groupings, and therefore somewhat more common, are met with 
under the explicit discussion of written language. 

The clinical facts enumerated, and the consequent guiding points of view, 
enable us more minutely to criticise our case of pure agraphia. In demon- 
strating the existence of a motor writing center, analogous to Broca's motor 
speech center, there must be a defect just as circumscribed as in Broca's 
aphasia, a loss of this faculty : " le souvenir du procede qu'il f aut suivre pour 
ecrire les mots." But internal speech, word conception, the " notion du mot " 
must be intact. For we do not write words but definite groups of letters, 
and this act, particularly the definite sequence of letters which form a word, 
presupposes an intact word conception. 

But in our case even the first requirement was not fulfilled. The patient 
could neither tell us how many syllables a word contained, nor could she select 
letters from tables of letters handed to her and form a word, not even when 
only three letters, a, n, d, were given her to form the word " and." And this 
is the almost invariable condition. Experience shows that agraphia, as under- 
stood at present, i. e., when not limited to one hand, is always accompanied 
by and is due to a disturbance of internal speech. More explicitly stated, 
it is a disturbance of the influence which the " word conception " has upon 
the choice of letters to form a word, and is a purely transcortical function. 



II. DISTURBANCES OF WRITTEN LANGUAGE AND GENERAL 
PATHOLOGY OF SPEECH DISTURBANCES 

That the disturbances of written language to which we now turn are of 
individual importance, and that they take place not only in connection with but 
independently of aphasic disturbances, proves their occurrence although they 
are rare, as is apparently isolated agraphia, with which we began our con- 
sideration. The contrast to this, isolated alexia, has also been observed with- 
out any other disturbance of speech, and so much more frequently that it 
obviously tempts us to assume a special center independent of the other func- 
tions of speech, or a cortical center for written language quite analogous in 
a motor and sensory respect to the true speech centers, of which the first is 
to be regarded as the seat of conceptions of writing movements, the second 
as the optical field for the memory of words. Charcot has been recognized 
as the most prominent exponent of this conception, which simply transfers 
the diagram of sound language to written language. However, the unten- 
ability of this gross diagrammatic consideration has gradually become obvious, 
and it has now but few adherents, existing and being widely distributed only 
in the consideration of a special and unilateral visual speech center in the 
angular convolution of the lower parietal lobe (Dejerine) ; even this v. Mona- 
kow denies. 

Correctly to appreciate the importance of written language as compared 



DISTURBANCES OF WRITTEN LANGUAGE 285 

with spoken language, we must remember first of all that the former is a 
comparatively late acquirement, and that it is not the common property of 
all persons as is spoken language. Because the possession of this faculty by 
different people is not invariable, it cannot be uniformly included in the 
mechanism of the brain, as is spoken language. Hieroglyphics, for example, 
presuppose a mechanical process in the brain different from that in the usual 
writing of individual letters among people more highly cultivated. The 
importance of the alphabet in written language is shown by the fact that 
reading is generally achieved by spelling. No matter how self-evident a sen- 
tence appears to us now, Grashey's investigations have shown that we learned 
to recognize it only by the previously described process. Grashey's successors, 
particularly Goldscheider, have found that this is not invariably the case, 
for combinations of even four letters are understood " at a glance," familiar 
w r ords as a whole, and independently of the letters which compose them, so 
that we must admit a faculty for optical word memory pictures commensurate 
with the individual vocabulary, either printed or written; usually the written 
picture of the name will be considered. In the course of our description 
we find another limitation. As a rule which applies to the majority of readers, 
we must regard spelling, reading and, consequently, optical memory pictures, 
as being only for letters and not for words, and comparatively few excep- 
tions to this can be admitted. When, in writing, we bring to remembrance 
the written picture of a word and for this purpose place letter after letter, 
it is evident from the process itself that we proceed by spelling. What w r e 
here produce is a manifold combination of letters similar to that we analyze 
by spelling when we read. Hence it follows that, aside from the previously 
mentioned exceptions, a direct relation of the conception of an object to writ- 
ten language must be denied. This point is so important that we shall sub- 
sequently discuss it more minutely. 

By what process internal thought is communicated by means of speech 
and written language is almost unknown to us. In many persons it is prob- 
ably and chiefly by means of sound pictures. That it may be by written 
pictures and not sound pictures appears absurd after our previous reason- 
ing; for we possess optical memory pictures merely of letters, not of words; 
certainly all the phenomena of reading and writing are explained by this sim- 
ple view ; should we proceed beyond the most obvious and most simple assump- 
tions, no one can tell to what extremes the pathology of the brain might lead 
us. The twenty-six letters of the alphabet are certainly not material for 
thought. Nevertheless, such an assumption is accepted in the differentiation 
by many psychologists and some pathologists, particularly Bastian, of three 
different formulas of thought, according to wdiich human beings are classi- 
fied as " moteurs, auditif s and visuels." The promulgator of this opinion 
was Charcot. He reported a case in which, during the act of speaking, there 
was a certain reading of the internal picture of the written word; far be it 
from me to attack his observation, although I have never seen anything simi- 
lar. But I regard such an occurrence as the rarest exception, and such a gen- 
eralization as not permissible. The possibility of its occurrence in deaf mutes 
does not disprove my theory, at least only in so far that by special training 
the brain may be enabled to perform such feats. But with most persons in 
possession of their faculties, there can be no question of such a training. 



286 THE SYMPTOM-COMPLEX OF APHASIA 

There is no doubt that intelligent deaf mutes may learn written language 
independently of speech. The combination of letters which form a word is 
for them an entirety, the separation of the word into letters is a later and 
artificial acquirement, analogous to the phonetic spelling of a word. If we 
admit that some persons usually think 'in word (of course not in letter) 
sounds, therefore, in deaf mutes this will appear in a corresponding optical 
combination of letters each of which in like manner is associated with a cor- 
responding concrete conception, or is united by transcortical tracts with the 
corresponding pictures of word memory, as was previously explained (Fig. 
137). In deaf mutes, therefore, we may distinguish between optical word im- 
ages and images of letters. This particular class of unfortunates must here be 
omitted from consideration, since they require special and precise investiga- 
tion. In all other persons the brain would be over-burdened if it retained 
all combinations of letters as special optical memory pictures of words, the 
corresponding word sound pictures being already present, and we must there- 
fore assume that this is not the case. It is positively disproven by the fact 
that in rare cases (Rieger and Sommer) a permanent loss of power to use 
certain letters of the alphabet in writing has been observed, the patients hav- 
ing no conception of the form of certain letters, yet there was no correspond- 
ing speech disturbance affecting the sounds represented by these same letters. 
In Rieger's x celebrated case this was true of three letters of German text, 
also of seven small and fourteen capital Roman letters, the identical letters 
in both the German and Roman alphabets. These cases are further remark- 
able from the fact that aphasic symptoms were merely indicated; they are 
the purest cases of disturbance of the function of written language alone 
which have yet been reported. 

It is quite remarkable that Sommer, 2 who published one such case, 
having investigated it by Rieger's method, comes to the conclusion of an 
immediate connection between object conceptions and writing movement con- 
ceptions. In Grashey's case he believes this explanation to be absolutely neces- 
sary. Here he goes a step further than the adherents of a substantive optical 
word center, some of whom (Dejerine, for instance) deny a motor writing 
center. His hypercritical tendency and his apparently unprejudicial stand- 
point here become apparent; for in reality there could scarcely be more 
positive proof of the connection of an optical word picture with its com- 
ponents, the pictures of letters, than the way in which Grashey's case found 
his words, and the explanations which we owe to the subsequent investiga- 
tion of this case by Sommer 3 and Wolff. 4 

Grashey's patient showed the peculiarity that he could only find words for objects 
shown him, and, as was observed later, only for objects and their properties brought 
within his psychical conception, by writing. Without this artifice it was impossible 
for him to express himself in words, but after he had finished writing the word he was 
able to speak it; if he was disturbed while doing this, each part of it remained discon- 

i " Beschreibung der Intelligenzstorungen infolge einer Hirnverletzung." S. A. aus 
Verhandl. der Phys. med. Gesellschaft zu Wiirzburg. N. F. 22. u. 23. Bd. 

2 Sommer, " Zur Theorie der cerebralen Schreib- und Lesestorungen." Zeitschr. f. 
Psych, u. Phys. d. Sinnesorgane. V, p. 305. 

3 Centralbl. f. Nervenheilk. u. Psych. Marzheft, 1894. 

* Gustav Wolff, " Ueber krankhafte Dissociation der Vorstellungen." Eabilitation- 
schrift, Leipzig, 1897. 



DISTURBANCES OF WRITTEN LANGUAGE 287 

nected from the object and useless. But only the complete word bore any resemblance 
to the word conception of spoken language which corresponds with the concrete object; 
parts of it, letters, a combination of letters, or even an unfinished word, have no con- 
nection with the object that is seen, or felt, etc. 

Grashey's case is an example of the possibility mentioned above of special training 
of the brain. Here the choice of the word independent of the object is impossible, a 
defect which we will consider later. To compensate for this defect he resorts to the 
artifice of utilizing written language, as, for instance, a deaf mute would, but, unlike 
the deaf mute, he must proceed by spelling. His writing is only a result of the intensely 
stimulated optical memory pictures of letters in which the motor components of " con- 
ception of direction " are the main thing, as we shall see later. 

In one respect I agree with Sommer : The defect in Grashey's case cannot be 
explained by assuming a loss or diminution of memory for recent impressions. Its vast 
importance for the conception of written language and its relation to spoken language 
have not been shattered by subsequent investigations, but have rather been strengthened. 

After these preliminary remarks I consider myself justified in assuming 
the same cause for disturbances of written speech as those contained in my 
report of 1886, to which I refer the reader for details. 1 

Ziehen's " concrete conception," as well as all abstract conceptions and, 
as we shall see, the entire material of thought, has no immediate connection 
with written language, being essentially a language of letters communicated 
only through the centers for speech. Therefore, reading and writing are 
transcortical subordinated activities from the centers of spoken language, 
and nothing more positively proves the necessity of separating focal symp- 
toms with a transcortical seat than the fact that there are isolated disturbances 
of written language due to a focal disease of a definite area. 

Hence the faculty of writing depends upon spoken language, and is lost 
as soon as the word conception or internal speech is damaged; it is retained, 
and furnishes a valuable criterion, when word conception and internal speech 
remain uninjured. 

Integrity of the power of articulation is an important sign in subcortical 
motor aphasia or pure word mutism, as well as in subcortical sensory aphasia 
or pure word deafness. In the transcortical disturbances of speech, the intact- 
ness of the word conception is also evident from the fact that purely mechan- 
ical reading and writing reveal no disturbance. The patient may read aloud 
either at dictation or from copy. Some individual points in the symptom- 
complex of written language are not yet sufficiently proven. As a rule, we 
may maintain that a defective power to write is analogous to defective speech, 
for with the loss of the power of speech the comprehension of what is read 
is also lost, with the loss of the spontaneous power of speech, the faculty of 
spontaneous writing also disappears; in reading aloud and in writing at dic- 
tation paraphasic admixtures and distortions are as numerous as on repeti- 
tion. This is particularly true of transcortical sensory aphasia, provided that 
the paraphasic symptoms are involuntarily increased by the act of writing 
so that writing becomes even more difficult, In the discussion of such cases, 
the terms paralexia and paragraphia are objectionable, and the expression 
paraphasic disturbances in reading and writing is preferable. In transcortical 
motor aphasia, it is evident that spoken language is characterized by an 
absence of spontaneity, and this becomes more marked on trying to write. 
It also appears in writing at dictation, for this form of writing, although 

iFortschr. d. Med., IV, pp. 371 u. 463. 



288 THE SYMPTOM-COMPLEX OF APHASIA 

possibly to a less extent, presupposes the spontaneous coaction of the writer. 
" Word finding " for the act of spontaneous speech, the actual function of 
the tract B b, necessitates for writing the further exercise of finding the 
letters, as the sounds of the letters, in our sense, belong to the word conception. 

The power of writing, as previously stated, is most severely damaged in 
the cortical forms of aphasia, for here the word conception itself sustains 
a lesion. Cortical motor aphasia, as a direct focal disease, usually results 
in just as persistent an alexia and agraphia. But the letters continue to be 
optical structures, and may be written individually, as is proven by the reten- 
tion of the ability to copy. For reasons to be subsequently more minutely con- 
sidered, in an intact brain which still responds, there is a greater probability 
of the restoration of the receptive faculty of reading than of the expressive 
faculty of writing. The sequence in which reading returns after cortical 
motor aphasia has been studied by Thomas and Eoux under Dejerine's direc- 
tion ; the inverse relation of learning to read was revealed by the circumstance 
that at first entire words, then syllables, finally the individual letters which 
compose a word, were read with confidence. Besides mutism, isolated or 
very marked agraphia may persist for years; an agraphia, however, which 
is characterized by the retention of the power to copy as well as to change 
letters from one form into another form, and this power prevents confusion 
with the substantive agraphia of our case detailed at the beginning of this 
article. Generally the ability to write improves just as does articulate speech; 
it is slower at dictation than on spontaneous writing (Dejerine). In writing, 
the integrity of the word conception is absolutely necessary ; on this point 
I agree with Dejerine and differ with v. Monakow. My experience does not 
permit me to coincide with the observation of v. Monakow that motor aphasics 
often find written expression easier than speech. This probably refers to cases 
of predominant subcortical motor aphasia, for I believe that v. Monakow's 
clinical picture of cortical motor aphasia has been based on too broad a con- 
ception; he recognizes a partial motor aphasia of much wider extent than 
I do. At all events there are exceptional cases — a case of Banti's is a proof 
of this — in which cortical motor aphasia leaves intact the faculty of written 
expression as well as the understanding of written expression. Monakow 
assumes that this can be due only to a partial disease of Broca's convolution, 
a condition to which we shall refer when describing the pathology. 

Sensory aphasia of cortical origin is generally admitted to occur without 
severely damaging the power to write. On the restoration of the power of 
speech the art of reading is sooner learned than that of writing; in a word, 
according to universal experience, agraphia usually persists as a severe per- 
manent disturbance. This corresponds with the fact that we attach the 
highest value to the acoustic components of the word conception during the 
time of its attainment. Perhaps, however, purely anatomical conditions are 
not unimportant, since the posterior and upper end of the first temporal 
convolution almost forms an important association tract for the motor act 
of writing. 

Moreover, there is a lack of casuistic material consisting of well observed 
cases of inability to write to show that in disturbances of speech the symp- 
toms disappear during the stage of improvement. 

The relation of written to spoken language enables us to appreciate in 



DISTURBANCES OF WRITTEN LANGUAGE 289 

a double sense the clinical symptoms of alexia, agraphia, paralexia and para- 
graphia whether they are the sequels of a damaged word conception or whether 
they appear independently. The former may be designated verbal, the second 
literal. The substantive disturbances of written language, which are observed 
with intact word conception or internal speech, relate also to the form of 
letters so that, for example, literal agraphia consists mainly in the fact that 
the reproduction of this form is impossible for the patient, and the case whose 
history was given in the beginning of the article is a marked example. In 
literal alexia the actual defect consists in a non-recognition of letters, and 
in consequence of this of words also. A special form of alexia and agraphia 
is produced, as we shall soon see, by a damage of the tracts which connect 
the word conception with the psychical elements represented by letters. 

It would lead us too far from our theme and would not be in consonance 
with its importance here to trace diagrammatically all the true disturbances 
of written language, as was done for those of speech. But in regard to the 
form which such a diagram must take, if it is to correspond with the major- 
ity of the facts, the most important points may be indicated. It generally 
corresponds to that developed for speech (Fig. 138) if, in place of the con- 
ception center, B, we use the word conception, c, in place of the motor speech 
center, we use the conception of writing movements, /3, and in place of the 
sound pictures we substitute the optical memory pictures of letters, a. While, 
however, in the diagram of speech processes we must adhere to the fact that 
a direct tract, B, b, exists, it is very unlikely that a corresponding tract, c /3, 
comes into question in written language. The diagram thus becomes more 
simple than for speech. It appears that in writing a path over the optical 
field of memory, a, is absolutely necessary, probably because of the method 
most often used in learning to write. I do not doubt that a change in this 
method might influence the clinical importance of this tract. Thus, the 
older among us, without exception, first learned to read letters and then 
to write them. Xow these arts appear to go hand in hand, so that children 
are first taught not onlv to read and then to write, but are taught to read 
while learning to write. I nder this method the motor components in the 
production of a letter gain an increase in dignity. 

On the other hand the difference between printed and written letters 
teaches us that the motor components of the conception of writing move- 
ments, at least in recognizing a letter, are indispensable, so that the concep- 
tion of writing movements of the letters cannot be regarded as an essential 
factor of this. 

In employing the expression, " conceptions of writing movements," I desire 
not to be misunderstood. The term signifies only that conceptions of move- 
ments, in Meynert's sense, produce writing movements just as, in quite a 
different muscular area, they produce speech movements. A certain recol- 
lection of the process to be followed in writing is therefore admissible and 
cannot be denied; neither can we deny a certain localization of this special 
function of memory in the left arm region, provided we write with the right 
hand. But it will be the same locality in which originate all the finer move- 
ments of the right hand which are not specially localized, but serve this pur- 
pose, as in conceptions of speech movement. " L'ecriture n'est qu'une des 
formes de motilite de la main" (Dejerine). When writing is done with the 



290 THE SYMPTOM-COMPLEX OF APHASIA 

left hand, the same, although less common conception of movements, is local- 
ized in the right arm region, and we localize these in the right leg region 
when we write in the sand with the left foot. These are the conceptions of 
movement described by Storch, which in writing, as in every motor act, dic- 
tate the movements of a certain portion of the body, because we have in the 
optical fields for memory of letters a special depot and a complete diagram 
of the conception of those movements of direction involved in writing. There 
are persons who can produce writing movements with their tongues. Exner's 
theory of a special motor center for writing at the base of the second frontal 
convolution therefore seems scarcely plausible, to say nothing of the fact that 
it was based on an uncritical utilization of doubtful casuistic material, and 
has not since been supported by pathologico-anatomical findings. An absence 
of these conceptions of movement, analogous to Broca's aphasia, must there- 
fore be assumed in all cases of paralysis of the right hand from a left-sided 
cortical focus. The general incapacity of the right hand includes actual 
inability to write, while the retained motility of the left hand permits writ- 
ing, and proves the continuance of its necessary conceptions of direction. 
Such a unilateral agraphia can under no circumstances be synonymous with 
Broca's aphasia. This was probably present in Pitres' case in which there 
was a cortical lesion, and, in spite of recovery of the finer motility of the 
right hand, an exclusively right-sided agraphia was permanent. I shall later 
discuss the explanation of this extraordinary and instructive case. 

There is no unanimity of opinion among authorities as to the existence of 
a so-called optical word center, actually an optical letter center. This mooted 
question from a purely practical and clinical standpoint, might well be left 
out of consideration, and we might be content with recognizing the insig- 
nificance of a circumscribed unilateral lesion in the posterior lower area of 
the lower parietal lobes in the sense of localization, an area which is important 
as the seat of origin of what Naunyn, upon the basis of statistics, called indefi- 
nite aphasia. Here the decisive point which belongs among the most positive 
facts of cerebral localization, is that a unilateral focus deeply situated in 
the medullary structure of the parietal lobe produces a combination of right- 
sided hemianopsia and isolated writing blindness or alexia as persistent symp- 
toms. If the affection is disseminated and reaches the cortex of this portion 
of the brain, agraphia is added to. alexia (Dejerine). 

If these facts are grouped and explained like those of spoken language 
in our diagram, the cortex of this portion of the brain would be considered 
the point of optical " alphabet memory pictures," and pure writing blindness 
as subcortical alexia analogous to subcortical sensory aphasia. It is self- 
evident that experience alone can determine whether or not we will accept 
this conclusion, and theoretic considerations must be held in abeyance. It 
may even be admitted that there are theoretic grounds for the assumption 
of such a center. The principle of conservation of energy appears to favor 
the unilateral condition, the especial peculiarity, which characterizes the mem- 
ory pictures of letters of the alphabet above all other optical memory pictures 
as a corresponding special localization; I shall revert to this later. Finally, 
it must be observed that this is the standpoint of such competent authorities 
as Bastian, Dejerine and A. Pick. Nevertheless, I cannot agree with them 
without sacrificing the theory of the internal connection and the fundamental 



DISTURBANCES OF WRITTEN LANGUAGE 291 

principles for the understanding of the structure of the brain which were 
taught with such conspicuous success by Meynert. This is also v. Monakow's 
view; he doubts the existence of a true optical word center, and maintains 
that optical memory pictures of letters of the alphabet are double. I had 
previously expressed this opinion and had declared myself opposed to the 
localization of the so-called optical pictures of word memory. Since a posi- 
tive theory of localization, if erroneously taught, may imperil the advance 
of the law of localization, as we have seen in the case of Broca's aphemia, I 
feel forced to explain somewhat more minutely the untenability of the assump- 
tion of an optical word center in the cortex of the gyrus angularis. 

Theoretically all that we know of the nature of optical memory pictures 
is opposed to such a narrowly limited localization of alphabet memory pictures, 
particularly to a unilateral one. More recent investigations have clearly 
shown that the memory pictures of the special senses have motor components 
which from their nature are inseparable. Storch has designated optical mem- 
ory pictures by the appropriate term, direction conceptions. Such concep- 
tions of direction also apply to all motor projection fields, but nowhere so 
clearly as in the optico-oculomotor projection field which, in contrast to the 
true field of light (H. Sachs), includes the convex surface of the occipital 
lobe and the gyrus angularis of the lower parietal lobe. For example, such 
conceptions of direction, in so far as they relate to the arm and hand, are 
localized in the so-called middle third of the central convolution. As shown 
above, they coincide with conceptions of writing movements. Now the optical 
memory pictures of letters, like those of figures or any other objects which 
have but two dimensions, differ from concrete things by the fact that to 
the sense of sight they each have but one form ( Storch x ) while all other 
concrete objects possess innumerable ones. Their limited number, and the 
exceedingly frequent use which we make of them, will cause them (accord- 
ing to the principle of well grounded tracts) to appear as a particularly firm, 
and consequently easily utilized, possession in pictures of memory. Finally, 
optical memory pictures of letters are characterized, as I previously attempted 
to explain, by the fact that they bear no direct relation to concrete concep- 
tions. Except a single tract which is the most marked, hence the most easily 
understood tract, they have no communication with a unilateral speech region 
nor, we may presume, primarily with the acoustic sound center. These three 
properties which they possess in a greater degree than any other optical mem- 
ory pictures, give us no reason to assume a specially circumscribed localization, 
except that the point of most distinct sight and of the finest differential oculo- 
motor conceptions of direction has a special localization in the cortex which 
has not yet been proven. Only one of these properties, namely, exclusive 
association with the left temporal lobe, is a factor important for the unilateral 
nature of the alphabet memory pictures. But to this I shall refer later. If 
we consider the functional acquirement of memory pictures of letters of the 
alphabet, it does not favor the predominance of the left hemisphere. In 
typical hemianopsia the form of the field of vision permits us to conclude that 
the immediate surroundings of the point of fixation are almost always bilat- 

i Details in an article by Storch : " Versuch einer psycho-physiologischen Darstel- 
lung des Bewusstseins." Berlin, 1902. 



292 



THE SYMPTOM-COMPLEX OF APHASIA 



eral, and supplied by each optic tract. Usually the picture of a letter is 
formed exclusively within this central area of sight so that its memory 
picture is formed in each hemisphere. If the letter is large, the glance will 
wander as with every large object, but central sight will always be exclusively 
implicated. 

We might believe that the possible unilateral nature of optical memory 
pictures of letters might be directly determined by ascertaining whether there 
is a difference in the peripheral portion of the field of vision, and whether 
the patient reads with the right or the left half of the visual field. This 
theory, however, is shattered by the fact that even the largest letters can be 
read only in the immediate vicinity of the point of fixation, while beyond 
this it is impossible to say with certainty whether or not there are any letters. 
It is usually maintained that central acuity of vision rapidly declines out- 
wardly from the point of fixation. How rapidly this becomes apparent on 
an attempt to recognize letters, and there is no difference between the right 
and left halves of the field of vision. But certain facts in the pathology of 
the brain are much more important than these academic considerations. Above 
all, we can form no conception of the combination of isolated word blindness 
with right-sided hemianopsia in the sense that hemianopsia, i. e., the functional 
interruption of the subcortical tracts which substitute for the left cortical 
tract, is to be regarded as the cause of word blindness, as the acoustic tract 
which terminates in the left temporal lobe is the cause of subcortical sensory 
aphasia. On the contrary, the majority of cases of typical right-sided hemi- 
plegia show no trace of word blindness or alexia; at most, reading is made 
difficult by the hemiopic defect itself, from the fact that the patient cannot 
see the letters or words immediately following, but every letter or syllable 
is recognized unless there is an impairment of memory as in a case of Bed- 
lich's. It is noteworthy that with the sudden appearance of hemianopsia 
this defect is soon recognized, therefore reading need not be even temporarily 
prevented by the hemiopic defect. In all of these cases we must assume that 
the patients depend exclusively upon the right hemisphere in reading. In 
occlusion of the tractus opticus the left hemisphere must to a certain extent 
be regarded as blind. If, in spite of this, the patient recognizes letters and 
their sequence it must be because of the termination of the optic tract in the 
cortex of the right hemisphere. This conclusion is so obvious, and so con- 
vincingly disproves the theory of the unilateral nature of the so-called optical 
word center in the left hemisphere, that its exponents, particularly Dejerine 
and Bastian, were forced to consider it. They attempted to nullify its im- 
portance by assuming that fibers of the corpus callosum, which pass from 
the optical projection field of the cortex of the right hemisphere to the sym- 
metrical cortical fields of the left hemisphere, were still able to reach the 
optical word center upon the left side. For this purpose they differentiated 
between a general visual center and a special optical word center : The for- 
mer is bilateral, the latter unilateral, and present only upon the left side. In 
the same way they differentiated a general center of hearing in both tem- 
poral lobes, and only a left unilateral and special center for the understand- 
ing of word sounds. I believe this entire reasoning to be erroneous, as it 
controverts the views of the pioneer Meynert, and ascribes functions to the 
fibers of the corpus callosum, which are merely a continuation of the fibers 



DISTURBANCES OF WRITTEN LANGUAGE 293 

of the tract of the optic nerve, or of the auditory nerve, beyond the cortical 
projection field into the opposite hemisphere. Were these the true conditions, 
the hemiopic defect in a unilateral lesion of the optic tract would eventually 
be compensated for by the other hemisphere, and no longer be noticeable. 
Finally, I must refer to the clinical condition known as soul blindness, which 
frequently accompanies word blindness or literal alexia. 

If pathology thus disproves the unilateral and narrowly circumscribed 
localization of an optical word center, all the more necessary is it specially 
to review the circumstances which produce this unilateral and circumscribed 
condition. Apparently it is the relation brought about by association fibers 
between the unilateral speech region and the optical memory pictures of let- 
ters, and chiefly of their acoustic components which are calculated to simulate 
this unilateral condition. Here two possibilities become obvious, but further 
investigation by necropsies will alone clear the situation. One view, accepted 
by Monakow, is that foci in the gyrus angularis, besides interrupting the optic 
radiation of Gratiolet, have also implicated the crossed optic-acoustic com- 
missure (Bastian), i.e., the association fibers between the left temporal lobe 
and the right optical oculomotor projection field. The optical memory pictures 
of the left hemisphere are then obliterated by the interruption of the optic 
radiation of Gratiolet (hemianopsia). The optic memory pictures in the 
right hemisphere might yet be stimulated by means of the right optic tract, 
but could not be utilized in reading because they could no longer evoke by 
means of the previously mentioned commissures the absolutely necessary acous- 
tic or word sound constituents of the letters. In other words, the power to 
form words from the individual letters would be lacking, and reading by 
spelling the words would be quite impossible. As an exception to the rule 
of reading by spelling, reading would be limited to the few words which the 
patient had previously learned to recognize. The majority of patients expe- 
rience great difficulty in naming objects shown them, and this is usually 
observed in cases of word blindness ; it is ascribed to a lesion of the communi- 
cating tract which leads to the left temporal lobe, but incorrectly so, as we 
shall soon see. Moreover, this does not explain the fact that letters, especially, 
are seen but not identified, all other objects being clearly recognized. We 
have seen that other relations than those of letters to the acoustic projection 
fields do not exist. It is a question, too, how in word blindness thus pro- 
duced the form of the letters themselves can be recognized, copying being 
done correctly, and also how it is that the same letters in different arrange- 
ment appear identical, being transferred from one arrangement to the other, 
the purely optical components remaining undamaged. For some of the cases 
so far observed this expectation has not been realized ; on the contrary, a most 
conspicuous feature of these cases is that the patients do not actually write 
the letter of the alphabet while anxiously following the copy provided, but 
are only able to draw it. If we have ever noted how these patients laboriously 
draw each letter, and how they find this equally difficult whether the copy 
be written or printed, we will scarcely search for the cause of this defect in 
the interruption of the decussated optico-auditory commissure. 

Of course we must bear in mind that the optical memory pictures of letters 
represent complexities of form conceptions, and these attain definite impor- 
tance only from the fact that they are associated with the word conceptions 



294 THE SYMPTOM-COMPLEX OF APHASIA 

of the letters belonging thereto. Articulate speech is composed of word con- 
ceptions, i. e., it represents the combination of a sound picture and a definite 
conception of speech movement, and these bear to the optical memory pictures 
the relation above described, and when this relation ceases the latter at once 
deteriorate into meaningless signs. This may explain why the identity of 
a printed and a written letter, or of a Latin and a German word, is no longer 
recognized if the sound which produces the relation can no longer be inter- 
nally produced. It is even questionable whether the pure optical components 
of letters, detached from their sounds, will permit us to recognize that we 
are dealing with the letters of the alphabet. 

If we transfer this first possibility to the diagram it would correspond to 
a combination of subcortical and transcortical symptoms. The unilateral 
affection of the tract would be subcortical, and the interrupted continuity of 
the crossed optico-acoustic commissure, transcortical. Alexia would arise be- 
cause only one of the tracts, c and a, the crossed and the uncrossed, would 
be interrupted, but the other, in consequence of the interruption of its sub- 
cortical tract, would be useless. On the other hand the unimpaired, uncrossed 
tract c a would be amply sufficient for the power to write as before. 

The second possibility is that which simultaneously assigns to the left 
optico-oculomotor projection field a predominant and indispensable role by 
permitting an ability to read; this is quite in contrast to the first theory, 
according to which the left-sided projection field appears to be especially 
excluded. According to the principle of the conservation of energy it permits 
the view that only the optical memory pictures of letters in the left side are 
united to the acoustic projection field by a well beaten tract; that, therefore, 
there exists only one uncrossed acoustico-optico commissure or tract c a. This 
would lead to the further assumption that the connecting link, c and a, neces- 
sary for reading, must always follow a circuitous route over the left optico- 
oculomotor projection field even when, in consequence of right-sided hemi- 
anopsia, reading is accomplished solely by means of the right hemisphere. 
The interruption of communication which alexia causes must be sought in 
the commissure between the two optico-oculomotor projection fields, particu- 
larly between the oculomotor projection fields, a view which in the main 
coincides with that of Bastian and Dejerine. The only difference would be 
that the memory pictures, not the point of perception, are united by fibers 
of the corpus callosum. I have elsewhere expressed myself concerning this 
possibility. 1 Transferred to the diagram this interruption would not affect 
the tract, c a; therefore in regard to a, it would be of double subcortical 
importance; first, on account of the tract fibers of the same side, and sec- 
ond, on account of its detachment from the optical memory pictures of 
the right side which are still susceptible to light. From this point of view 
the power to write which emanates chiefly from the left center, a, would, 
because of the integrity of the tract, c a ft, show no disturbance. 

The question of the unilateral origin of an optical word or letter center 
is evidently of great importance for the entire law of localization. If we 
deny it, as in my opinion we must, there is still some hope of a pathology 
of the fibers of the corpus callosum; for it would be comparatively easy to 

iL. c. 



DISTURBANCES OF WRITTEN LANGUAGE 295 

decide which part of the corpus callosum must be implicated in order to pro- 
duce the symptoms of subcortical alexia or pure word blindness. 

I must admit also that our case of apparently isolated and literal agraphia 
described at the beginning of this article cannot be better explained than by 
adopting the view of the unilateral location of the center, a. The tract, a ft, 
would then be interrupted at a point transcortically beyond a; the assumption 
of two tracts, namely, one upon the same side and one upon the crossed arm 
region, makes this the more plausible because they have the same point of 
origin in a. Nevertheless, as we shall soon see, the apparently natural and 
obvious explanation is in absolute opposition to other experiences. In con- 
trast to the view of a special position for the letter center among the optical 
pictures of memory, this case exhibits a singular feature since the patient, 
besides having lost the faculty of writing, had also lost the power of drawing 
the simplest figure. 

The clinical picture of pure word blindness, more correctly letter blind- 
ness, or, in accordance with my suggestion, subcortical alexia, is based upon 
quite a number of cases ; Redlich * has collected from literature no less than 
twenty-seven pure cases and, as has already been stated, there is no lack of 
anatomical foundation for this view. The chief symptom of the clinical pic- 
ture may be delineated by a case which is quite typical. 

L., a plasterer, aged GO, understood questions I addressed to him, and answered 
them all correctly. He was intelligent and showed no defect in speech. When asked 
to write something at dictation he did it correctly and uninterruptedly, and also wrote 
down any figure required of him. At my request he wrote a letter to his married daugh- 
ter; although there were errors in spelling and the alignment of the writing was poor, 
and although there were breaks and repetitions as from uncertainty of motive, the 
letter was upon the whole correct and comprehensive; had he been a man of education 
it would probably have been perfectly correct. The man was then asked to read what 
he had written, and the surprising fact was reveaied that he could not read a word, 
not even a letter or a figure. 

The same condition is found in all analogous cases, provided they are as 
well marked as that of my patient. The cases differ only in that figures are 
often clearly recognized. We learn from this case that the difficulty is not 
due to visual disturbance, for the patient said his sight was good, which was 
also proven by the fact that he could copy letters and words which he did 
not know, of course in a mechanical way, copying character for character like 
entirely unfamiliar figures. Hence he did not understand what he had writ- 
ten, and it has been repeatedly observed in analogous cases that by this cir- 
cuitous method the patients are able to decipher what has been written, 
although slowly and with great difficulty. The investigation of the acuteness 
of vision proves that this is quite sufficient for the recognition of letters. 
Nevertheless, this patient's sight was defective; this was evident from the 
peculiar position of his head while writing, from the running together of the 
words, and the defective alignment. That is. he had a typical right-sided 
hemianopsia, with vertical separation of the halves of the field of vision into 
the familiar form in which a narrow portion, passing over to the right, still 

i Redlich, " Ueber die sog. subcortical Alesie. Jahrb. f. Psych., Bd. XIII, 2 und 
3 Heft. 



296 THE SYMPTOM-COMPLEX OF APHASIA 

retains the faculty of sight. Bilaterally the acuity of vision was at least 
one-half of the normal; the eye-ground was absolutely normal. This defect 
manifested itself by making fluent reading impossible, because there was no 
view of the following letters, and in our case the only letter seen in totality 
was not recognized. By showing objects we convinced ourselves that the 
patient's sight was sufficient; he was able to recognize any object. But a 
phenomenon is frequently observed in analogous cases, and is sometimes more 
highly developed than in this case: Difficulty in properly naming an object. 
In our patient this occurred only now and then. Expressions of embarrass- 
ment or descriptive terms were first uttered; finally, on being prompted, the 
correct word was eagerly accepted. In other cases not a single object shown 
can be correctly designated, and this was the case with a patient that I saw 
about 25 years ago in the Charite. It was the only symptom which indicated 
aphasia. In spontaneous speech, words are not lacking, not even those for 
visible objects. When, on the other hand, this symptom is more conspicuous, 
as, for example, in my earlier case, there is more or less difficulty in finding 
words for concrete objects, even in spontaneous speech, but, as a rule, this is not 
so difficult as naming objects which are shown. 

During the first period of observation another phenomenon appeared which 
is apparently of much more serious import than the mere difficulty of finding 
words. The patient for a time was absolutely unable to recognize objects that 
he saw, although he perceived them distinctly; he therefore presented the 
well known symptom of mind blindness. This condition is not the result of 
accident, but is frequently observed in cases of so-called pure word blindness. 
Deducting conclusions from the course of our case, we recognize an increase 
of this defect in alexia, and in our case an apparently secondary effect depend- 
ing upon an indirect focal symptom of the lesion producing alexia. In regard 
to the simultaneous occurrence of these two phenomena, experience teaches 
that mind blindness usually leads also to word blindness, but isolated writing 
or word blindness frequently occurs without mind blindness. In the first 
case I observed there was no indication of mind blindness. In the case just 
considered it was remarkable that during the time of soul blindness the patient 
was unable to recognize objects even by touch. Mind blindness, therefore, 
existed even to the extent of asymbolia, the most severe sensory defect which 
can be referred to focal disease. Concerning the history of our case, its course 
so far and experience in similar cases permit the conclusion that we are deal- 
ing with a stationary condition in which a possible improvement may be hoped 
for. While there is no present sign of hemiplegia there were symptoms of 
this six months ago, but these completely disappeared within two to three 
weeks, a fact which indicates that embolic processes were present. The 
symptom-complex now existing attracted attention because the patient was 
no longer able to read his newspaper as usual. We are justified in attribut- 
ing this to embolism and to resulting softening. 1 The probable locality of 
the pathologic focus will be subsequently pointed out. 

Besides the typical pathologic picture which our case presents, no doubt 
there are other isolated disturbances of the. faculty of reading which are of 

i E. Storch, " Zwei Fiille von reiner Alexie." Monatschr. f. Psych, u. 'Neurol., 
XIII, Erganzungsheft. 



DISTURBANCES OF WRITTEN LANGUAGE 297 

different nature; for example, the dyslexia first described by Berlin/ a partial 
inability to read which manifested itself subjectively, and soon increased to 
complete inability to read; after a little rest the patient was able to go on 
reading but it was with recurring signs of exhaustion which rapidly increased. 
Here the feeling of disinclination was remarkable ("fear of reading/'' 
Bruns). We cannot refrain from assuming that this is a functional affection 
of the same or similar origin as is pure word deafness, and the suspicion of 
an organic foundation, no matter of what nature, must be borne in mind all 
the more as Berlin's experience led him to emphasize the unfavorable prog- 
nostic significance of this symptom which usually appears suddenly. Hemi- 
opia may be present but it is not a requisite as in alexia. Most of the cases 
succumbed in a few jears from serious affections of the brain. Atheromatous 
and syphilitic disease of the cerebral arteries were repeatedly found. 

As we have seen, isolated word blindness is a combination of various con- 
ditions, usually of the interruption of a subcortical tract and the integrity 
of a transcortical tract. From the well known location of the pathologic focus 
which corresponds to these requirements, deep in the medulla of the gyrus 
angularis, we may conclude that the tract of the cortical surface of the gyrus 
angularis, which has remained intact, must lie nearer than the destroyed one. 
Therefore, the fact mentioned above (which was utilized by Dejerine in the 
consideration of a center of optic word memory pictures), that widespread 
destruction in this region produces literal agraphia as well as literal alexia, 
entirely destroys the perception of written language, also the fact that if a 
disease at the cortex be added to a disease of the deep medullary substance, 
agraphia is added to the previously isolated word blindness. According to 
our diagram, agraphia and alexia are produced by the interruption of the 
tract, c a, that tract which forms the necessary power for all perception of 
written language. As I have reiterated, this power most likely exists in nature 
in two entirely different tracts whose anatomical relations we have yet to dis- 
cuss. This may explain why their common division causes an exclusive arrest 
of the function of written language without any implication of speech, a 
condition so far very rarely observed; we shall see that the two cases of 
Rieger and Sommer are the only examples which approach this. It would, 
however, be a somewhat common occurrence if the cortex of the gyrus angularis 
were actually an optic word center, as Dejerine maintains. It is evident from 
the diagram, which illustrates Dejerine's views of the extension of the speech 
region, that this author ascribes an influence to spoken language, to the so- 
called optical word center, similar to that of the acoustic word center. This 
opinion we cannot accept ; it is opposed to our theory developed above that 
in persons mentally sound letters, not words, form optical memory pictures. 

As already indicated the coincidence of literal alexia and agraphia with- 
out essential affection of speech has been observed only in the two cases of 
Rieger and Sommer. They are of such great importance that I must relate 
some of their details. Rieger's report, which is a remarkable example of 
impartial description in a difficult realm.' shows that the patient had lost 
all conception of three of the small letters of the German alphabet, p, x, and y, 

i Arch. f. Psych., 15. Bd., p. 276, " Wanderversajnmlung siidwestdeutsch. Irrenarzte 
u. Neurologen," 1883. 



298 THE SYMPTOM-COMPLEX OF APHASIA 

and of the small Eoman letters these three as well as d, h, k, and v. Of the 
capital letters he had lost the conception for fourteen of the Eoman as well 
as the German alphabet; namely, all those previously mentioned with the 
exception of D, as well as B, E, F, M, ~N, E, T, and W, so that he retained 
the power to use only eleven letters. This defect included both written and 
printed letters. When the patient was shown these letters he behaved as 
though they were absolutely foreign things, and when he was told " This is 
a B " or " This is an E " he shook his head and said " I do not know." 
But he at once recognized the letters he knew, being able both to name and 
to write them. He responded in the same manner to words composed of 
letters with which he was familiar. He was absolutely unable to write spon- 
taneously the missing letters; he could laboriously draw them from a copy 
as if they were unfamiliar arabesques. So far as his perception of letters 
extended, the patient could write from dictation or from copy, but at first 
without understanding what he wrote. Spontaneous expression by writing 
was absolutely impossible, and in general he read without any comprehension 
of the subject. In his spontaneous speech there was nothing conspicuous. 
Now and then he was disturbed by being at a loss for a word, usually a sub- 
stantive. Of figures he knew only 0, 1, 2, and 3, all others and all combina- 
tions of those he knew being absolutely incomprehensible to him. Besides 
this more or less localized defect the patient exhibited another which usually 
belongs to the realm of speech; that is, an inability to name objects shown 
him. He handled these, and in a general way had some perception of them, 
as well as of their properties. There was also an impairment of memory 
and of all the realms of special sense; this was no less marked than in 
Grashey's celebrated case which was subsequently investigated by Sommer 
and Wolff. As showing other defects, we must also mention the loss of the 
art of drawing in this man who was previously a skilful sculptor. All appre- 
ciation of curves was gone. In a restricted, practical sense there was no 
diminution of intelligence. For our purposes it is interesting to report that 
this patient could always find words for objects shown him, even though after 
an abnormally long time, and that among the objects presented to him he 
seemed to prefer letters inasmuch as he could name these in one-half the 
time consumed in naming other objects. According to Eieger's explicit report, 
this condition remained unchanged for two and a half years. The patient 
was a sculptor and teacher of drawing, aged 32, who had sustained a severe 
fracture of the skull in a railway accident. The defect did not appear at 
once, at least not to its full extent, but developed six months after the acci- 
dent and finally reached the degree which has been described. In Sommer's 
case x there was a similar and persistent lack of perception of certain letters, 
and a partial literal alexia and consequent agraphia, the result of an attack 
of apoplexy. In this case the recognition of a number of other letters varied 
and was uncertain. Moreover, the patient was unable to combine the letters 
he knew into words, and thus to read, so that the condition was practically 
a complete alexia. Still more complicated was the perception of written 
language which was somewhat better retained; hence the case reminds us of 

i Sommer, " Zur Theorie der cerebralen Schreib- u. Lesestorungen." Ztschr. f. 
Psych, u, Phys. der Sinnesorgane, 5. Bd., p. 305. 



DISTURBANCES OF WRITTEN LANGUAGE 299 

subcortical alexia. For all practical purposes there was also agraphia. It is 
interesting to know that immediately after the apoplexy, and for about a year 
and a half, the patient was unable to read or write. At first he had little 
command of words. After fourteen days the right-sided hemiplegia improved, 
and while under observation right-sided hemianopsia was demonstrated. 
Aside from this disturbance of the power to read and write and his inability 
to find words with which to express himself, the patient was intelligent and 
sensible, hence we must assume that this disturbance of speech was not so 
marked as in Kieger's case. Xo autopsy was held in either of these cases. 

In explanation of the peculiar partiality of this isolated impairment of the 
perception of written language it has been pointed out that in all disturbances 
of reading the more infrequently used letters, such as x and y, are more apt 
to be implicated than those in frequent use. However, in these patients it 
was not the letters most seldom used which were lacking but the quite com- 
mon ones, and the persistency of the defect appeared to exclude a purely func- 
tional nature. In all our experience in speech disturbances in focal diseases 
of the brain we know of nothing analogous except one case of mental disease 
after recovery from which a partial motor sensory aphasia and a defective 
use of words persisted. 1 In fact, as in mental diseases which show a prefer- 
ence for certain association tracts and functions, we must assume in the first 
case a similar degenerative process as a consequence of shock to the brain, 
and in the second case an apoplectic attack. Obviously the affected tract is 
the tract c a. 

As an illustration of pure isolated agraphia the case might answer which 
I mentioned at the beginning of this article (unusual and rare as this case is) 
because it presents the postulated defect in a comparatively pure form, and 
careful investigation demonstrates that the patient also suffered from a dis- 
turbance of internal speech and word conception. Besides we would neces- 
sarily assume that there was formerly a decided disturbance of speech which 
had greatly improved. The reduction in the power of spontaneous speech 
which was conspicuous during the time of observation and even two years 
later, was in contrast to the otherwise active intelligence of the patient, and 
this justifies the differentiation of the disturbance as one of transcortical motor 
aphasia, although not of high grade. In this connection I may refer to the 
published report of my own case. 2 Therefore, it will be seen that the same 
complication was present in the few comparatively pure cases of agraphia 
which I compiled from literature, and I reached the same conclusion as 
Dejerine that there is no pure literal agraphia, or motor agraphia, as Pitres 
called it. Notwithstanding this it is certain that the peculiar symptoms of 
the case cannot be explained solely by the disturbance in word conception, but 
that aside from this, the exquisitely literal or. better, the motor, character 
of the agraphia forms the predominant feature in the pathologic picture. 
This is also true of the few analogous cases which I was able to find. My 
patient, as is proven by the comparatively well retained faculty of reading, 
undoubtedly received optical memory impressions of letters, but, unlike a 

i Heilbronner, " Aphasie und Geisteskrankbeit." Peychiatriscbe Abhandlungen, 
herausgegeben von Wernicke und Zeitschr. f. Psych, u. Physiol, d. Sinnesorgane, XXIV, 
p. 83. 

2L. c. 



300 THE SYMPTOM-COMPLEX OF APHASIA 

normal person, these could not be transmitted to the motility at his command. 
Comparing this with our diagram of written language there appears to be 
an interruption of the tract a /3. From my previous presentation it is evi- 
dent that this tract is bilateral, and the especial peculiarity of my case, as 
well as of the few analogous ones, is the bilateral absence of function. 

In this case symmetrically situated foci in both hemispheres, but less 
marked in the right, might possibly be thought of; the periodic appearance 
of left-sided symptoms might be regarded as favoring this view, and such a 
possibility must be borne in mind. But the analogous cases presented signs 
of left-sided focal disease exclusively, and cannot be explained by the assump- 
tion of symmetrical foci. We are therefore forced to the conclusion that the 
disturbance of word conception and also a general damage to the functions 
of the brain might under some circumstances have such an effect that besides 
directly damaging the unilateral tract it might render the other also incapable 
of function. In our case the transcortical disturbance of speech, still evident, 
is probably insufficient to permit the utilization of a tract on one side for a 
function which is too difficult and, therefore, impossible to perform. As a 
rule, when these conditions exist we must consider the unilateral interruption 
of the supposed tract. We actually find an excellent example of this in the 
case which Pitres, in spite of the unilateral nature of the disease, designated 
as " agraphie motrice pure." In this case it appears that some time pre- 
viously an agraphia similar to that of my case had existed. This, however, 
improved, persisting only in the right hand, although the motility of the hand 
was otherwise restored. Eight-sided hemiopia with good vision was perma- 
nent. There was originally a right-sided hemiplegia with severe general 
symptoms. In these cases of unilateral disturbance of the power to write, the 
use of the term agraphia might very properly be questioned. But no doubt 
this is such, and scarcely any other explanation is permissible than that it 
is a unilateral interruption of the tract a ft of our diagram. Liepmamr's case 
of unilateral apraxia showed on minute investigation the same phenomenal 
motor agraphia limited to the right hand, only this formed a part of the 
total picture of unilateral apraxia. The patient was able to write with the 
left hand, but he performed mirror writing. This example, it appears to 
me, throws light upon the function of the right-sided tract, a ft. 

According to Liepmann mirror writing or, more appropriately, abduc- 
tion writing, is a striking proof of the purely mechanical way in which mem- 
ory pictures of letters are transferred to motility. While learning to write 
most persons practise this process by chiefly using the left-sided tract, a ft', 
which, we may at once explain, is in accordance with the principle of the 
conservation of energy. But we must also take into account the general 
experience that in such practice symmetrical movements of the other half of 
the body are unconsciously practised, movements which we attribute not only 
to the functions of the motor anterior horn cells of the spinal cord but also 
to complicated cerebral functions. The so-called conclusion by analogy de- 
pends upon this. If, however, the same innervation of the right arm center 
takes place by means of right-sided memory pictures of letters in the right- 
sided tract, a ft, as in ordinary writing, and the left arm center by means 
of the left-sided tract a ft, mirror writing or abduction writing must result. 
Now it is also the function of this tract voluntarily to conduct writing move- 



DISTURBANCES OF WRITTEN LANGUAGE 301 

merits of the left hand in such a way that they correspond to the identical 
direction conception instead of to the symmetrically opposed ones, as in writ- 
ing with the right hand. But this is primarily drawing, which is done with- 
out practice, and even in opposition to the involuntary innervation passing 
in an opposite direction. It therefore represents a much more difficult cere- 
bral activity than mirror writing, which is produced by a tract which is some- 
what well marked. Subjectively this necessitates a special exercise of the 
will. Thus we comprehend that a transcortical motor aphasia, even though 
only feebly indicated, renders correct writing by means of this tract impossi- 
ble; the necessary .stream of innervation is too weak at its source. What, 
however, is not so easily understood is the fact that our patient with agraphia 
never showed the slightest inclination to mirror writing; our suspicion of a 
very circumscribed, symmetrical focal affection of the right hemisphere must 
be thereby increased. Moreover, the conditions which produce mirror writ- 
ing are still imperfectly understood. Accompanying right-sided hemiplegia, 
either with or without disturbance of speech, it is apparently sometimes ob- 
served under the same conditions, and sometimes not. It is chiefly noted in 
persons whose minds are somewhat impaired, with or without signs of uni- 
lateral atrophy of the brain, and especially in idiotic children (Soltmann). 
If normal children who have had the same elementary instruction are forced 
to write with their left hands, some produce mirror writing, others do not. 
When mirror writing is noted it is usually quite correct, and proves that there 
is no literal agraphia. 

I shall return to the presumable position of the tract a /?. We see that 
a certain point in this tract may be determined with some degree of likelihood, 
and thus isolated agraphia as a focal symptom becomes as important in local 
diagnosis as is isolated alexia as a focal symptom of the gyrus angularis. 

One of Rieger's cases which was reported by Wolff x appears to be an 
example of pure cortical agraphia. Here total central blindness due to bilat- 
eral destruction of the optical projection fields of course also resulted in cor- 
tical alexia. 

For the rare cases of isolated literal agraphia which are analogous to our 
first case, it is practically unnecessary to search our diagram for a designation. 
Nevertheless it is instructive to consider this analogy and the corresponding 
disturbances of speech; it then appears to be the much disputed conduction 
aphasia which forms the prototype for the assured possession of this form, 
so that a lesion of the tract a ft would justify the conception of conduction 
agraphia. As I have repeatedly emphasized, its nature is evident from the 
fact that in writing there is no conception of the form of the letters, while 
the power to produce them is still intact; hence those cases of agraphia in 
which the conception of the form of the letters is well retained, no matter 
how incorrect the writing, do not belong to the pure disturbances of written 
language, but to the sequels of disordered speech or disturbed communication 
of word conceptions. Such cases are therefore differentiated from the previ- 
ously described literal agraphia as verbal agraphia. That this verbal agraphia 
may possibly result from a lesion of the tract c a is shown by a case which 

i G. Wolff, " Ueber krankhafte Dissociation der Vorstellungen." Habilitation- 
schrift, Leipzig, 1897, pp. 43 et seq. 



302 THE SYMPTOM-COMPLEX OF APHASIA 

I mentioned in my previous report; certainly it is an extremely rare occur- 
rence, for I have never seen anything similar. It is quite conceivable that 
cases of sensory aphasia which have recovered, and these are not infrequent, 
may leave such a circumscribed defect because restitution if not perfect may 
perhaps be • incomplete in only one respect; namely, that the most difficult 
function is impaired. Without doubt the transmission of the internal word 
conception to its written expression is the most difficult function which is here 
called into exercise. This view bears out the general experience that among 
all the symptoms of aphasia the disturbance of written expression is the most 
intense and tenacious. This explains why paraphasic symptoms which are 
not noticeable in speaking are observed in writing. It often happens that 
paraphasia which is only slightly evident in speaking becomes most obvious 
on spontaneous writing, or the existing paraphasia may be greatly aggravated. 
This condition is frequently noted in paralytics with aphasia of varying 
degree. 

Paragraphia in a restricted sense, or literal paragraphia, in which the form 
of letters is distorted, is of practical importance, being the disturbance of the 
power to write noted in paralytics. A tentative diagnosis of progressive paral- 
ysis may not infrequently be made by observing a few words which contain 
distorted letters. A general impairment of the memory in senile or other 
organic atrophy of the brain, in presbyophrenia and polyneuritic psychosis, 
produces a similar paragraphia, apparently because the patient forgets the 
exact formation of the letters. 

A review of the disturbances of written language shows that I was justi- 
fied in claiming that we are dealing with essentially transcortical symptoms, 
and it is especially interesting to note that these transcortical symptoms may 
follow lesions of quite definite areas of the brain, just like cases of pure word 
blindness and so-called pure literal agraphia. 

It is not a mere accident, therefore, that the study of written language 
has up to the present time enabled us to arrive at most valuable conclusions 
concerning the purely transcortical process which, according to prominent 
authors, we may most aptly designate as " word finding." Word finding, par- 
ticularly for individual letters, is a prerequisite for ordinary reading by means 
of spelling and, for practical purposes, the letters which must be found are 
like all other word conceptions. Therefore, reading is a part of the expressive 
portion of the speech process, in which the finding of words for different con- 
ceptions is the first and most necessary object. According to Rieger and G. 
Wolff, word rinding for letters that are seen occurs normally and unisensually 
by optical conceptions, like those of color. Word finding for letters is, how- 
ever, the most necessary and most common part of expressive language; 
therefore, in comparison with all other visible objects, it may be preferable 
or easiest. In Eieger's case word finding for visible objects was about twice 
as rapid as for others; in Wolff's case (identical with Grashey's) it occurred 
instantly, and apparently even without the otherwise necessary writing move- 
ments, for reading was immediately possible. For word finding the optical 
memory pictures of the letters equals the different memory pictures of con- 
crete substances which form the conception, B. If we east a glance at Fig. 
137 it at once becomes apparent that the direct communication, B b, which 
we must maintain for clinical purposes, is probably unnecessary for letters., 



DISTURBANCES OF WRITTEN LANGUAGE 303 

hence we postulate merely the association tract, B a. This tract which, like 
B, here equals the optical memory picture, is contained in the optico-acous- 
tic commissure (Bastian) of the same and of the crossed 'side. Its ana- 
tomical foundation for the same side is perhaps the lower longitudinal 
bundle; for the crossed side, the forceps tapetum tract, as it was called by 
H. Sachs. 

C. S. Freund and H. Sachs also assume that the same association tract 
produces word finding for objects shown, and its injury is held responsible if, 
under some circumstances, an object which when first seen cannot be correctly 
named, although it is clearly recognized, is correctly designated after exam- 
ining it by the sense of touch. 

For this symptom, if restricted as above, the name optical aphasia 
(S. Freund) is not inappropriate. It occasionally appears in the form just 
described, and then belongs to the interesting group of conditions known as 
" dissociation of conceptions." It cannot, however, be referred to the previ- 
ously mentioned tract, since all experience shows that for visible objects a 
recognition of their meaning is indispensable to word finding, and no case 
has yet been observed (for instance, of mind blindness) in which objects not 
recognizable by the sense of sight were nevertheless correctly named. For the 
recognition of visible concrete things their secondary identification (in this 
case the association of their optical fields of memory) is absolutely necessary 
for the tactile conceptions belonging thereto. As we have seen, the conditions 
are different with letters. 

Although most conspicuously lacking for visible objects, the symptom of 
impaired or entirely arrested power of word findings may usually be deter- 
mined more quickly than any other impression of the general senses. In 
Rieger's case all of the special senses responded to the same stimulus which 
proved so successful in word finding, but they invariably required a certain 
time which was about uniform. 

The preponderating power of the sight in comparison with the other senses 
was noticeable in the Wolff-Grashey case; the object seen, not merely the one 
felt or heard, enabled the patient to find the word (in writing). In the 
unisensual conceptions of the tactile realm some exceptions were noted, such 
as wind, heat, cold, and in the auditory thunder, polka, waltz. That word 
finding for such paramount tactile conceptions as smooth, rough, pointed, 
sharp, and the like, requires the conjoint action of optical perception, also 
proves the special influence in this case of optical memory pictures. 

From the valuable work of Wolff it appears that various conceptions, 
according to their meaning, cannot be composed of partial conceptions or 
memory pictures of equal value, but that a main determining area of special 
sense, and secondarily of memory pictures, may be differentiated. He pre- 
sumes by analogy that the sense of sight is the chief and determining one 
for word finding, as in his case; but this is quite unlikely, and is disproven 
by Rieger's case. If, however, in Wolff's case, we ascribe to the optical tract 
a comparatively better retained function than to the other projection fields, 
we can readily understand its exceptional action, otherwise never observed, 
which shows that the patient found his words by the roundabout method of 
writing pictures. 

When, as in the cases of Rieger and Sommer, the power to find words 



304 THE SYMPTOM-COMPLEX OF APHASIA 

for letters is so disturbed that only the perception of certain letters is miss- 
ing, others being perfectly unimpaired, we may quite properly maintain that 
the patient had lost all conception of the letters in question. Much more 
common is a condition in which there exists merely an uncertainty in word 
finding, especially for certain letters, without this symptom being constant. 
In spite of this, patients can occasionally read without effort words contain- 
ing letters of which previously there had been no conception. An example 
of this was furnished while our case of agraphia was under observation. 
Inability to read because of the loss of individual letters was much more 
marked in a case of Bastian's in which, in consequence of extreme paraphasia, 
not a single letter could be correctly named nor could words be read aloud; 
nevertheless the patient understood what he read. I mention these cases 
because, more than any others, they expose to probable criticism our view 
that reading is the result of spelling. They are explained, however, by two 
generally recognized experiences. The one is this, that every virtuosity in 
the sequence of individual acts, which is acquired by practice, suffers as soon 
as close attention is directed to this individual act. The simplest example 
of this is running down stairs, with or without observing the individual steps. 
Attention drawn to the individual act, in this case manifested for the indi- 
vidual letters, is therefore a deleterious factor for the complete performance 
of an act, and we therefore understand that the complete act would much more 
readily follow than the individual performance. A similar experience is found 
in the more frequently observed cases of pure word blindness, in which ordi- 
narily there is no impediment of speech nor paraphasia, but in which the 
name for objects shown cannot be recalled or only with the greatest difficulty. 
Here also the attention bestowed upon the object may prevent the word find- 
ing. It appears to me that Bleuler maintains the same in differentiating the 
more automatic and fully conscious association processes. A second general 
fact, in which spelling appears to compensate for some defects in reading, 
is the familiar one that all impression processes are facilitated by secondary 
identification. The most marked example of this is the recognition (in spite 
of demonstrable and severe sensory disturbances) of objects that are only felt. 
That this process, closely akin to guesswork, normally plays a great role, is 
known to us all (as in the reading of handwriting). 

The celebrated case of Grashey was first explained by the supposition that 
the aphasic disturbance and inability to find words were due to a purely func- 
tional disturbance, namely, the loss of memory for recent impressions or, more 
accurately, a decrease in the power of memory. I formerly agreed with the 
opinion that in Grashey's case it was necessary to recognize a special form of 
amnesic aphasia. In the meantime, Sommer showed the untenability of this 
view, and G. Wolff's subsequent careful investigation of the case led to the 
same result. The peculiarity of Grashey's case must not be sought in the 
nature of the aphasic disturbance, but in the artifice dependent upon the indi- 
vidual activity which the patient resorted to in order to conceal the actual 
aphasic defect of word finding for visible objects. We have noted how this 
artifice favors the fact that our written language is essentially a language of 
letters. The difficulty of word finding has, however, always been regarded as 
an important aphasic factor; very early Trousseau, for example, contrasted 
it with Broca's aphasia, and it actually corresponds to the practical require- 



DISTURBANCES OF WRITTEN LANGUAGE 305 

ments according to which Pitres x lately included under the definite term of 
amnesic aphasia all cases in which this symptom is either observed alone or 
is prominent in the clinical picture. 

We cannot go beyond the recognition of this practical requirement, for, 
on the contrary, we must emphasize the other variations in the cases which 
in this one point agree. The majority of cases of isolated word blindness 
belong in this group, also Rieger's case, and the very peculiar case of Grashey. 
Both of these have the common symptom of an almost complete loss of mem- 
ory, but this phenomenon alone could not have been the cause of the inability 
to find words, for this is absent in some cases of pure word blindness marked 
by difficulty in word finding. Besides, the majority of cases are of different 
origin, for they are usually cases of aphasia in which there is a certain com- 
pensation as regards speech. 

Pitres collected 10 cases in 8 of which he found that the lower parietal 
lobe was implicated; this is not mere coincidence, but agrees with Xaunvn's 
statistical report that in the cases which he called " indefinite aphasia " there 
was the predominance of a lesion at the boundary of the lower parietal and 
occipital lobes. Of all the disturbances of speech, motor aphasia appears to 
be the one in which, when recovery follows, a real disturbance of word finding 
is most rarely found; we are therefore forced to agree with Pitres, inasmuch 
as he also fails to include the implication of Broca's convolution. For the 
rest, however, the great dissimilarity of the cases proves either that very dif- 
ferent localities are implicated, or that functional disturbances of different 
localization must act together. Pitres, who recognized the majority or the gen- 
erally acknowledged disturbances of speech and written language, emphasizes 
the great rarity of pure cases which would fulfil all of his requirements. He 
considers it essential that they show absolutely no disturbance of the under- 
standing, either in reading or in writing, but only a difficulty in word finding. 

The meaning in such cases of a severe impairment of the memory has not 
escaped him. lie points to the universal experience that a patient with diffi- 
culty in word finding, notwithstanding repeated instruction, has the same 
difficulty in finding definite words (which he gladly accepts if prompted) 
as when only the beginning of the word is given him. Moreover, Pitres 
attempts to explain the want of understanding in reading by the theory of 
a disturbance of the memory, but this is opposed by Grashey's case of mark- 
edly impaired' memory with intact ability to read. It is very interesting, 
however, to know that Pitres does not absolutely accept the view that the 
lower parietal lobe is the seat of disturbance; on the contrary, he recognizes 
that in word finding the stimulus of the true speech center is distributed 
by neurons over the entire cerebral cortex, and these are to be regarded as 
the carriers of psychical operations; the lesions therefore have no fixed topog- 
raphy, but act by the interruption of the commissure between the different 
centers of the word pictures and the cortical areas in which the higher psy- 
chical acts are carried out. " Amnesic aphasia consists solely and alone in a 
lack of connection between the conception and the word which serves to express 
it." In a note we even find the statement that amnesic aphasia should prop- 
erly be designated " transcortical aphasia." 

i A. Pitres, " L'Aphasie amnesique et ses varietes cliniques." Paris, 1898. 
21 



306 THE SYMPTOM-COMPLEX OF APHASIA 

For the practical recognition of amnesic aphasia it might be advantageous 
to differentiate between those cases which show only a difficulty in word finding 
and those which disclose, in addition, the symptoms of paraphasia. The latter, 
by far the most common, are almost always the residua of past disturbances 
in speech, and therefore need not be considered. Aside from these, difficulty 
in word finding shows itself almost exclusively or most markedly when objects 
are presented to view (and also probably when listening, touching, etc.), and 
sometimes by the absence of definite expressions, especially substantives, in 
the otherwise fluent and correct spontaneous speech. A defect scarcely notice- 
able at first becomes more prominent when we ask the patients to name objects 
that have been shown. To cover their confusion the patients frequently use 
roundabout expressions which denote embarrassment, such as " thingumbob." 
The choice of incorrect or unsuitable expressions, however, belongs to the realm 
of paraphasic symptoms. 

The difficulty in finding words may, as is well known, be met with under 
normal circumstances. Eieger very aptly remarks that this occurs in normal 
persons only when using words of limited importance ; for example, the word 
" magnolia " cannot be recalled. " When, however, a person can no longer 
say ( This is a tree,' he must unquestionably be regarded as having a disease 
of the brain." It is also true that the extent of these terms of limited impor- 
tance may vary greatly according to the occasion, therefore according to the 
mental condition in which the person happens to be. Eieger, so far as I know, 
was also the first to call attention to the fact that all persons whose command 
of a foreign language is imperfect show a very marked inability to express 
themselves in words, many of which they may understand perfectly. Were 
this equally true of their mother tongue, it would correspond to our concep- 
tion of amnesic aphasia. Under the same conditions an increase of this defect 
may be apparent when they attempt to name objects shown them. This is 
generally more difficult in the course of conversation than at other times, and 
probably is due to the closer attention devoted to the object. 

Pitres shows that the " systematic aphasia " of polyglots presents a stage 
in which word finding in the foreign tongue is impossible, while the power 
to understand has been retained; therefore a stage of amnesic aphasia. 

After this description it is obvious that difficulty in word finding is noth- 
ing more than a special form of transcortical motor aphasia; in other words, 
a disturbance of the function of the tract B b of our diagram. In Eieger's 
case, as well as in that of Wolff-Grashey, this disturbance consisted of a slow- 
ing in the function which, however, was finally performed correctly. In 
other cases, word finding is either impossible or to a certain degree is acci- 
dental, and this is prone to be the case in the individual whose brain is not 
actually diseased. 

Nothing is more obvious than the assumption that a disturbance within 
the complicated tract B is the cause of the difficulty in word finding. Aside 
from the already mentioned defect in the conception of letters, the fact that 
in Eieger's case there was also a decided and demonstrable impairment of 
conception favors this view; all conception of playing cards, coins, paper 
money, stamps and similar things had been completely obliterated, and it 
was impossible to awaken any conception of these objects, as well as of any 
figures higher than figure 3, of algebraic signs, of musical notes, of the mean- 



DISTURBANCES OF WRITTEN LANGUAGE 307 

ing of piano keys, or of punctuation marks. Something analogous, a decrease 
in the number of conceptions, appears only in consequence of mental disease, 
and the condition Rieger reported reminds us most forcibly of a case of 
aphasia following mental disease which I described, as did also Heilbronner 
in its later stages ; 1 in this instance partial motor and sensory aphasia were 
manifested by the fact that there was only a number of words for which 
conception was lacking. For the retained power of conception we assumed 
a damage of slighter extent, namely, a decreased stimulation of the con- 
ceptions themselves as well as of the partial conceptions of which they were 
composed (for instance, memory pictures), which change we were forced to 
assume by the clinical findings, since the slowing of the process for the 
recognition of objects could be physiologically explained only as a slowing 
of the stimulation of some special sense. The same slowing could be demon- 
strated in regard to the understanding of words which express the concep- 
tions. The contrast between word understanding and word finding, which 
might be urged in opposition to the theory of an affection of the conceptions, 
is therefore not absolute; both processes were slowed, but one much more so 
than the other. When objects were shown or handled, etc., the period of 
time required for the recognition of the conception could be determined, and 
it was at the same time evident that the chief delay was caused by the act 
of word finding. 

At the same time it was demonstrated that the conception itself, not its 
partial memory pictures, always led to the knowledge of the name. In Wolff- 
Grashey's case the conception in toto, not a partial conception, produced the 
word. To the above emphasized unisensual conceptions there were naturally 
some exceptions. The contrast between sensory and motor functions, between 
the apparently retained understanding of a word and the difficulty in word find- 
ing, is also evident here, but the intactness of the conception itself is far from 
proven. If, for example, in answer to the question " Is the leaf blue ? " 
Wolff's patient had answered " It may be " or " I do not know," this would 
indicate that his understanding of the sense of the words " leaf " and " blue " 
was also affected ; therefore, for the receptive portion of the process of speech, 
a defective conception was demonstrated. If the condition is such as to neces- 
sitate a certain stimulation of the entire association complex which we term 
conception in order to innervate upon the tract B b the correct word concep- 
tion, or the correct movements of word conception, we may understand the 
influence which perception by the senses may under some circumstances have 
upon word finding: The corresponding conception thereby gains in intensity. 
This influence is greater the closer the relation of distinct individual ideas 
which together form the conception. In referring again to the diagram. Fig. 
137, the importance of the foregoing conclusion is obvious. No less than 
four equally important innervation streams must unite in b in order to pro- 
duce the correct word conception. 

We now understand why disturbance in word finding is so common after 
focal diseases of the brain which run their course with sensory defects. Of 
the many tracts which unite in b, only one or the other will be damaged, 
only the innervation of a partial conception need be decreased, to render 

iL. c. 



308 THE SYMPTOM-COMPLEX OF APHASIA 

word finding difficult. That conceptions are also less intense under relaxa- 
tion is readily understood. 

It may perhaps not be deemed snperfluous when I remark that neither 
in Grashey-WolfFs case nor in Bieger's case had the understanding of speech 
suffered, for both patients were able to repeat words without any slowing 
of the speech. 

Usually we refer to transcortical motor aphasia only when the impairment 
of the power to find words has increased to an entire absence of spontaneous 
speech or to a state which almost equals this defect. If, however, the disturb- 
ance is confined within the limits of amnesic aphasia (Pitres), the phenome- 
non of a focal disease is apparently no longer present, but we suspect a dis- 
turbance of conception or intellect which, as we have seen, finds its analogy 
chiefly in mental diseases. Since Eieger has given his excellent directions 
for the investigation of such cases I do not doubt that in most instances some 
such intellectual defect will be found. Any focal symptoms which are also 
found will be only of secondary importance as indicating the area which is 
the seat of general decrease of cerebral activity; as, for instance, in cases of 
post-apoplectic dementia. That the symptoms of transcortical motor and 
sensory aphasia due to senile atrophy of the brain may frequently be recog- 
nized even without traces of a focal disease has been shown by Heilbronner. 
In such cases there are usually signs of mental blindness, or asymbolia, which, 
when there is mental disease, depends upon a disturbance of the power of 
secondary identification. To enter more minutely into the study of these 
phenomena is impossible, since the richness of their number and their impor- 
tance demand special treatment. But I must point out one of the most recent 
developments in this borderland between mental disease and focal affection 
of the brain, because it is most intimately connected with the explicitly treated 
theme of word finding. This is the pathologic picture of unilateral apraxia, 
constructed and masterfully described by Liepmann. As an unusually favor- 
able coincidence is necessary in order to produce the typical pathologic picture 
of transcortical motor aphasia, we also owe it to such a coincidence that in 
Liepmann's case a focal disease of the brain was so situated, and of such 
nature, that it severed the innervation of the transcortical tracts for the motil- 
ity of one-half of the body. Moreover, as the patient in consequence of sub- 
cortical motor aphasia was deprived of the power of speech, the intelligence 
could be tested only by means of his motor reactions. The reaction of the 
members on the right side of the body indicated that the most extreme psy- 
chical defect, absolute dementia, existed. However, when the left members 
of the body were exclusively used in carrying out the reactions, a compara- 
tively high degree of intelligence could be demonstrated. The autopsy of this 
case, which has not yet been published, confirmed the diagnosis of focal dis- 
ease of the brain. 

I shall not give explicit instructions for the examination of aphasics; it 
seems unnecessary, for this article embodies the main points. But two phe- 
nomena require special mention and careful consideration. One of these is 
the extreme exhaustion which these patients often manifest and which soon 
compels the physician to interrupt the examination, which can be only resumed 
after the patient recovers from the fatigue. If no attention is paid to this 
point, the different examinations will lead to absolutely contradictory results. 



DISTURBANCES OF WRITTEN LANGUAGE 309 

The second source of error is perseveration (Xeisser) ; it consists in the in- 
clination of the patients to repeat a reaction they have once found, even though 
they know or observe that they are doing wrong or are speaking incorrectly. 
The patients often express their annoyance at this, and show plainly that the 
second reaction was not intended. They are therefore subjected to a certain 
constraint. In the case of aphasics this very common symptom is extremely 
annoying, for the physician is often forced to pause, or to divert the attention 
of the patient into another channel, in order to obtain a new and correct 
reaction. As A. Pick has demonstrated, this disturbance may be so increased 
as to form a variety of pseudo-apraxia. 

I cannot conclude these essential, clinical, symptomatologic considerations 
without discussing more explicitly the question of the unilateral position of 
the centers of speech, as set forth in the preceding. My entire description is 
based upon the fact, learned by experience, of the unilateral position of the 
so-called centers, and this fact must have been particularly impressive, for 
one of the earliest investigators in attempting to localize these centers main- 
tained that the left hemisphere of the brain was exclusively the seat of the 
function of speech (Marc Dax). 1 In my presentation of the subject I have 
referred only to the left hemisphere, my purpose being to speak of the excep- 
tions subsequently. Here two series of facts, abstractly connected, must be 
chiefly considered. One series of exceptions relates to speech disturbances in 
left-handed persons: These'are almost exclusively due to disease of the right 
hemisphere, the seat of the focus and the symptomatology bearing the same 
relation to each other as the corresponding focal diseases of the left hemi- 
sphere in the right-handed. We do not know how to explain the fact that the 
conspicuous right-handedness observed in most persons, i. e., the choice of 
the right hand for all movements which require great strength or dexterity, 
causes the functional development of the speech center situated in the left 
half of the cerebrum, and only this one principle is absolutely plain, that the 
unilateral location in itself appears justified because it indicates a conserva- 
tion of energy. A step forward is noted in Oppenheim's report of a case in 
which marked sensory aphasia due to a tumor appeared simultaneously with 
left-sided hemiplegia; the history revealed the fact that the patient was not 
left-handed from birth, but that in her seventeenth year, after sustaining an 
injury to her right hand, she was compelled to use the left, and had since 
become left-handed. Autopsy revealed the suspected tumor in the right hemi- 
sphere. This observation leads to two conclusions : first, that the right hemi- 
sphere may assume the function of speech even in adult life in place of the 
left hemisphere whose function it is normally; second, that the left hemi- 
sphere may completely lose the previously acquired function of speech if the 
person becomes left-handed. I know of no other observation which so strongly 
confirms our view that the localization of speech centers is the functional 
acquirement of each individual. 

The other exceptions are pathological experiences in childhood. In focal 
diseases of the left hemisphere in childhood, disturbances of speech are ob- 
served which vary in degree according to the development of the child; these 

i The first investigator was Bouillaud, who taught that the anterior lobes of the 
brain were the seat of the faculty of speech, 



310 THE SYMPTOM-COMPLEX OF APHASIA 

are proportionally and rapidly compensated for, even when subsequent find- 
ings reveal the complete destruction of the well known left-sided speech cen- 
ters. Apparently, especially in childhood, the preexisting left-sided cerebral 
function is readily transferred to the right hemisphere. Under these circum- 
stances left-handedness is not necessarily at the same time produced; if the 
case comes to autopsy at a more advanced age there may be no symptom 
referable to the left hemisphere, and the lesions may simulate those in right- 
handed persons ; or, fresh lesions of the right-sided centers may cause aphasia 
in persons who are not really left-handed. As is evident, the faculty of purely 
individual acquirements belonging to the centers in question leads to a num- 
ber of possibilities which, under some circumstances, especially when the 
history is obscure, may form an incomprehensible exception to the otherwise 
regular localization of the speech centers. In fact we cannot deny a greater 
individual latitude than heretofore to the presumption, according to which 
at one time the left hemisphere, at another time the right is exposed, or 
perhaps both in the same case, with or without the predominant assumption 
of the function of speech by one hemisphere. 

This point of view becomes especially important when we consider the 
greatly discussed question whether in the fully developed brain, and in how 
far, a substitution for destroyed speech centers takes place by means of tracts 
in the same localities of the other hemisphere. The explanation of the symp- 
toms during convalescence from aphasic disturbance, in the widest sense of 
the term, depends chiefly upon this prior question, but, concerning it, we are 
still in ignorance. It is generally conceded that slowly growing pathologic 
foci which act exclusively by displacement, at least to the same extent as is 
otherwise the case, produce no symptoms, not even of speech disturbance, 
although the autopsy findings may indicate that the center or the tracts under 
consideration have been severely affected. But this experience is by no means 
general. There are cases which appear to prove the contrary, as for exam- 
ple, Oppenheim's case, in which during the growth of the tumor the trans- 
mission of the sensbry speech function to the left hemisphere would have 
been much more likely, as this merely involved the resumption of a function 
previously exercised. The explanation of this contradiction need not be based 
entirely on individual differences. It may readily happen that the number 
of general symptoms accompanying the focal symptoms will guide us to a 
decision. The two focal diseases which here come chiefly into consideration 
are brain tumor and brain abscess. These diseases may sometimes present 
but few accompanying general phenomena, at other times the general symp- 
toms are most pronounced. The activity of the other hemisphere is to the 
same extent preserved or damaged; and it is obvious that its injury does 
not favor the re-establishment of function. According to general experience, 
the sensory speech function shows a greater resistance to injury than the 
motor. In this connection it is more strictly correct to differentiate between 
the impressive and the expressive parts of any speech process. The conditions 
are clearer when we consider the substitution by the other hemisphere in pure 
diseases of the brain, or in those in which there is a predominant destructive 
action, such as cerebral hemorrhage or cerebral softening. Here the possi- 
bility of restitution by a new acquirement may be markedly influenced by the 
general symptoms of the apoplectic insult, for we have often noted that a 



DISTURBANCES OF WRITTEN LANGUAGE 



311 



severe attack, besides leaving unilateral focal sjrniptoms, produces general in- 
jury to the brain as shown by the ready exhaustion, the impaired memory, 
and the emotional weakness which become chronic. Since experience teaches 
that the accompanying insult in motor aphasia is usually decided, and in 
sensory aphasia and pure word blindness is usually only slight or absent, 
we also conclude that this favors restitution of the sensory speech function, 
which is the rule, and heightens the importance of pure alexia as a focal 
symptom. 

Concerning the unilateral position of the speech centers there is another 
condition which, in my opinion can only be explained by the theory that the 




ft 

Broca's convolution 



Sensory speech center 



Fig. 139. 



motor or, more correctly, the expressive, portion of the speech process is not 
always, and is not exclusively, unilateral. As is well known, the indirect 
focal symptoms of acute focal disease of the brain after well marked apoplectic 
attacks in general are limited to the affected hemisphere. To this, however, 
there are often exceptions; for instance there may be a more or less well 
developed motor aphasia and a resulting left-sided hemiplegia after severe 
apoplectic attacks. This condition usually lasts for only a few days, a proof 
that we are not dealing with left-handed persons in whom aphasia, even as 
an indirect focal symptom, must persist for a longer time. It can be explained 
only by the assumption that in the affected individual, prior to the attack, 
the right hemisphere with a certain portion of the process of innervation 
was implicated in active speech; when, therefore, the apoplectic insult origi- 
nating from the right hemisphere causes any injury whatever to the left 
hemisphere, obviously that function which was previously exercised by the 
conjoint action of the right hemisphere is most easily affected by the disease. 
Whethei- we are here dealing with a permanent damage to the left-sided 
Broca's convolution by the trauma of apoplexy, or only with a temporary func- 



312 



THE SYMPTOM-COMPLEX OF APHASIA 



tional disturbance, will be evident in the further course. There is a symp- 
tom which is positive proof that the apoplectic insult has damaged Broca's 
convolution. This is the condition designated as syllable stumbling, usually 
regarded as a reliable indication of progressive paralysis, and therefore a 
so-called paralytic speech disturbance. Exactly the same speech disturbance, 
syllable stumbling, is observed in the majority of patients who have recovered 
from motor aphasia, even though it has been merely an indirect or transitory 
focal symptom of the affection of the left hemisphere. Little as this fact 
is appreciated, it is nevertheless proven by many experiences, and I call special 




Fig. 140. 



attention to it because of its importance in differentiating this affection from 
progressive paralysis. 

We must now discuss the anatomical region of speech in the brain, or, as 
Dejerine briefly called it, on the basis of the preceding description, the " zone 
du langage." I believe this cannot be better done than by presenting the 
illustrations (Figs. 139 and 140) taken from the works respectively of Mona- 
kow and Dejerine; the darkly shaded portions in both illustrations are the 
centers which have been positively recognized, and the shaded areas represent 
the speech regions. On comparison these two illustrations show a very satis- 
factory agreement, especially when we consider that the two investigators 
worked independently. It is evident that, with the exception of the anterior 
processes and the lower connection of the central convolutions, the entire 
first embryonic convolution, i. e., the convolution limited by the Sylvian fis- 
sure, may be included within the speech region. The portion situated in the 
frontal lobe corresponds tc Broca's convolution, therefore to the motor speech 
center; that situated in the temporal lobe, therefore below the Sylvian fissure, 
is the sensory speech center; that above the termination of the posterior Syl- 
vian fissure, the so-called marginal convolution which is also known as the 



DISTURBANCES OF WRITTEN LANGUAGE 313 

supra-marginal convolution, both authors have included within the regions of 
speech, without having assigned to it a definite and specific function. These 
authors are also unanimous in no longer considering that the part above Broca's 
convolution, the so-called foot of the second frontal convolution, belongs to 
the speech region, although Exner, Charcot and Pitres, and recently Ziehen, 
locate the motor writing center in this region. Moreover, these authors con- 
cur in believing that the part of the convolution situated in the lower parietal 
lobe and adjoining the first embryonic convolution is still included with the 
speech region, inasmuch as it is prolonged posteriorly by means of an anas- 
tomosing and communicating bridge to the second embryonal convolution. 
This area extending horizontally and posteriorly is the angular convolution, 
the " pli courbe " of French authors. Dejerine also includes in his speech 
zone a neighboring part of the second temporal convolution, but this is not 
depicted in Monakow's illustration; in my opinion it corresponds with the 
majority of the findings. 

Within the shaded speech region of Monakow's illustration there are 
two, and in Dejerine's three, darker areas which, in the latter, are sharply 
demarcated; two of these almost absolutely agree, and they correspond to 
the more sharply defined sensory and motor speech centers. Dejerine alone 
indicates in a restricted sense a third center, which occupies the region of the 
angular convolution, and is designated by him as the center for optical mem- 
ory pictures of words. I have explained above in what sense we are to under- 
stand this; v. Monakow does not recognize such a special center. The two 
illustrations show distinctly that the speech region not only consists of the 
centers previously differentiated but also embraces the neighboring parts of 
the convolutions of the convexity. Nevertheless, they represent only a part 
of the speech region, for, according to the two previously mentioned authors 
and almost all other authorities, the entire island covered by the first embry- 
onic convolution and situated in the depths of the Sylvian fossa should also 
be included. In order to understand this, the Sylvian fossa must be deeply 
penetrated and the adjoining convolutions — those belonging to the embry- 
onic convolution — should be separated. The island is then exposed to view 
as a deeply situated, triangular, cortical area covered with compartment-like 
convolutions which are separated from the cortical substance, according to 
Burdach, by three deep spaces, an anterior, an upper, and a lower space. 
Where the lower and upper spaces meet and form an acute angle, a deep 
furrow covered by the marginal and first temporal convolutions passes to some 
extent in the direction of the upper space and backward, so that in the brain 
it almost reaches the transverse plane of the angular convolution. The adjoin- 
ing illustration (Fig. 141), which is taken from Henle's " Anatomy/' gives us 
an idea of these relations. 

The importance of the island for the function of speech, everywhere rec- 
ognized, permits us to conclude that it is the objective point for the association 
tracts, and the area where the two centers of speech, and presumably also 
the different areas of the speech region, unite with each other. In fact the 
finer anatomy of the region of the island permits no other explanation than 
that we are dealing with an actual association center. This is obvious, not 
merely because the three deep spaces which bound the island show everywhere 
at their bases the well known U-shaped, so-called, fibriae propriae or laminae 
22 



314 THE SYMPTOM-COMPLEX OF APHASIA 

arcuatae Arnoldi to be richly developed, but also because of the typical struc- 
ture of the cortex of the island. As is well known, the extension of the cortex 
of the island reaches the external border of the largest ganglion of the brain, 
the lenticular nucleus. Between the lenticular nucleus and the cortex of the 
island are found two layers of white substance, of which that adjoining the 
lenticular nucleus shows a very delicate internal structure known as the cap- 
sula externa, that belonging to the cortex of the island a much coarser one 
following its outline, and designated as capsula extrema. Between the capsula 
externa and capsula extrema we note a structure of gray substance, the claus- 
trum, which is found nowhere else in the brain. This compartment-shaped 
ganglion, formed like the cortex of the island, is broader from above down- 
Upper space 




Anterior space 



Lower space 



Fig. 141. 

ward, while it narrows longitudinally ; as Meynert has said, with its processes 
pointing toward the periphery it wholly disappears in the first embryonic 
convolution; he tells us, too, that it consists exclusively of spindle or associ- 
ation cells, and is nothing more than a cortical layer of unusual development 
and deeply penetrating the medullary substance which everywhere consists of 
spindle or association cells. In fact it disappears at the so-called foot of the 
island, i. e., at the lower point of the junction of the radially situated convo- 
lution of the island with some of the cortex of the island as well as with the 
substance of the amygdalate nucleus. Besides the cortex of the island and its 
claustrum, there are still two important association bundles which must be 
considered in the anatomy of the speech regions. One of these is the fascicu- 
lus uncinatus, penetrated by the widened base of the claustrum which divides 
lit into several layers, and this suggests the thought of a connection between 
the cells and fibers. If the cortex is detached, the chief mass of the associa- 
tion bundle is found at the foot of the island and further toward the interior, 
and here is best seen its hook-shape which reminds us of the composition of 



DISTURBANCES OF WRITTEN LANGUAGE 315 

the laminae arcuatae. From this mixed piece the bundle splits and bifurcate? 
into the two cerebral lobes (whose shortest combination it represents), the 
frontal and the temporal lobes. Some portions of it certainly extend to the 
third frontal convolution, including Broca's convolution and the speech region 
of the first temporal convolution. 

While the fasciculus uncinatus appears to unite by the shortest connec- 
tion with parts of the frontal and temporal lobes, the second bundle, or 
fasciculus arcuatus, is not actually a special bundle, but a general system of 
association fibers of the convex surface of the brain having a sagittal direction. 
Nowhere are the ganglion cells included in this. The anatomical conditions 
of the brain are such that a special bundle of association fibers is most read- 
ily produced where the foot of the corona radiata passes from the internal cap- 
sule, therefore above and outwardly from the lateral edge of the lateral ven- 
tricle to the upper border of the lenticular nucleus. As it is also contained 
in the substance which bounds the Sylvian fossa, it is perceptible in the hard- 
ened brain if the cortex of the upper space with its fibriae propria? is detached. 
This will then convince us that a special bundle penetrates the deep medullary 
substance of the marginal convolution as far as the first temporal convolu- 
tion, encircling the posterior process of the Sylvian fissure in an arch. The 
portion of the arch extending sagittally in the former direction lies medially 
from the just described path, and the interparietal furrow penetrating deeply 
into the convexity as well as the posterior processes of Burdach's upper space 
narrowly constricts this portion. In this area fibers which originate from the 
marginal convolution and the posterior third of the temporal convolutions 
no doubt join the arch bundle. The medullary substance of the lower parietal 
lobe consists largely of these fibers, which are closely pressed together in an 
extremely narrow space. When we consider that a part of the anterior por- 
tion of the fasciculus arcuatus extends to the top of the lateral ventricles above 
the lenticular nucleus, and that its sagittal continuation for the parietal and 
occipital lobes is first narrowed by the previously described furrows, the usu- 
ally very deep posterior end of the parietal or first temporal furrow at once 
makes it obvious that the medullary substance of the lower parietal lobe in a 
comparatively small space interrupts a large part of the association fibers con- 
tained in the fasciculus arcuatus. 

I will limit myself to these anatomical remarks which mainly show the 
peculiarity of the lower parietal lobe (that is, the angular convolution) which 
must contain a very important passage for the region of speech. Besides this 
passage to the interior, i. e., toward the ventricle, we find anatomically three 
sharply demarcated layers, namely, the most external one, that of the lower 
longitudinal bundle, then the so-called optic radiation of Gratiolet, and, finally, 
the tapetum of the corpus callosum, which together form the sagittal, medul- 
lary layer of the occipital lobe ; in this restricted space lie tracts of such varying 
importance that the appearance of a center may readily be simulated, and 
in my opinion this is the origin of Dejerine's erroneous conception. To those 
more deeply interested in these anatomical relations I recommend Dejerine's 
" Anatomy of the Central Xervous System " and my " Atlas of Sections of 
the Brain." Fig. 142 is taken from the work of v. Monakow, and shows a 
horizontal section through the left hemisphere of the brain at the height 
of the first temporal convolution, the third frontal convolution, and the 



316 



THE SYMPTOM-COMPLEX OF APHASIA 



splenium of the corpus callosum. The three obliquely shaded areas upon the 
convexity show the seat of lesion in motor and sensory aphasia and in pure 



Motor Aphasia 



Sensory Aphasia 




Pure Alexia' 



Germ of the 
^"Corpus Callosum 



Splenium of the 
//Corpus Callosum 



Fig. 142. 



alexia. In contrast to the oblique shading, we observe in the illustration a 
long-stranded fiber mass composed of three bundles; it shows diagrammat- 



DISTURBANCES OF WRITTEN LANGUAGE 317 

ically the above mentioned sagittal medullary portion of the occipital lobes 
which in the hardened preparation is likewise sharply demarcated from the 
medullary layers of the temporal and lower parietal lobes, as in the illustra- 
tion. The circular shaded area which is noted in the lower parietal lobe, 
v. Monakow considers to be the interruption of these three fiber layers which 
is a prerequisite for the occurrence of pure alexia, and which, according to 
his conception, arises from the destruction of the connecting link between the 
sensory speech center and the two occipital lobes. 

As already indicated, I concur in v. Monakow's opinion that Dejerine's 
assumption that the cortex of the angular convolution is the seat of a uni- 
lateral writing picture center is not at all proven. Dejerine's observations 
do not permit this conclusion, but only prove that the destruction of this 
region invariably and severely impairs the power to write, and the degree 
of this action varies according to whether the deeper layers alone or simul- 
taneously the superficial layers of the angular convolution are implicated. In 
the first case pure alexia results, in the second, alexia and agraphia. But in 
either case the cortex of the angular convolution may not be at all involved. 

The anatomical relations which have been outlined merely permit us to 
conclude that the medullary substance of the lower parietal lobe, to a com- 
paratively slight extent, severs all of the connections between the centers 
of speech and the two occipital lobes. In the near future autopsy findings 
must be utilized for the study of these points, and the following anatomical 
tracts, which I shall briefly enumerate, then come under consideration : First, 
the sagittal medullary substance of the occipital lobe with its three individual 
layers. The middle layer is the so-called " optic radiation of Gratiolet," the 
one which presents the most marked clinical symptoms, namely, the accom- 
panying hemianopsia. The internal layer, the so-called tapetum, originates 
in the splenium of the corpus callosum, and contains at least two known 
tracts, namely, that which connects the two temporal lobes with each other, 
and that which unites the temporal lobe of one side with the occipital lobe 
of the opposite side, the forceps tapetum tract of II. Sachs. It also appears 
certain that the latter tract, which corresponds to the crossed optico-acoustic 
commissure of Bastian, passes the splenium of the corpus callosum. The 
external layer, as the fasciculus longitudinalis inferior, permits a free com- 
munication between each of the temporal lobes and the occipital lobe of the 
same side; it may, therefore, perhaps contain Bastian's optico-acoustic com- 
missure of the same side. According to Probst's recent investigations it con- 
sists exclusively of corona radiata fibers which pass to the optic thalamus; 
this, however, I cannot confirm. It may be admitted for the upper half of its 
extension, but for the rest we acknowledge only its importance as an asso- 
ciation tract between the temporal and occipital lobes. The sagittal medullary 
layer with its three component parts is situated not only within the lower 
parietal lobe but also within the sensory speech center in the posterior third 
of the first temporal convolution. Secondly, the true medullary substance 
of the angular convolution lies not only external to, but also above, the sagittal 
medullary layer. In a diffuse medullary mass, which cannot be further ana- 
lyzed, it contains at least two principal constituents, namely, the fibers of 
the corpus callosum originating in the splenium of the corpus callosum which 
comes from the forceps major, bundles of which permeate the sagittal medul- 



318 THE SYMPTOM-COMPLEX OF APHASIA ' 

lary layer, and the above described component parts of the fasciculus arcuatus. 
This is composed of all the tracts which unite the motor cerebral regions 
with the occipital or lower parietal lobes of the same side, and is the shortest 
means of communication between the sensory speech center and the lower 
parietal or occipital lobes. The above considerations make it seem to a high 
degree probable that, besides destruction of the sagittal medullary layer, the 
intactness of the latter tracts contained in the medullary substance of the 
angular convolution is a prerequisite for the production of pure alexia. 

Autopsy findings in pure word blindness combined with hemianopsia do 
not always reveal merely one focus localized as in v. Monakow's illustration, but 
often several foci interrupt the optic radiation, that is, their point of origin 
in the occipital lobe, as well as portions of the fibers of the corpus callosum. 
The splenium of the corpus callosum is especially involved, and it is pointed 
out that the same artery — the posterior cerebral — supplies the occipital lobe 
and the splenium of the corpus callosum, the regions belonging to the area 
of softening. Which portion of the corpus callosum is interrupted, whether 
the forceps major which unites symmetrical areas of the convexity or the 
forceps tapetum tract of H. Sachs, is still doubtful. 

The tapetum of the temporal lobes can no longer be supposed to exist in 
the area designated by v. Monakow (identical with that of Dejerine), for it 
lies too near the occipital region; hence my thorough description, previously 
given. This, however, does not preclude the consideration in the clinical 
picture of both tracts with their variations; for example, the ability to copy 
may be due to this. 

In the relatively rare form of pure, isolated, literal agraphia, the approxi- 
mately definite seat of the focal lesion may be determined by the comparison 
of a few positive cases. This corresponds to the point where the sensory 
corona radiata of the extremities decussate certain deeply situated portions 
of the fasciculus arcuatus, its frontal plane lying anterior to the angular con- 
volution in the region of the marginal convolution, its horizontal plane being 
higher than the lesion of the medulla of the angular convolution found in pure 
word blindness. Explicit details will be found in the report of my case. 

The complexity of the anatomical relations of the regions of speech ren- 
ders it obvious that objections may often be made to the meager description 
of the pathologic findings in those infrequent cases which have been thor- 
oughly investigated clinically and reported by reliable observers. A purely 
casuistic standpoint does not affect the utilization of autopsy findings, for, 
on the contrary, we then find that almost all such experiences as I considered 
of general application are opposed by other observations. The unbiased study 
of C. H. Bastian's valuable report, noted for its rich material, and the most 
exhaustive compilation on the subject since the time of Kussmaul, forms the 
best proof of this statement. In spite of this, it is a great satisfaction to 
me to maintain the operation of a fixed law in this realm, which is also the 
standpoint of A. Pick, v. Monakow, and Dejerine, investigators who, in addi- 
tion to their undoubted clinical ability, have at the same time shown them- 
selves to be masters of the structure of the brain. The following remarks are 
based partly upon the observations of these authors, partly upon my own expe- 
riences and conclusions. 

As to the exact limits of the motor and sensory speech center, I shall 



DISTURBANCES OF WRITTEN LANGUAGE 



319 



confine myself to the description given above. The anterior obliquely shaded 
area in Fig. 142 shows the usual extent of the destruction in cases of pure 
motor aphasia ; this, according to our nomenclature, at the same time includes 
verbal agraphia. Most cases of motor aphasia without agraphia have proven 
to be subcortical motor aphasia. According to the findings at hand, we must 
also reckon with a partial destruction of Broca's convolution, a damage to 
the lower part of which toward the Sylvian fossa causes word mutism; the 
integrity of the remaining parts, according to v. Monakow, permits the power 




Fig. 143. — CI, Claustrum; Ci, internal capsule; Li, lenticular nucleus; y, fasciculus arcuatus; 
a, secondary degeneration after cortical motor aphasia. (After v. Monakow.) 



to write. Fig. 143 shows the seat of the lesion in cases of cortical motor 
aphasia which, exceptionally, are not combined with agraphia. 

The secondary degeneration which persists after cortical motor aphasia 
has its definite seat in the internal capsule, indicated by a t in the accompany- 
ing figure. The accurate seat of the slightest destruction which causes sub- 
cortical sensory aphasia or pure word deafness can unfortunately be proven 
in only a single case, but in this, in my opinion, absolutely beyond doubt. It 
is the case of Liepmann, previously mentioned, the autopsy report of which 
was recently published. On account of accompanying conditions this proof 
was merely by exclusion; the destruction of the corona radiata of the tem- 
poral lobes at the point where it unites with the island of Reil was evident, 
hut this proof was convincing because at the same time a secondary degenera- 



320 THE SYMPTOM-COMPLEX OF APHASIA 

tion of the tapetum of the temporal lobe of the opposite side, the right hemi- 
sphere, was found which could be looked upon only as an old lesion in the 
medulla of the temporal lobe of the left side, since the cortex of the temporal 
lobe and the entire occipital lobe upon the left side were well preserved, the 
right hemisphere alone showing recent destruction. In explaining his case 
Liepmann quite properly refers to H. Sachs who, on account of the narrow 
compressed course of the corona radiata of the temporal lobe between the poste- 
rior and lower processes of the third link of the lenticular nucleus and the 
tail-piece situated upon the roof of the lower horn of the caudate nucleus, 
stated that subcortical sensory aphasia is most readily produced by an injury 
of this area. 

Many years ago I quoted experiences 1 which show that within the poste- 
rior processes of Burdach's upper space must lie fibers whose injury produces 
motor aphasia, I believed at that time that these fibers extended to the inter- 
nal capsule and formed a special motor speech tract, that they therefore con- 
tained the fibers of Broca's convolution which projected to the nuclei of the 
bulbar nerves. After much experience had taught me that sensory aphasia, 
if combined with motor aphasia, is usually overlooked, and that lesions of 
the internal capsule do not produce subcortical motor aphasia, I finally came 
to the conclusion that these experiences refer to cases of transcortical motor 
sensory aphasia, v. Monakow, who still adheres to the opinion that the im- 
pulse for spontaneous speech must be transmitted over the sensory speech 
center, positively declares that except the cortex of the island the fasciculus 
arcuatus is the only association tract between the motor and sensory speech 
centers. Aside from this view, Heilbronner has recently and quite properly 
emphasized that the richness of the fiber communications between the tem- 
poral lobes and the third frontal convolution forces us to consider the tem- 
poral lobe as the chief point of origin of the transcortical fibers for Broca's 
center. In deciding this question I attach great weight to a case reported 
by Heubner which admits of no other explanation than that the principal 
constituents of the fasciculus arcuatus were separated; certainly the layer in- 
tended for the medullary substance of the first temporal convolution must 
have ruptured. Clinically, however, this case unquestionably presents the 
characteristics of sensory as well as of motor transcortical aphasia. In this 
case there was an area of so-called cortical softening which took the form of a 
furrow, and the marginal convolution and the posterior third of the first tem- 
poral convolution almost appear to be sequestered from the surrounding cortical 
substance; this proves that foci of softening may be of remarkable form and 
extent, and that the conditions produced by nature in pathologic cases may 
present the most manifold combinations, and admit of the most instructive 
applications. 

In regard to diagnosis, I refer to the leading facts in the general pathol- 
ogy of the brain, according to which, when focal symptoms are present (and 
to these the phenomena of aphasia in the widest sense belong) we must deter- 
mine whether we are dealing with direct, indirect, or transitory focal symp- 
toms. I shall not here describe these, as I take it for granted that they are 
known. But it appears to me that the transitory focal symptoms are not 

i"Lelirtmch der Gehirnkrankheiten," 2. Bd. ; 173, 176, 179. 



DISTURBANCES OF WRITTEN LANGUAGE 321 

always sufficiently appreciated. I must therefore state that these phenomena 
are caused by the occlusion of an artery with succeeding compensation. The 
affected cortical area is then periodically in a state of ischemia, but does not 
soften because the circulation is re-established. Substantive and acute focal 
symptoms which disappear in the course of one to three weeks must always 
awaken a suspicion of this sort of development. In an individual case, pre- 
ceding transitory focal symptoms may enable us to make a positive differ- 
entiation between embolism and cerebral hemorrhage, which in the majority 
of cases is otherwise impossible. The diagnosis of subacute and chronic focal 
diseases and also of aphasia is based upon the same common principle, i. e., 
the utilization of the accompanying general symptoms. The same is true of 
the diagnosis of aphasic disturbances occurring in meningitis and progressive 
paralysis ; here I must emphasize that motor as well as sensory aphasia is fre- 
quently observed after paralytic attacks, and these usually share the favorable 
prognosis of the focal symptoms. In all cases, the preliminary question of the 
right-handedness or left-handedness of aphasic disturbances in childhood from 
which the patient recovered, the full possession, the absence, or an impair- 
ment of the ability to write prior to the attack, must be decided. Thanks to 
the researches of A. Pick, the processes of a chronic and progressive disease 
of the brain, namely, of simple cerebral atrophy usually of senile or alcoholic 
etiology, have recently been made susceptible to diagnosis. A. Pick has shown 
that, when general cerebral atrophy occurs, localized atrophies of more decided 
character may betray themselves as focal symptoms; one or both temporal 
lobes, and combined with this or even by itself the first temporal convolution, 
may be the seat of extreme focal atrophy. This shows itself by a diminution 
in the size of all the constituents of the convolution, of the cortex as well 
as of the medullary substance. The resulting pathologic picture is that of 
transcortical sensory aphasia, the occurrence of which is unilateral focal atro- 
phy, although indicative of a slowly increasing process, is comprehensible 
because the generalized atrophy, although only slight, makes compensation 
by the other hemisphere difficult. A. Pick and Dejerine have given reliable 
descriptions of cases which, on account of accompanying extreme deafness, 
were considered to be subcortical sensory aphasia or pure word deafness. 
Liepmann, however, has proven that the clinical picture was that of trans- 
cortical sensory aphasia, but the conspicuous bilateral deafness rendered the 
diagnosis of the cases difficult. A more extreme bilateral deafness, especially 
if the atrophy is chiefly seated in the comparatively small sensory speech cen- 
ter at either side, may really lead to absolute pure word deafness, which of 
course prevents us from recognizing a simultaneously existing transcortical 
sensory aphasia. We must therefore reckon with the occurrence of such tran- 
sitional cases. That the cortical function from the periphery can still be 
determined when a process almost uniformly implicates all parts of the con- 
volution which suffers damage, although one and the same medullary layer 
of the convolution contains subcortical as well as transcortical fibers, is read- 
ily explained by the well known persistence of sensory memory pictures once 
acquired, and their faculty of complete reproduction even when very deficient 
impulses are transmitted in a distorted way from the periphery. The most 
familiar example of this is the fact that the tactile property of one hand 
may be retained even when there are serious sensory disturbances of both 



322 THE SYMPTOM-COMPLEX OF APHASIA 

nerve tracts, the median and ulnar, so that only very imperfect tactile impres- 
sions are transmitted from the periphery to the cortex of the brain. Monakow 
takes the opposite standpoint, and believes that this specific function of the 
cortex of the sensory centers may suffer damage by a slight disturbance in 
function; upon the basis of much experience I must deny this. The preced- 
ing explanation embodies the important diagnostic law that slowly develop- 
ing sensory aphasia of a transcortical nature (the recognition of which may 
be rendered difficult by extreme deafness), always permits the assumption 
of a localized atrophic process in the first temporal convolution upon the left 
side, and we here have a rare example of a definite focal symptom permitting 
the recognition not only of the area but also of the nature of the pathologic 
process. Sensory aphasia due to embolism or thrombosis always occurs sud- 
denly, and often with more or less conspicuous symptoms of apoplexy. 

In the later stages of motor aphasia it will be noted that syllable stumbling, 
and in sensory aphasia a difficulty in finding words for objects shown, usu- 
ally persist, and if there are deeper lesions of the affected centers further com- 
pensation is not to be expected. As a rule we cannot utilize the symptom of 
difficulty in word finding for a focal diagnosis ; on the contrary, it is often 
merely the expression of a general impairment of function, which may at 
different times develop to a different degree. A. Pick has shown that im- 
provement of sensory aphasia occasionally occurs by a stage of agrammatism, 
i. e., by a disturbance of speech in which merely the essential parts of a sen- 
tence are used, without connectives or conjunctions, and without any regard 
to the tense of verbs, as is common in sending a telegram, or with a very 
rudimentary knowledge of a foreign language. This fact also may be of 
value in the diagnosis. 

In by far the great majority of well developed cases we must remember 
that of the two opposite types of arrested or still fluent speech, only the for- 
mer can be designated as motor aphasia. With a well retained power of 
speech, paraphasia of varying grades is the most conspicuous symptom, ina- 
bility to understand what has been said is usually not appreciated, even by the 
most expert observers, and in every case is a symptom that must be sought 
for and determined with the utmost care. Apparently the prevalent opinion 
in regard to the present situation, and its power to readjust itself, is the 
reason why such a gross defect is so frequently overlooked. This may very 
readily occur if partial compensation has taken place for ordinary questions 
or commands. If motor aphasia at the same time exists, naturally an opinion 
as to whether spoken sounds are understood is even more difficult, therefore 
we must be the more cautious in examining for this symptom. In regard 
to disturbances in writing, I refer to my above descriptions. Any one desir- 
ing additional instruction for the diagnosis of finer speech disturbances and 
their importance as focal symptoms will find these in A. Pick's book * which 
indicates a decided advance in this branch of disease. 

i " Beitrage zur Pathologie und pathologischen Anatomie des Centralnervensystems," 
Berlin, 1898. 



TREATMENT 323 



TREATMENT 



In the therapy of aphasia I may be brief, as this can only come into ques- 
tion when the focal symptom exists in an otherwise healthy brain. 

The best treatment is exercise, which in sensory aphasia will of itself 
produce a gradual compensation of the defect. This spontaneous improve- 
ment is lacking when there are special circumstances, for example, an accom- 
panying general atrophy of the brain or a bilateral disease of the temporal 
lobe. In motor aphasia, as has already been observed, even systematically con- 
ducted exercises are usually of little use. But the successes in the treatment 
of motor aphasia which Dejerine and Thomas report indicate that, under 
some circumstances, and after systematic practice, speech movements may be 
understood by the sense of sight, therefore all persons who understand this 
method should be advised to employ it. The few physicians who have de- 
voted themselves to this specialty and teachers in deaf-mute institutions will 
be the most suitable instructors. In pure word blindness we usually succeed 
in teaching the patients to trace writing with their fingers, and by this method 
they learn to write themselves, and also learn to read. The patient whose 
history I related at the beginning of this article has in this way learned to 
recognize most letters and to read some words. But the method presupposes 
such attention, energy and endurance that compensation of the defect is only 
attainable in very exceptional cases. 

My description will have given the impression that the aphasic symptom- 
complex, while to-day incomplete, forms a full, exceedingly rich, and inter- 
esting chapter in cerebral pathology; not in the remotest degree do I claim 
that it is complete, but I limit myself in the present state of our knowledge 
to presenting its principal characteristics, so that to some extent we may learn 
to recognize the outlines of this large and complicated structure. A few his- 
toric notes I have used for the purpose of elucidating certain points of view, 
which have so far aided in rearing this structure. 

As in every investigation not yet concluded, we have here found that our 
field of activity appears to widen more and more, that even now the mind 
can hardly grasp all the facts that have come to light, and new questions 
constantly arise and demand an answer. If we penetrate beyond the true 
aphasic symptom-complex, we meet with ever expanding fields which are 
actually the offspring of aphasia; I need only mention mental blindness, 
asymbolia and apraxia. I am not claiming too much when I maintain that 
the pathologic picture presented by these affections would not have been cor- 
rectly observed, described, nor understood if the points of view which I have 
delineated as the true kernel of the teaching of aphasia had not facilitated 
their understanding. Everywhere we meet with complicated psychical phe- 
nomena which are not infrequently also observed as partial symptoms of true 
mental disease. These form, in fact, a borderland between focal diseases of 
the brain and mental diseases. But even the restricted realm of aphasia fur- 
nishes in itself such transitional cases, which lead us to the conclusion that 
so-called mutacism, the strange muteness of patients with mental disease, is 
often only a peculiar form of transcortical motor aphasia. A minuteness of 
clinical observation and analysis, of which we may be justly proud, is begin- 



324 THE SYMPTOM-COMPLEX OF APHASIA 

ning to form a special pathology for localized psychical disturbances. I need 
mention only the researches of Lissauer into mental blindness, and of Liep- 
mann into the pathologic picture of unilateral apraxia, both of which may 
be termed classic. A purely descriptive presentation of mental diseases which 
would include all of this recently acquired knowledge might be attempted. 
If the value of a scientific hypothesis depends upon the question in how far 
it renders research possible and leads to new questions, we must admit that 
the much disputed and actually the most disputable question, the transcor- 
tical form of aphasia, owes its origin to a valuable and at present indispensable 
hypothesis, which justifies and maintains its position. Moreover it gives me 
satisfaction to state that the best text-books, such as Oppenheim's, v. Mona- 
kow's and Goldscheider's, practically recognize the transcortical forms of 
aphasia, i. e., as indispensable for the symptomatologic differentiation of cer- 
tain pathologic pictures. 



THE NATURE AND THE TREATMENT OF DISTURBANCES 

OF SPEECH 

By H. GUTZMANN, Berlin 

Disturbances of speech are divided by a natural method into three prin- 
cipal groups: (1) Peripheral impressive; (2) Central; (3) Peripheral dis- 
turbances of expression. As the faculty of speech includes not only the ex- 
pression of thought but the prior understanding of what has been spoken, we 
designate as peripheral impressive those disturbances of speech in which the 
means by which we learn to speak (upon which is based the entire apparatus 
of speech the individual parts of which are so difficult to recognize) are lim- 
ited, are difficult of apprehension, or are even entirely absent. 

PERIPHERAL IMPRESSIVE DISTURBANCES 

In peripheral impressive disturbances of speech we ordinarily consider only 
the method by hearing and, as a matter of fact, this is the most common and 
the best way of learning to articulate. Children who from birth or from 
infancy have defective hearing or are deaf do not learn to speak at all ; they 
are mute: Deaf-mutism, surdo-mutitas. In these cases, therefore, the areas 
of the brain which we assume to be the seat of sensory and motor speech are 
entirely unused, and do not develop. It is not always easy to determine what 
sense of hearing is possessed by a speechless child. While in older children 
the hearing may be accurately tested with tuning-forks, in younger children, 
under six or even seven years, this is not the case. I have frequently observed 
gross errors in testing the hearing of little children, and therefore I advise 
the utmost care in making this test, and that we base our conclusions upon 
what the parents themselves have noticed in regard to the hearing of the child 
rather than upon the results of our own examination. 

I once examined a little girl aged 4% years. The parents desired my opinion as 
to whether or not the ehild "was a deaf-mute. The little girl, when looking at pictures 
or other objects as well as when at play, was often so engrossed that it was difficult to 
divert her attenion to any other channel. Her gait was steady, not dragging as is so 
frequently the case in deaf-mute children, nor did the child otherwise give the impres- 
sion of being a deaf-mute. What we are to understand by giving the impression of 
being a deaf-mute cannot be described. There is no doubt, however, that congenital 
affections stamp a general impress upon the patient which cannot fail to be recognized 
by one who has observed many such persons. I was born in a deaf-mute institution 
and grew up among deaf-mutes, and therefore I am familiar with the general appear- 
ance of deaf-mute children without being able to define accurately its individual char- 
acteristics. This child did not impress me as being a deaf-mute, and when I stated this 
to the parents they declared that neither did they believe the child to be deaf. They 

325 



326 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

related to me a number of facts they had observed in the child: She could hear the 
tinkle of a bell and the ringing of a bell; she sometimes heard what was spoken and 
proved that she understood by some corresponding action, without any motion or indi- 
cation on the part of the speaker which might aid the understanding. The child also 
made sounds of a senseless character while playing with her doll, a sequence of sounds 
which clearly imitated the speech melody of those about her ; that is, sometimes high, 
sometimes low, sometimes loud, sometimes soft. At other times her reaction to impres- 
sions of hearing and also sometimes to impressions of feeling was very slight. 

In the meantime the child was occupied with a picture-book which I had handed to 
her, and was so busy looking at the pictures that when from behind her I suddenly blew 
at her with my mouth she showed no reaction to this wave of air. Now we know that 
even adults, if greatly preoccupied, react but slightly to external impressions, and we 
say of them that they are entirely oblivious of what goes on about them. Yet in these 
cases there is no disturbance of sight or hearing. From these observations, therefore, 
I came to the conclusion that the child could hear, and that her deafness was not deaf- 
mutism, surdo-mutitas, but idiopathic dumbness, audi-mutitas (without deafness). 

Only after the examination did the parents tell me that they had previously been 
to an ear specialist, and that after a long and thorough examination he had declared 
the child to be perfectly deaf. During the examination by the ear specialist, while the 
child standing at the window was completely engrossed with something going on in 
the street, the physician suddenly made a very loud noise with some instrument behind 
her. She did not turn around at this, and the ear specialist therefore assumed that she. 
did not hear. A similar result was obtained by a test with the tuning-fork. In spite 
of this opinion I believed my diagnosis to be correct, and I was not a little pleased 
when six months later the little girl was brought to me again, and I found that she 
spoke as well as any child five years of age. The power of speech appeared spontaneously 
soon after the consultation, and had developed very rapidly. 

I have detailed this case minutely because it is a classical case — idiopathic 
dumbness (without deafness) — and because it clearly demonstrates that in 
children of this age even the most careful examination by a prominent ear 
specialist may lead to erroneous conclusions. I must remark briefly that the 
child was quite intelligent. The only conspicuous feature in the case was the 
child's great preoccupation when at play or engrossed with anything which 
had attracted her attention. 

If children are really deaf, other errors may arise; it may be supposed 
that they hear. As a proof of hearing it is often stated that such children 
hear a bell. We know, however, that extremely loud and shrill bell sounds are 
frequently noticed by deaf-mute children. It is also declared that noises in 
an adjoining room are heard. This, however, does not necessarily indicate that 
they hear ; the knowledge may be conveyed by sensation, and it is by no means 
a rare occurrence for children who are very deaf to notice the moving of a 
chair in the next room more readily than those of normal hearing. A trust- 
worthy sign of deafness in children is an immediate reaction to impressions 
of sensation while there is no response to impressions of sound. A congenital 
deaf-mute will always turn when the foot is stamped upon the floor, because 
this has been to him a signal from his earliest years, and deaf-mute malingerers 
can readily be unmasked if, after a number of sound tests have been made, 
they do not turn when this experiment is tried, but believe that they must 
feign not to hear the sound. Therefore the observations of parents must be 
very carefully tested if upon these we must base our opinion as to the faculty 
of hearing or the deafness of children. A valuable proof of the sense of hear- 
ing in a child is that in a lalling play it attempts to imitate the speech melody 
of those around it. 



PERIPHERAL IMPRESSIVE DISTURBANCES 327 

Let us assume that we are actually dealing with a deaf-mute child, and 
that the central areas for motor as well as perceptive speech are undeveloped, 
since these usually develop only by way of hearing. This peripheral impressive 
way is, however, not the only one which we control. The second method for 
perception is by the eye. This is therefore utilized to teach children the mo- 
tions of speech. The third method is by sensation, and by this they are enabled 
to recognize the vibration of the vocal cords corresponding to sounds which 
are readily transmitted to the entire thorax, and may there be perceived by 
the hand (pectoral fremitus). Feeling and sight are the methods of percep- 
tion which remain to the deaf-mute child, and the German method of edu- 
cating deaf-mutes by these means not only promotes the complete structure 
of speech, but by reading the movements of speech from the mouth an almost 
complete perception of spoken language is attained. Therefore the sensory 
speech center in the deaf-mute must be sought in the optic cortical fields of 
the occipital brain, while the motor speech center is most likely situated in 
the same area as in a person who hears. 

An illustration will show how speech is taught in deaf-mute institutions. 
The teacher takes one hand of the deaf-mute child and places it upon his own 
larynx while the other hand of the child is placed upon its own larynx. Then 
the teacher opens his mouth and articulates a. The child sees the opening of 
the mouth, it feels the vibration of the larynx, and, as is readily noted in deaf- 
mute children, its speech producing organs not being paralyzed and its vocal 
organs being capable of function, it imitates as nearly as possible what it sees 
and feels. Perhaps the vowel which first results is not a pure and resonant a, 
but by modulating the height and depth, the strength and weakness of the 
tone, which may be also felt, it gradually becomes purer and clearer. Now 
the teacher closes his lips and places the hand of the child upon the roots 
of his nose; he enunciates a prolonged m, the child feels the vibration of the 
walls of the nose and attempts to imitate this by placing its other hand upon 
its own nose. This attempt also is soon successful, and now the teacher com- 
bines the consonant with the vowel and says " ma." If he repeats this syllable 
twice the child has learned its first word, of course only in the form of a purely 
mechanical articulation: "Mama." Xow the conception alone is wanting. 
This is conveyed by showing pictures which represent a mother in the family 
circle, for example, waiting upon the children, distributing food, etc. The 
intelligent deaf-mute child understands immediately what is meant by the 
sequence of sounds, and at once makes use of it. 

It is evident that this method of building up the power of speech is 
difficult and circuitous but absolutely certain, and psychologically it is the 
only natural way. It has always been incomprehensible to me that this 
German method of educating deaf-mutes could be designated an artificial 
one. Certainly it is the most natural and progressive way which can be 
imagined. 

It must be mentioned that in deaf-mute children some power of hearing 
may still be frequently found, particularly in congenital deaf-mutes; and it 
can scarcely be doubted that the utilization of this power facilitates the devel- 
opment of speech, so increasing the volume of sound and the purity of tone 
that under some circumstances the perception of speech also may be promoted. 
On the whole, in deaf-mute children perception will be chiefly by sight, in the 



328 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

form of reading from the lips, since only in rare cases is the sense of hearing 
so acute that perception by the ear is practicable. 

When impairment of the hearing and deafness occur earlier than the four- 
teenth year, the faculty of speech may be completely lost. Ordinarily it is 
absolutely lost if deafness occurs before the seventh or eighth year of life. 
If deafness or partial loss of hearing occur later, the power of speech once 
developed may remain more or less perfectly. If impairment of hearing or 
deafness appear in the adult his perception of spoken language and also his 
own power of speech suffers, but by no means in proportion to the loss of 
hearing which has developed. Persons who have become wholly deaf may, 
nevertheless, have the faculty of almost normal speech, and others who reveal 
but a moderate loss of hearing may articulate so imperfectly and so indis- 
tinctly that conversation with them is almost impossible. Their difficulty in 
speech does not depend upon the seat of the pathologic process. If the impair- 
ment of hearing is so extreme that in ordinary intercourse perception by the 
ear is insufficient, such persons must learn to read from the lips. With suffi- 
cient practice all articulate sounds which form words and sentences may be 
read from the movements of the lips, the cheeks, the lower jaw, and the floor 
of the mouth. In these persons a mere displacement of the sensory speech 
center into the central cortical field of the opticus has taken place. Whenever 
necessary in such cases speech production is improved by employing the sensory 
tracts. In teaching such persons by sensation the difference between height 
and depth, strength and weakness of tone, they are taught to exercise a con- 
trol over their own speech which had previously depended almost absolutely 
on hearing alone. 

This finishes our discussion of peripheral impressive speech disturbances. 
It might be supposed that loss of the sense of sight would result in impair- 
ment of speech, and in fact it has been maintained that children who are born 
blind learn to speak later than those in otherwise similar circumstances who 
see. It is also possible that a congenital or acquired deficiency of tactile and 
muscular sense in the organs of speech may interfere with the development 
of speech; for a coordinated movement of the parts is only conceivable when 
sensation is undisturbed, and we know that the ataxic gait of the tabetic is 
the consequence of disordered sensation. As has been stated these are possi- 
bilities, but I must mention for completeness' sake that they practically come 
into consideration as disturbances of speech only because this derangement 
is a secondary condition. 

Among the central disturbances of speech is aphasia, the nature and forms 
of which will be discussed in another chapter (Wernicke). But I should 
like to call attention to its nomenclature, and to state that aphasia — that is, 
speech which has been lost — can occur only in those who formerly possessed 
this faculty. The inability of the deaf-mute child to speak cannot be called 
aphasia because it has never possessed the power of speech, and the same is 
true of inability to speak in a child in whom hearing has been inhibited. In 
these cases I shall constantly refer to mutitas, and it must be borne in mind 
that I previously differentiated between surdo-mutitas and audi-mutitas. 
There is no possibility of such a condition as congenital aphasia. 

The aphasias which develop in consequence of apoplexy, embolism, etc., 
may be divided into two great groups, sensory and motor. In both forms 






PERIPHERAL IMPRESSIVE DISTURBANCES 329 

good results may be expected from exercises in speech persistently practised. 
What has been lost may again be acquired provided that the general intelli- 
gence of the patient has not suffered too severely from the attack, that the 
destruction of the center is incomplete, and that the patient is not too old. 

We have frequently been content with instituting practice in speech at 
any time after the attack, and of course it soon became evident that success 
was to be expected only when the aphasia had existed for a short time. Appar- 
ently, the spontaneous improvement noted in by far the majority of cases of 
aphasia has either been left entirely out of consideration or not accurately esti- 
mated. If we desire to gauge correctly the value of practice in aphasia, we 
must try it in aphasia which has continued unchanged for a long time. From 
professional policy I advise that only this method of practice be instituted in 
such aphasias. The patients themselves will then correctly appreciate the 
results. Moreover, it is wise not to subject to practice too early a brain that 
has only recently recovered from a severe disease, or one in which the patho- 
logic process itself has perhaps not yet been arrested. 

For lip-reading in such aphasias we must necessarily begin with the ele- 
ments of speech, and must first systematically practise elementary sounds. We 
presuppose that the patient with motor aphasia is absolutely unable to speak. 
We naturally begin with the simplest sounds, and, according to experience, 
these are the sounds in the first realm of articulation, those made with the 
lips and the upper teeth. We first practise on the vowels, beginning with 
a for the production of which the mouth is wide open and the voice is intonated. 
For the vowel o the lips are contracted in an oval shape ; and this contraction 
is still more marked with the vowel u. For e the mouth is opened more 
widely, and in i still more widely. We invariably observe that the vowel a 
is the easiest for the patient with motor aphasia to enunciate, that the vowels 
o and e necessitate a moderate muscular contraction of the lips which is 
soon learned, that, on the other hand, the extreme vowels u and i require 
long practice. In these exercises the patient must carefully observe the mouth 
of the physician who teaches him to speak. By this means he receives optical 
impressions of memory which aid him in producing these sounds spontane- 
ously. To heighten this effect, I urge the patients to use a mirror by means 
of which each may observe the phases of his own speech motions and compare 
them with those of his physician. It is interesting to note how the patient 
who has just attempted unsuccessfully to reproduce the sound of o which the 
physician has made for him is often enabled to do so immediately if he takes 
the mirror in his hand and imitates the position and movements he has just 
seen. After learning some of the vowels we proceed to the labial sounds, and 
here the toneless p which is most easily formed is the one we practise first. 
The lips are closed, then by a sharp expiration are parted, after which by clos- 
ing the nose we sometimes see that the column of air actually passes through 
the mouth. The consonant which has been learned is then combined with the 
vowels, at first in the meaningless syllables of pa. po, pe. Memory for the 
newly acquired sounds is at first very weak, so that even two successive sylla- 
bles can scarcely be repeated; frequently either the last or the first is for- 
gotten, according to the attention which the patient has given to the move- 
ments of the vocal organs. Sometimes his ability to remember and reproduce 
the syllable depends upon its emphasis. By patience and perseverance we soon 



330 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

teach him to speak correctly the three syllables in varying sequence. This 
practice of meaningless syllables is of the greatest advantage in strengthening 
the memory for speech processes. Then we take up b. The difficulties increase 
to such an extent as to try severely the patience of the learner. If the con- 
sonant is not soon clearly differentiated from p, it is well to postpone its prac- 
tice to some subsequent period, and take up m instead. Here the use of 
the mirror alone is insufficient; besides the optical stimulation of the picture 
by the mouth movement, the tactile control of the resonant column of air 
which comes through the nose must be utilized. For this purpose, just as 
previously in the case of the deaf-mute child, the patient places his finger 
upon the physician's nose so that he feels the resonance of the air which comes 
through the nose. Even when practising the vowels, it is well to allow him 
also to practise this sensation control, as well as to place his hand upon his 
larynx. 

As is obvious from the illustrations given, the teaching of the various 
vocal sounds presupposes on the part of the physician a thorough knowledge 
of the physiology of speech. Without this the results attained by his endeavors 
will be merely accidental and clumsy. If the physician desires to treat apha- 
siacs, the first prerequisite is that he thoroughly learn the physiology of speech. 
It is not my purpose here to describe the individual processes in the develop- 
ment of speech in motor aphasia from the point of view of the physiology 
of speech, nevertheless we must devote some attention to a few measures which 
assist this process. 

With the exercises in articulation which have been described I combine 
systematic writing exercises with the left hand. As is well known, Broca 
refers the right-handedness of most persons to the action of the left brain. 
The congenital preponderance of the left brain results in the early use of 
the right hand for skilful movements. Accordingly the left brain rather 
than the right brain is also the central seat for the finer movements of speech. 
In aphasiacs who were left-handed an anatomical defect was found in the 
right half of the brain. Therefore, without fear of challenge, we may draw 
the conclusion that by practising the left hand for finer movements, for exam- 
ple, for writing, the movements for speech are better set in motion in the 
right brain. Such considerations have been utilized by numerous authors; I 
shall only mention Clarus, Berkhan, and especially Bernhard who points out 
that even with total destruction of the left speech tract compensation in the 
faculty of speech may result if the right half of the brain influenced either 
spontaneously or by exercise substitutes for the left-sided region in which the 
function has been lost. Consequently Bernhard believes it to be the duty of 
parents, even from the infancy of their children and even in healthy children, 
to see that they learn to use their left upper extremity as well as the right, 
so as to evolve from the preponderating property of the left brain a double 
brain condition. In all the cases of aphasia brought to me for treatment, 
I have instituted the systematic exercise of the left hand for writing with the 
exception of those cases in which, from long continued loss of power or per- 
manent paralysis of the right hand, a greater facility with the left hand has 
been acquired. I was invariably able to show a distinct parallelism between 
the improvement in the power of articulation and improvement in writing 
with the left hand. A further advantage of the writing exercises is that 



PERIPHERAL IMPRESSIVE DISTURBANCES 331 

they assist the memory by a sequence of sounds. It may happen that, even 
after long practice, a word cannot be produced in front of the mirror, yet 
success is readily attained after a few preceding writing exercises. Therefore 
each process assists the other. I combine the reading exercises with those 
in articulation just as soon as possible by writing the individual letters upon 
small slates which are sought out from a large number of slates, the letters 
being therefore actually read, and are then placed in combination with one 
another. In acquiring the power of speech, writing and reading are of course 
to be regarded only as aids, for, otherwise, patients would regain their power 
of speech only by a very circuitous route. 

It is remarkable that, in almost all cases of motor aphasia, the combination 
of perception and word formation needs particularly to be practised. The 
patient repeats certain words distinctly, and understands their meaning per- 
fectly. If, however, the object is shown him he cannot name it. Therefore 
he is able to repeat the word, but he cannot innervate the motor speech con- 
ception from the optical picture. If the word " slate " is spoken he repeats it, 
and also shows that he understands it. If, however, after some time, we 
point to the slate and ask him to name it, he finds it impossible to do so. 
This peculiar psychologic condition may be explained by quoting what Storring 
has said in his excellent " Lectures upon Psychopathology " : " In normal 
psychical life the perception of an object is very rarely followed by the repro- 
duction of the corresponding name, but the hearing of a name is followed by 
the conception of the object designated/' Storring mentions as an example 
that when we hear the word " horse " we much more frequently form a con- 
ception of the object — therefore reproduce mentally the picture of a horse — 
than we reproduce the name " horse " on seeing the object. We note that in 
the development of speech the first named method is the one earliest utilized 
by all persons. For this reason it is longest practised, and in all aphasias it 
is the last to be lost. From this it also follows that " in reviewing the vari- 
ous stages of diminished ability to develop correlations of reproduced concep- 
tions in the centers for object pictures and sound pictures with a definite and 
uniform decrease of the activity of these centers, it must be that sound pictures 
bring to mind the object pictures, while the object pictures are powerless to 
recall the sound pictures" (Storring). We must, therefore, employ pictures 
systematically in motor aphasia. I bisect the leaves of a small book, pasting 
on one side one-half of the picture of an object, and writing the name of 
the object upon the other side. The patient must learn to combine the name 
with the picture just as we learn the vocabulary of a foreign language. 

All that I have thus far stated relates to motor aphasia. In sensory 
aphasia in which perception is chiefly disturbed, we must attempt to substi- 
tute for this another mode. Exercises in hearing such as were practised by 
Westphal, Oppenheim and Xemann were unsuccessful. I have therefore tried. 
as in the case of deaf-mutes, to substitute vision as the method of perception, 
and have taught the patient with sensory aphasia to read from the lips. After 
sufficient time for practice, a control test showed in how far the patient with 
sensory aphasia had practised perception by the eye. I spoke to the patient 
by merely making the motions of speech, so that he could hear no sounds but 
only see the movement. This succeeded as well as could be expected, the 
results depending on the amount of practice in reading which the patient had. 



332 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

If I turned away from the patient and spoke aloud, so that he could hear 
my words but could not see the movement of my lips, perception was almost 
as poor as at the beginning of the exercises ; if I turned toward him and spoke 
aloud so that he saw the movements as well as heard them, perception was 
absolutely perfect. It follows from this that training of the eye enables us 
to utilize to better advantage the acoustic method. 

French authors (Fere and Danjou) have pointed out that in motor aphasia 
gymnastics of the organs of speech should precede the practice of individual 
articulations. In fact, practice in the movements of the lips and tongue, per- 
haps by means of a glossodynamometer and in the form of resistance gymnas- 
tics, most effectively facilitates the production of individual sounds in the 
special speech exercises which are later undertaken. If this preliminary prac- 
tice is instituted the desired results are more speedily attained. 

The results of this method of treatment of aphasia of course vary greatly 
according to the length of time the practice is continued and the patience 
of the sufferer. In absolute motor aphasia, as a rule, five or six months of 
practice is necessary before the patient can spontaneously use the words and 
sentences which he requires in daily life. 

I must reiterate that the treatment by exercise which I have here described 
is not to be instituted until a spontaneous cessation of the cerebral process 
has been observed, or until (for example, in syphilitic cerebral processes) 
this has been thoroughly treated. In aphasia due to syphilis we frequently 
note astonishing improvement after thorough inunctions and the use of potas- 
sium iodid. Naturally this aphasia is characterized by relapses; nevertheless 
the treatment is simple. Exercises should be resorted to only when there 
is great destruction of the cortical substance or the conduction tracts. By 
exercises I taught several patients, in whom aphasia was unquestionably due 
to syphilis, and in whom no improvement followed the use of mercury and 
potassium iodid, to speak even after the aphasia had existed for years. 

Aphasia, too, which is not merely functional, especially that of a hysterical 
nature, is more amenable to treatment by this method than by hypnotism or 
suggestion.^ It is questionable whether we are justified in differentiating be- 
tween " organic " and " functional " aphasias, for we may generally assume 
that with a disturbance of function an organic disturbance also is present 
although we cannot invariably discern it. If, at the autopsy, no lesions are 
found in that part of the brain which controls these functions, this should 
never be regarded as proof of the absence of disturbance. We recognize many 
so-called functional disturbances of the center which we are unable to demon- 
strate. Apparently the functional disturbances are of milder nature than the 
organic, certainly this is true of the phenomena of aphasia. But this by no 
means disproves that they may exist unchanged for years. We recognize a 
voluntary mutism not only in certain forms of mental disease but also as an 
unpleasant symptom of psychical depression in mild, functional, disturbances 
of speech. Finally, we must enumerate in this category the hysterical aphasias 
in which treatment by exercises is decidedly valuable. In cases in which the 
action of the electric current is absolutely negative, patients are sometimes 
enabled by this purely physiologic method to regain not only the voice but 
also speech in a surprisingly short time. 

It is especially difficult to regain the voice in hysterical aphasia, and here 



CENTRAL DISTURBANCES: STAMMERING 333 

the process must naturally be the same as that which we employ in hysterical 
aphonias when we desire merely to regain the voice. It is well known that 
electricity (feeble galvanic currents applied to the larynx) sometimes gives 
remarkable results; we also know that there may be a relapse of the aphonia, 
and that subsequently the galvanic current often loses its efficacy. In these 
cases I have almost always secured a permanent effect by the physiologic 
development of the voice. If, after an oral inspiration, expiration is practised 
so that we pass from breathing to whispering, and from whispering to the 
spoken voice, we innervate in a sequence first, in breathing, the vocal muscles 
or the internal thyreoarytenoids, then in whispering the lateral crico-arytenoid 
muscles, after which merely the action of the oblique and transverse muscles 
is necessary for the production of voice. The exercise is graphically portrayed 
in the following diagram : 



e 
o 


h 








a 






a 




U 














j 


a 

— * 




Y 

Breathing 




"Y 

Whispering 




Voice 





In hysterical aphonia we must at first be satisfied if the patients pass from 
breathing to whispering, and then very cautiously attempt to use the voice. 
This is true not only of hysteria which produces ordinary flaccid aphonia but 
also of spastic aphonia. That aphonia spastica resists all external remedies is 
well known, and this affection has even been looked upon as incurable. De- 
cided irritation at once produces spasm, and is therefore prohibited. Painting 
the larynx, the application of the electric current, and many other curative 
measures are unsuitable in these cases. The treatment by exercises here de- 
scribed produces the least possible irritation, but it may be mentioned that, 
if we proceed too rapidly, even this practice may cause severe spasms. I 
once attained a permanently good result in aphonia spastica that had existed 
for years. It was in the case of a soldier who, frightened from his sleep 
by an attack made by a comrade, took to flight, and for days wandered aim- 
lessly hither and thither. He was absent from his regiment for fourteen days, 
and on returning was charged with desertion. Besides numerous other psy- 
chical symptoms of depression, complete mutism now gradually appeared. 
Every attempt at speech caused severe respiratory and vocal spasms which 
subsequently became general. Careful examination revealed that this was 
not a case of malingering, but that, probably owing to the nocturnal attack 
of his comrade, a traumatic psychosis had developed. The symptoms on the 
part of the voice were most intractable. After the disease had lasted for a 
year and a half, the Minister of War sent the soldier to me for treatment, 
and a few weeks later he was discharged, his speech being perfectly normal. 



CENTRAL DISTURBANCES: STAMMERING 

Unquestionably the most familiar and most prevalent central disturbance 
of speech is stammering. Viewed from a purely external standpoint, it rep- 
resents a spastic neurosis of coordination. The spasms inhibit, hinder or 
interrupt speech, they occur in the three chief muscular regions which are 



334 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

called into action by coordinated speech, in respiration, in voice, and in articu- 
lation. We designate it as a neurosis because we do not know its anatomical 
seat; that is, its pathology. That it is a central affection is obvious from the 
symptomatology, and whether we refer it to the accepted seat of motor speech, 
the third left frontal convolution, as Ssikorski does, to the cortex of the cere- 
brum in general, as was previously done by Schrank, or to a congenital irritable 
weakness of the entire coordination of articulation (Kussmaul), none of these 
theories may be regarded as absolutely acceptable. The last mentioned view, 
that of Kussmaul, appears to me to be the most likely, as will appear when 
we consider the etiology of the affection. 

Etiology. — I have treated in my Clinic 569 cases of stuttering in which 
the etiology was carefully studied (doubtful and incomplete cases have been 
excluded from this review). Almost all of these cases occurred in children, 
and the individual data were obtained from the mothers themselves; where 
investigation in the family was necessary, the desired data were obtained by 
my assistants. In adult stutterers we are very apt to arrive at erroneous con- 
clusions as to the etiology. An example of this is reported by Ssikorski who 
concluded " fright " to be the cause of stuttering in over 70 per cent, of his 
cases. In my cases 422 were boys, 147 were girls. The affection was found 
running in families in 162 cases, therefore in 28.6 per cent. Direct heredity 
was proven in 47 of these cases, therefore in 8.3 per cent. In this reference 
to direct heredity I wish it clearly understood that the stammering child did 
not hear its stuttering ancestor (a fact which Epstein also emphasizes) ; other- 
wise, imitation would have great significance in the etiology. We shall pres- 
ently discuss this point more minutely. In 57 cases it was ascertained that 
the father stuttered, and in 20 of these cases the child did not know that his 
parent stuttered. In 16 instances the mother stuttered, but in 11 of these 
cases only in her earliest youth; the paternal brother stammered in 13 cases, 
the maternal brother in 9, an uncle of the mother in one case, a great-uncle 
in 2, aunts in 3, the grandfather in 11, and cousins in ten cases, brothers and 
sisters 115 times. Therefore, in these 162 cases, 237 people who were related 
stuttered. Peculiar hereditary leaps in the etiology of stuttering are best 
illustrated by a few genealogical trees which I here reproduce. 1 

In the first ancestral tree we note that among the grandparents the 
grandmother stuttered from earliest youth. She had two sons, the elder of 
whom stuttered in youth but not the younger. The son that stuttered married 
(long after he had ceased to stutter) a woman who did not stammer, and they 
had three sons, of whom the eldest did not stutter, but the two younger ones 
did. In the second case the father had stuttered in youth, and his two chil- 
dren, a boy and a girl, both stammered throughout life. The girl married 
a man who did not stutter, and from this union four non-stuttering boys 
were born. In the third case, the ancestral tree starts with non-stuttering 
parents, but the brother of the husband was a stutterer. From these parents 
a stuttering daughter was born who married a non-stuttering man, and they 
had five children, four boys and a girl, all of whom stuttered. The fourth 
and fifth cases may be easily understood from the diagram. 

i The black circles indicate " stutterers." If these are within parentheses, it indi- 
cates that stammering had previously appeared in the family. 



CENTRAL DISTURBANCES: STAMMERING 



335 



From these illustrations it is evident that, besides heredity, there is an 
individual congenital predisposition to stuttering; for, in the first case, the 

r 



w 

i 



i 

o+ 



> 
% 



CO 

oo 



c 

T3 









*• 
% 




^; 



O 
I 



as 



r 



i < 






o 

Cm 





336 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

eldest son did not stutter, but the two younger did; in the second case none 
of the children stuttered, although the hereditary predisposition was great; 
in the third case the hereditary predisposition was relatively slight as only 
a brother of the grandfather stuttered, nevertheless all of the grandchildren 
stammered; in the fifth case the hereditary predisposition was marked, yet 
the four elder boys did not stutter, while, on the other hand, the two younger 
children, a girl and a boy, did. Only an individual congenital predisposition 
will explain this irregularity in the appearance of the affection, and the 
assumption of a congenital, irritable weakness (Kussmaul) affords the most 
plausible theory. We may the more readily adopt this view since, as we shall 
soon see, we find in stuttering children many symptoms which indicate a 
neuropathic congenital taint. 

In the same category apparently belong the 26.7 per cent, of cases in 
which, notwithstanding closest investigation, we were unable to find the cause 
of the affection. Stuttering developed in earliest youth, gradually increased, 
and became very embarrassing, especially in the first years at school. I have 
pointed out in another article that in the development of the power of speech 
in the child there are several factors which conduce to stammering. In 10.2 
per cent, these are infectious diseases, in 1-1 per cent, a fall or fright, and in 
11 per cent, other diseases are named as causes. There can be no doubt that 
disturbances of speech may follow infectious diseases and brain shock ; in fact, 
any psychical shock. Nevertheless the number of cases to be attributed to these 
occasional causes is relatively small, and I fear that in spite of the utmost care 
in the investigations which have been mentioned this is over-estimated. Imita- 
tion of a stuttering comrade or schoolfellow was expressly mentioned as the 
cause of stuttering in 9.5 per cent, of all cases. 

Among external abnormalities which may bear some relation to stammer- 
ing, I must particularly mention adenoid vegetations, those hyperplastic 
structures of the pharyngeal tonsils which have, in part, a purely mechanical 




Quiet Breathing. Speech Respiration. Quiet Breathing. 

Fig. 145. — Diagram showing the normal process of speech in man, taken with the Gutzmalin- 
Oehmecke girdle pneumograph. I, inspiration. E, expiration. 

and inhibitive, in part a reflex effect upon speech. We found these prolifera- 
tions — and I have included only those cases in which they were large enough 
to protrude above the upper choanal border and thereby visibly hindered res- 
piration — in 30.9 per cent, of the cases, that is about three times as frequently 



CENTRAL DISTURBANCES: STAMMERING 337 

as among normal school-children. I do not intend hj this statement to main- 
tain that adenoid vegetations are the cause of stammering, for if this were 
actually the case there would be more stammerers. But they secondarily cause 
an impediment in speech acting on a neuropathic predisposition. Such im- 
pediments occur only in those persons who are very susceptible to irritations 
which in normal persons would produce no reaction ! We frequently observe, 




Quiet Respiration. Marked Stuttering. Quiet Respiration. 

Fig. 146. — Stuttering in a girl, aged 21. Taken with the Gutzmann-Oehmecke girdle 

pneumograph. 

and this may be done by any one who has practical experience in disturbances 
of speech, that an acute coryza during the course of treatment will cause a 
severe relapse in a stammerer. That this relapse is a reflex phenomenon is 
obvious from the fact that in some cases it may at once be relieved by cocain- 
izing the nose. It is impossible for me to give a better illustration of the 
fact that in persons who stutter the wave of irritation is more deeply situated 
than in normal persons, that, accordingly, they must be regarded as nervous 
or neurasthenic with a neuropathic predisposition. By plugging the nose with 
cotton, I have in some cases succeeded in reproducing the stuttering in a few 
minutes. We may here observe what is almost a parallel between stuttering 
and asthma as reflex phenomena. 

Symptoms. — The symptomatology of the condition may be briefly described. 
It consists in spasms of respiration, of the voice, and of articulation. These 
spasms are occasionally of such a nature that they are susceptible to and rec- 
ognizable by inspection and palpation, and are at other times so slight and 
so transient that careful instrumental examination is necessary to demon- 
strate them objectively. 

As the scientific study of stuttering and other spastic disturbances of 
speech is essentially of most recent date, I shall permit myself in this article 
to discuss this investigation somewhat minutely. In normal speech, inhala- 
tion through the open mouth is brief and inaudible; expiration takes place 
in the same way, but it is the vehicle of speech, and therefore much slower. 
Speech respiration differs decidedly from normal rest respiration, which, as 
is well known, takes places through the nose, and during which the duration 
of inspiration about equals the duration of expiration ( I — § E ) . These rela- 
tions are best shown by employing Marey's pneumograph or similar apparatus. 
23 



338 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

In all such apparatus the principle of transmission depends upon the fact 
that an air capsule pressed over the epigastrium, and thus dilating the cir- 
cumference, transmits this pressure by means of a rubber tube to an ordinary 
Marey or Engelmann sphygmograph. If it is directly transmitted, inspiration 







Respiration during rest. Sighing. Rest. Stuttering. Rest. 

Fig. 147. — Stuttering in a girl, aged 21. The period of time, two seconds, is indicated over the 
curve. The respiratory curve was taken with the Gutzmann-Oehmecke girdle pneumograph. 

causes a movement of the writing lever upward, if indirectly transmitted it 
produces a downward motion. The curve here reproduced has been taken by 
a girdle pneumograph which I invented, and shows the interchange of inspira- 
tion and expiration in rest, subsequently the very distinct differentiation of 
this process of respiration in speech. It will be noted that speech expiration 




Fig. 148.— Stuttering during the enunciation of "1" and "p" in the word "lamp." The spastic 
movements of the floor of the mouth were taken with Marey's cardiograph. The limit of 
time was one-tenth of a second. 

is extraordinarily full, and, compared with respiration in rest, consumes a 
decidedly longer time. It may also be noted that otherwise it runs quite 
uniformly; the slight excursions are only indications of the greater or less 
air pressure required to enunciate the individual sounds. 



CENTRAL DISTURBANCES: STAMMERING 339 

'The curve of respiration in stammering is quite different. Here there are 
various types of faulty respiration, of which I shall mention and describe the 
three which are most frequent. The first form is that in which the stam- 
merer, when he wishes to speak, expels most of the air in a single rapid 
expiration, and then attempts to speak with what remains. The second type 
is seen when speech becomes impossible because of an inspiratory spasm of 
the diaphragm. Very distinct clonic spasms are shown in the curve. The 
third type is marked by the fact that the curve at times remains upon a 
monotonous line, an expression of the fact that a tonic contracture of the 




Jaw. 



Upper Lip. 



Floor of Mouth 



1-10 Sec. 



Fig. 149. — Diagram of stuttering, made with Zwaardemaker's apparatus. This curve was fin- 
ished by Professor Zwaardemakcr (Utrecht). The clonic spasm at "p" and again at "t" 
in pronouncing the word "pitten" is distinctly visible. 

diaphragm results, and prevents the further course of the expiration. Between 
these three types we find every possible transitional stage, and not infrequently 
all may be combined in one and the same person. 

Spasm of the organs of speech may be readily diagnosticated in the great 
majority of cases by auscultation, particularly by means of the panendo- 
scope, the rod of which may be shifted to various parts of the thyreoid cartilage 
and the incisura thyreoidea superior. It is rarely possible to view a spasm 
of the laiwnx by the laryngoscope. Yet some authors, and I myself, have 
succeeded in a few cases. Clonic or tonic spasms are then seen, in most cases 
spasm of the constrictors of the larynx, in a few instances of the muscles which 
open the larynx, and this enables us to see the position of the vocal cords which 
form a large triangle; the vocal cords tremble spasmodically in the vain 
effort to produce voice. I regard this as a distinct spasm of the posterior 
cricoarytenoid muscle, and I observed such a case with Th. S. Flatau. Ob- 
jective pictures of these vocal spasms may be obtained by a phonautograph, 
although even with the most complete instruments the reproduction leaves 
much to be desired. 

All spasms of articulation may be reproduced most readily and with great 



340 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

exactness by means of Marey's cardiograph. The palette of this instrument 
is placed lightly upon the muscular area in question, and its motions are 
transmitted as usual by means of the rubber tube and the sphygmograph. 
The tracing shows how exactly even unusually rapid spasms and spasmodic 
movements are reproduced in the curves. Zwaardemaker has applied a num- 
ber of these or similar capsules to the head by means of a forehead band so 
that the normal processes of speech may simultaneously be written in a lower 



Floor of 
Mouth. 



Lower Jaw 



Respiration. 




Fig. 150. — Diagram of stuttering in a patient, aged 24, taken with Zwaardemaker's apparatus. 
On the first attempt to pronounce the word "kakao," the patient halts, and repeats the 
"k" three times. On the second attempt, he does not stutter. The respiration was taken 
with the girdle pneumograph. While speaking the lower jaw was held in a spasm; hence 
the almost straight line. 

jaw curve, upper lip curve, lower lip curve, and the curve of the floor of the 
mouth. This instrument may also be employed in the case of the stammerer, 
and the first curve taken by Zwaardemaker shows very characteristically the 
trembling spasm of the various parts. The use of Zwaardemaker's apparatus 
for the stammerer is somewhat difficult because its application distinctly irri- 
tates him, children and even nervous adults being afraid of the apparatus. 
It is therefore much more convenient, as I have found, to employ Marey's 
cardiograph which can be easily and correctly applied to the various areas, 
and just as readily be removed. 

The psychical symptoms manifested by the stutterer may be accurately 
investigated objectively. It is characteristic of a neuropathic individual soon 
to become fatigued. This ready exhaustion, particularly after attempts to 



CENTRAL DISTURBANCES: STAMMERING 341 

speak, may be demonstrated with exactness in the stutterer by chronoscopic 
investigation such as Ziehen has employed in neurasthenics, as well as by 
Mosso's ergograph. The psychical disturbances which are revealed in the res- 
piration of rest after stuttering should be used as objective tests for the ready 
psychical irritability, only it must be borne in mind that these investigations 
easily produce psychical irritation even in the normal person, and they must 
therefore be repeated if we would avoid the possibility of error. 

From this brief review it is evident that we are not content to-day with 
the conclusion, " We are dealing with a stutterer," but we must determine to 
the minutest detail the nature and course of the spastic processes so that a 
special diagnosis may be made in the individual case ; this was formerly never 
attempted but is nevertheless of immense imjjortanee in treatment. The old 
treatment of stuttering was a mere groping around in the dark and experi- 
mentation, while at present, by a comprehensive and careful diagnosis, we 
follow definite methods which lead directly to the goal. 

The spasms of the stutterer previously mentioned need by no means occur 
in all cases and in all of the three muscle groups called into action by articu- 
lation; it may be prominent in one stutterer while in another there are only 
spasms of respiration, in another the action of the voice may be specially 
implicated, and in si ill another only the organs of articulation. This fact 
alone furnishes definite indications for treatment. The general symptoms of 
the stutterer and the nervousness which is almost invariably present require 
treatment, and experience soon shows that upon the basis of these considera- 
tions stutterers may be practically divided into three groups: 

(1) Those stutterers in whom the spasms are principally due to absent- 
mindedness and inattention; among children these form the majority, and 
they may be easily discriminated by requiring the child to repeat immediately 
the sentence which was just stuttered, when, almost invariably, the repetition 
is less stuttering or it may even be fluent. In these cases the absent-minded- 
ness and inattention to speech may almost always be referred to nervousness 
in the child, and this is often exaggerated and increased by adenoid vegeta- 
tions or other abnormalities and disturbances of the upper air passages. 

(2) The stutterers in whom a repetition of the stuttering sentence shows 
no amelioration but even an aggravation of the spasms. This increase of 
spasm upon repetition may be so great that no sound can be enunciated. In 
such patients attention to the speech only increases the inhibition. The inten- 
tion of the will becomes an irritant which intensifies tlie spasm. Here, quite 
as frequently as in the first group, abnormalities and disturbances of the upper 
air passages augment the difficulty. 

(3) The stutterers in whom stuttering has lasted so long as to produce 
psychical depression, a dread of speaking, a sense of inferiority in comparison 
with their fellows, idiosvnerasies as to definite sounds in which erroneous con- 
ceptions are such marked secondary phenomena of stuttering as to be the 
most prominent of all the symptoms. In the main. 7 regard these psychical 
phenomena as secondary symptoms, since they are usually absent in children 
and present in adults. That they are not a necessary result of stuttering is 
evident from the fact that a number of adult stutterers do not manifest them, 
and also from the fact that they may be associated with other disturbances of 
speech. 



342 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

It is evident that this classification is of great therapeutic importance ; for, 
in the first group, everything will depend upon cultivating the habit of atten- 
tion in the child and removing all factors which interfere with this. In the 
second group, careful speech movements, beginning with the most elementary, 
form the only remedy with which to overcome the spasms. In the third group 
we must be guided by the conviction that upon concentrating the attention 
on normal processes of speech the spasm will under all circumstances be 
averted and the psychical auxiliary symptoms at once be relieved. In the 
third group an increase of the secondary phenomena is rarely so severe as to 
dominate the picture. Here we are dealing with markedly neurasthenic per- 
sons who should receive general treatment before they have any specially 
directed to the difficulty in speech. I once treated a young lady who, even 
with her first exercises in breathing, conceived the erroneous idea that I re- 
garded everything she did as incorrect. This impression was so real that she 
burst into tears after the first exercise, and this crying prevented her from 
carrying on the breathing exercises. With every attempt there was a repeti- 
tion of the tears. In such cases it is obvious that general treatment, perhaps 
in an institution for cold water cure, is indicated before the difficulty in speech 
can be especially treated. 

In conclusion, it must be mentioned that in some stutterers associated 
movements follow these spastic symptoms, associated movements which are 
either reflected by the spasm to neighboring muscle areas, such as contortions 
of the face, closing the eyes, nodding of the head, and the like, or from the 
effort to overcome the difficulty they may occur in any part of the body, such 
as stamping, knocking with the fists, even jumping and the like. These con- 
strained movements also must, of course, be regarded as secondary symptoms. 

Treatment. — In passing to the treatment of stuttering I must remark from 
the onset that with the general disturbances almost invariably present, which 
primarily appear as nervous phenomena, a constitutional treatment of the 
stutterer, a careful regulation of his entire mode of life is an absolute pre- 
requisite, provided we wish the cure to be permanent and not to be constantly 
surprised by relapses. Such a general treatment and regulation of the mode 
of life is naturally only possible in clinical treatment, and even then only 
when this clinical treatment is carried out in somewhat of a domestic manner. 
I have previously called attention to the extraordinary importance of proper 
diet in the treatment of disturbances of speech. General bodily exercise, 
abundance of fresh air, bathing of the entire body both morning and evening, 
sufficient sleep, plentiful but not too monotonous nutrition, are the important 
factors. That this mode of treatment fell into desuetude after the earlier 
clinicians had clearly recognized its utility can be due only to the fact that 
the treatment of disturbances of speech has recently been taken from the hands 
of the physician and entrusted to quacks and empirics. Any one who has 
made even a few clinical observations of stuttering children will surely come 
to the conclusion that the treatment of the stuttering child should be in pro- 
fessional hands, and whenever the family physician notes the development of 
this fault he should, above all things, regulate the child's general nutrition 
and mode of life. I have observed numerous instances in which such a diffi- 
culty in speech has been favorably influenced or even removed chiefly or solely 
by such general treatment. 



PERIPHERAL EXPRESSIVE DISTURBANCES 343 

We pass from these preliminary remarks to the special treatment of stut- 
tering, which has been based on physiologic premises by no lesser authorities 
than Johannes Muller and Dubois-Reymond. Dubois-Keymond points out 
the fact that our bodily exercises are not only muscle, but also nerve, gymnas- 
tics. The normal muscle promptly obeys the nerve; the condition of con- 
traction in the body present at any moment is determined by the state of 
stimulation of the nerves in the preceding moment. Since the nerves trans- 
mit only the impulses coming from the motor ganglion cells, it is obvious that 
the actual mechanism of coordinated movement has its seat in the central 
nervous system, and consequently practice in such movements is in the main 
merely an exercise of the central nervous system. This possesses the ines- 
timable advantage that a series of movements which frequently conform to 
a certain law are readily repeated in the same order, increasing and decreas- 
ing and merging into each other as soon as an impulse of the will calls them 
forth (Dubois-Reymond's Lecture, " Ueber die Uebung"). The application 
of this law to our case is the following : If the normal respiratory movements, 
the normal movements of the voice, and the normal movements of articulation, 
carefully proceeding from the more simple to the more difficult, are systemat- 
ically practised, and practised so long that they are made without attention 
to rules, therefore involuntarily, normal speech is the result, and stuttering is 
cured. Hence there can be but one rational treatment of stuttering, and this 
consists in practising the necessary movements of speech, by a physiologic 
method. By this mode we will observe that constrained movements are sup- 
pressed. 

Opinions vary as to the adaptation and development of this treatment in 
the individual case. Consequently there are numerous methods, many of 
which may ultimately lead to satisfactor}' results, but all must be based upon 
this rational therapeutic principle. The best model for the corrective exer- 
cise in stuttering is the person who speaks perfectly. Respiration must be 
practised until the previously indicated respiration curves of normal speech 
are acquired; the voice must be trained by such elementary exercises as I 
have advised for aphonia spastica until spasms no longer appear, and articu- 
lation exercises for the most elementary movements, combined with the use 
of the eye and sensation, must be practised before a glass as long as any 
spasms occur. 

It would lead us too far afield to explain in this article how the principles 
of the treatment are to be carried out. But I may refer the reader to the 
book published by Albert Gutzmann, to my lectures " Speech Disturbances " 
(1892), and to the monograph upon stuttering which I published in 1898. 

PERIPHERAL EXPRESSIVE DISTURBANCES 

We shall now consider briefly peripheral expressive disorders of speech. 
Here also we differentiate between functional and organic disorders, and then 
review the individual disturbances. I prefer, however, to discuss a few of these 
peripheral expressive disorders of speech, and at the same time to illustrate 
the entire group of these affections. It is clear that disturbances of respira- 
tion and of the voice which we find in diseases of these organs may also influ- 
ence the speech, but in such cases the disease which affects the organs so 






344 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

engrosses the professional interest that the disorder of the speech is regarded 
as merely secondary. I must, however, briefly mention an exception. This 
is the disturbance of speech which develops during puberty, and which may 
permanently impair the voice, and also the speech. It sometimes happens 
that the falsetto voice persists beyond the period of puberty, in which case 
we find grown persons with a ludicrously high voice who are mortified by 
their speech, and spare no pains to attain the normal male voice. Here elec- 
tricity and local remedies are of little avail, but a satisfactory result may almost 
always be readily attained by the method, previously described, of transitions 
from breathing to whispering, and finally to the speaking voice. If, at the 
same time, mild lateral pressure is exerted upon the larynx, the patient, as 
a rule, is soon able to enunciate a deep wavy tone, and with subsequent prac- 
tice a strong chest tone is developed. By holding the tone as long as possible, 
individual vowels and combinations of syllables are practised, and we soon pass 
to reading, after which it is only necessary for the patient to use this newly 
gained power of speech in his daily intercourse. Patients are best treated in 
an institution because here they most readily lose their diffidence with their 
newly acquired proficiency in speech. 

Among peripheral expressive disorders of the organs of articulation, I must 
mention congenital and acquired paralysis, the cleft palate, inhibitions which 
result from a faulty formation of the tongue, and an abnormal position of the 
teeth and their influence upon speech. Defects of the tongue are rarely the 
cause of disturbances of speech, and it is remarkable that this fact so long 
failed to be recognized; it was apparently due to the synonymous use of the 
expressions, tongue and speech. From most remote antiquity the tongue has 
been regarded as the seat of speech. This view may be attributed to Aris- 
totle, who not only expressed it in simple form, but referred all errors in 
speech to the tongue, either because it was too broad, too short, or not suffi- 
ciently motile. In at least many cases of disturbance of speech careful clin- 
ical observation would incline us to another view, and we note that even 
before Aristotle old Father Hippocrates held a much more correct theory 
of disturbances of speech. He says : " Indistinctness of speech is caused either 
by disease or by defective hearing, or because, before a thought is expressed, 
other thoughts arise, before words are spoken other words are formed, etc." 
How little speech is influenced by the loss of a large part of the tongue under 
operation is well known to the modern surgeon. In former times this would 
have been regarded as a miracle. Wonderful was considered the speech of 
the so-called African Disciple of Tipasa, who had his tongue cut out by the 
vandal Huneric in the year 484, yet, notwithstanding this deficiency, was able to 
proclaim aloud his belief in Christ. Twistleton in his little book, " The 
Tongue NTot Essential to Speech," London, 1873, has shown that this phe- 
nomenon need by no means be regarded as a miracle. Nevertheless it is cer- 
tain that an imperfect tongue cannot enunciate all sounds distinctly, and that 
a faulty expression, that is stammering, must be the result of such a radical 
operation. Even partial excisions of the tongue may result in faulty speech. 
To illustrate, I saw a patient who was successfully operated upon for cancer 
of the tongue, but after the operation a faulty articulation of the s sounds 
appeared, and was such an impediment that the patient thought of giving 
up his occupation. His family physician (Dr. Peltesohn) advised him to 



PERIPHERAL EXPRESSIVE DISTURBANCES 345 

practice exercises, and after much labor he succeeded in producing the 5 sounds 
perfectly. The faulty speech was due to the fact that the lateral excision of 
the tongue had drawn the tip of the tongue completely over toward the affected 
side, and had entirely changed the sensation for the position of the parts in 
expression. The patient articulated all the sounds of speech which are formed 
with the tip of the tongue or the areas in its vicinity with the side upon which 
excision had been performed. This produced, particularly on articulating s, 
a very disagreeable sound accompanied b}" the spurting of saliva, which in fact 
rendered conversation with him most unpleasant. The exercises undertaken 
were of such a nature that first of all by means of probes a new localization 
was produced for the points of articulation which, instead of originating at 
the tip of the tongue, were now formed upon the healthy side, at the point 
where the tip was formerly situated. 

Among numerous defects in expression are the different forms of lisping 
(sigmatism) which are very prevalent. The difficulty is relieved by practice 
in sounds which correct the position of the tongue. 1 In one form of this 
sigmatism, the so-called lateral lisping, we almost invariably find an abnormal 
position of the teeth which probably causes the development of the disorder. If 
the alveolar border of the jaw is too small compared with the number of 
teeth, the teeth in their growth must overlap in such a manner that vertical 
or horizontal arches develop. Hence if the two jaws articulate upon one an- 
other at the point where the arch is situated an opening forms which appar- 
ently predisposes the person to direct a column of air to this point. As these 
dental arches are almost invariably found in lateral lisping — I found them in 
90.3 per cent, of all cases — it is a mooted question whether these are not the 
actual causes of the difficulty. I shall briefly report a case which bears upon 
this question. 

The four young daughters of a family were sent to me for examination on account 
of the same defect in speech, lateral lisping-; I was told that the eldest child had 
acquired this from a wet-nurse, and although this wet-nurse was at once sent away 
she subsequently became the nurse of the younger daughters of the family who had 
contracted the faulty mode of speech from their elder sister. The parents as well as 
the grandparents spoke normally. In all four children I found lateral dental arches, 
and also found the same in the parents and grandparents, who apparently had no incen- 
tive to use the dental arches in this way. 

This demonstrates that dental arches are not a direct cause of this form 
of faulty speech but only a predisposing cause. The same conditions which 
may be produced by a dental arch may be also developed by a tooth projecting 
posteriorly from a row of teeth, since it presses upon the tongue at this area, 
and causes a deviation of the column of air which normally passes along the 
middle of the lower row of teeth. We may see from casts of the jaw how 
variable are these dental arches. Where two dental arches exist, bilateral 
lisping may occur or the lisping may be only on the side where the larger of 
the two dental arches is found. 

It might be supposed that in order to relieve the difficulty these dental 
arches should be remedied, but that the fault cannot be overcome in this 
way is evident from the causal relation which has been mentioned. A person 

i Details will be found in my lectures upon "Disturbances of Speech," Berlin, 1893. 
24 



346 NATURE AND TREATMENT OF DISTURBANCES OF SPEECH 

may become a lateral lisper without such a dental arch, and the dental arch 
itself is by no means the only cause of lateral lisping. I therefore believe 
the remedy proposed by Berkhan to be unnecessary; he advised that a tooth 
be drawn so as to give more space, and in this ray to widen the arch. If 
we train the tongue to a correct position by treatment with sounds, and in 
this way teach the patient to close the arch and direct the column of air to the 
middle of the lower row of teeth, we relieve lisping without a special change 
in the dental arch becoming necessary. [Good dental practice, everywhere 
available in the United States, readily corrects deflections of the dental arcades, 
and should always be enlisted in the management of these cases. — Ed.] 

I shall close my description with this brief review of peripheral expressive 
disturbances of speech. Of course it is impossible within the limits of a short 
article to consider the entire range of disturbances of speech. From this 
review, however, it will be observed that these disturbances of speech have 
lately been restored to the domain of the physician, and the cultivation of this 
field constitutes for him a worthy and beneficent task which he may facilitate 
with all modern methods, and thus bring about a permanent and gratifying 
success which is impossible and will remain impracticable for the empiric and 
the quack. 



NEOPLASMS OF THE SPINAL CORD AND OF ITS 

MEMBRANES 

By FR. SCHULTZE, Bonn 

While tumors of the brain are much more common than those which affect 
the membranes of the brain, tumors of the spinal cord are less frequently 
observed than those of its membranes. On account of their practical impor- 
tance we shall chiefly consider the latter, neoplasms of the spinal cord having 
merely an anatomical, although important, diagnostic interest. 

VARIETIES OF NEOPLASMS 

On briefly reviewing the intramedullary tumors, tubercle which may occur 
either singly or be multiple is comparatively frequent, with or without a 
simultaneous affection of the membranes, and preferably attacks the enlarge- 
ments of the spinal cord. Gummatous tumors are more rare, and are usually 
combined with like changes in the meninges. 

Gliomata and " glio sarcomata" which are either circumscribed or may 
extend throughout the entire spinal cord, even to the medulla oblongata, are 
frequently observed. Sarcomata also are numerous. Earely do they attack 
the substance of the cord alone; their seat and extension vary greatly, and 
in size they range from that which is microscopic to an extent which involves 
the entire spinal cord. Often they are metastatic, solitary, or multiple, or they 
may proliferate into the medulla spinalis from without. Furthermore, there 
is a multiple sarcomatosis in which nodules simultaneously develop in the 
membranes of the central organs. 

In isolated cases, angiomata, cliolesteatomata, and cysts have also been 
noted in the spinal cord. Primary carcinomata of the spinal cord are un- 
known, and secondary ones with no direct proliferation from without are 
exceeding^ rare. 

External to the cord, therefore extramedullar!/, we must first mention 
those tumors which originate from the nerve roots; above all, the multiple 
fibromata or neurofibromata which are frequently merely a partial phenome- 
non of general neurofibromatosis of the nervous system, and are most often 
found in the nerves of the cauda equina. Although usually small, about the 
size of a hemp-seed, they may become so large as to produce pressure symp- 
toms. Sarcomata may also grow externally from the plexus into the vertebral 
canal. Gummatous tumors are also observed. 

347 



348 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 

Much more frequently the meninges of the spinal cord are attacked with 
primary neoplasms which develop within them. 

We chiefly observe circumscribed tumors which originate either from the 
dura, or from the pia, or, now and then, even from the arachnoid. These are 
usually either fibromata, fibrosarcomata or myxomata, while lipomata, adeno- 
sarcomata, lymphangiomata, and teratomata are very rare. More common than 
the last named are psammomata, which are difficult to differentiate from endo- 
theliomata, but which may also be multiple. Sarcomata of the meninges either 
press upon the spinal cord, or proliferate into it, or they develop simultaneously 
in the cord and in the membranes; they are rarely multiple. They may be 
primary or secondary; the latter in rare cases also in the form of multiple 
melanosarcomatosis. It is a significant fact that a quite diffuse sarcomatosis 
of the meninges may develop, which permeates their entire length, and decid- 
edly thickens them. Tumor-like, circumscribed, tubercular foci and gum- 
matous swellings may also be produced. Now and then echinococci are found, 
and, in about one-fifth as many cases (according to Schlesinger), cysticerci. 

These tumors vary greatly in size. Isolated tumors are chiefly found 
opposite the dorsal cord. According to pathologico-anatomical statistics, they 
are more frequently intradural than extradural. 

I shall not here enter into details, since we are mainly interested in the 
clinical phenomena of these tumors and their treatment, the paramount ques- 
tion for us being, How can we diagnosticate circumscribed extramedullary 
tumors ? 

SYMPTOMATOLOGY 

The following is the history of a young man, aged 24, who reported that as a child 
he had frequently suffered from tonsillitis as well as the various acute exanthemata, 
but from the time of puberty he had been perfectly well. Syphilis, gonorrhea, and the 
abuse of alcohol were absolutely denied. There had been no trauma. About six months 
prior to his coming to the hospital he first felt a slight pain low down in the back and 
to the left of the vertebral column. At first this pain occurred upon bending his body, 
and on rising from bed in the morning. A month later he felt it while lying down, 
and subsequently even when sitting. Coughing and sneezing greatly increased it. 
About three or four months after it first appeared, he experienced an abnormal sensa- 
tion in the left leg which he designated as " dull," later it was accompanied by formi- 
cation. Soon uncertainty and a feeling of exhaustion appeared while walking, and the 
pains in the back decreased, but were still very severe upon sneezing and coughing. A 
few weeks after the appearance of these abnormal sensations in the left leg there was 
also paresthesia in the right; the urine could not be voided with the same force as 
formerly. 

This history shows as the most characteristic factor a progressive disturbance, 
chiefly manifested by an increasing weakness of the legs after pain in the back of vary- 
ing intensity, and of regressive nature. The examination of the powerful young man 
showed, briefly, a spastic paresis of the legs which was more marked on the left than 
on the right side, a somewhat uncertain and ataxic gait, and pathologically increased 
tendon reflexes. The abdominal reflex could be evoked only in the upper portion of 
the belly. Tactile sensibility, as well as sensitiveness to pain, was diminished in the 
legs, also up to a certain height upon the trunk, while the temperature sense was but 
little disturbed. There were no other symptoms. The functions of the brain and of 
the cranial nerves were intact. Above all, there was neither nystagmus, anomaly of 
the pupil, nor any ophthalmoscopic change. No symptoms were referable to the arms. 

Since hysteria and other functional neuroses as well as polyneuritis may be ex- 
cluded, a spinal affection will naturally be thought of, above all myelitis or meningo- 
myelitis dorsalis. The onset of pain, the early sensory disturbances, the uniformly 



SYMPTOMATOLOGY 349 

progressive course of the disease, and the absence of ocular symptoms as well as 
increased reflexes in the arms, preclude multiple sclerosis ; and since the ordinary causes 
or auxiliary factors of myelitis and meningomyelitis are absent, such as syphilis, infec- 
tious diseases, exposure to cold, and over-exertion, we must, as in similar cases, con- 
sider compression of the spinal cord, for progressive myelitis without multiple sclerosis 
or syphilis is exceedingly rare. 

This compression could originate only from the vertebral column, and here, above 
all, tuberculous disease might be assumed. Upon accurate investigation, however, we 
find a somewhat marked lateral deviation in the dorsal portion, but neither Potts' 
kyphosis nor a lateral deviation of individual vertebrae. But little importance is to be 
attached to the moderate prominence of some of the vertebral processes, provided that 
simultaneously no motor disturbance of the vertebral column or demonstrable sensitive- 
ness to pressure appears in these abnormally situated vertebras. After investigating 
many normal vertebral columns, we shall be convinced that projection of the vertebra? 
posteriorly has no special significance, but that the same or similar irregularity is by 
no means rare in normal persons. 

After numerous examinations of our patient, it was noticed that pressure upon a 
definite point in the vertebral column, somewhat to the side of the median line, invari- 
ably produced intense pain, although the vertebral column could be easily moved in 
all directions, but not without some pain. The sensibility of the different vertebra? to 
pressure may be tested in various ways. I have found it advisable to make pressure 
with the posterior surface of the index finger, or with the second finger much flexed at 
the first phalangeal joint. This leads to more accurate results than if we make pressure 
with the tip of the finger or with the fist. 

When we recall the various pathologic processes in the vertebrae which may lead 
to compression of the nerve roots or of the spinal cord, everything is opposed to the 
view of tuberculosis: the absence of hereditary predisposition in this robust young man, 
the negative findings, the free movability of the vertebral column, the absence of fever 
and emaciation. Since a. carcinoma of the vertebrae could not be assumed, we could 
consider only those rare tumors of the vertebral column which are occasionally observed 
as of primary origin; for secondary neoplasms there was no proof of a primary and 
pathologic focus. These rare tumors are sarcoma and myeloma (the latter, however, 
being usually multiple), even osteoma, chondroma, and angioma. Primary sarcoma, 
however, usually produces alterations in the form of the vertebral column before it 
causes symptoms of compression. Myeloma is multiple in other bones, and, aside from 
other pathologic symptoms, not rarely leads to albumosuria, which was absent in our 
patient. Exostoses and osteoma develop more slowly, and during their growth toward 
the spinal cord do not produce symptoms so rapidly as was the case in this instance. 

Enchondromata, which also grow very slowly, are so rare that we should rather 
consider instead the presence of one of those extra- or intradural tumors of which I 
spoke in the introduction, particularly as the X-ray examination revealed nothing 
anomalous in the bones of the vertebrae. Intramedullary tumors, however, cannot be 
primarily diagnosticated because they do not set in with a neuralgic, prodromal stage, 
and because as a rule they early lead to partial sensory paralysis. Sometimes they may 
accompany chronic meningitis, and pain will be produced; in compression of the spinal 
cord from without, the various forms of sensation need not be uniformly implicated. 

This much is certain, — that other authors as well as I have usually found an extra- 
medullary tumor in cases similar to the one described. We should ascertain its exact 
position and size, since its relief is only possible by operation, and it is our duty to 
inform the surgeon as accurately as possible where it is located. 

Besides pain on pressure, the localization of which must be accurately 
determined by frequent tests, we must also ascertain the upper boundary of 
the tumor by correctly outlining the upper limit of hypesthesia and hyper- 
esthesia, no matter how slight. 

We assume that the patient to he examined is capable of accurate responses, 
which is generally the case; we must not regard the limit to be that point 
above which there is no perceptible anesthesia, but that in which there is a 



350 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 

decreased sense of contact, in distinct contrast to the other normal cutaneous 
areas. Sometimes we find in this line another area in which there is hyper- 
sensitiveness to cutaneous irritation ; upon minute investigation this was found 
in our case. 

We should also endeavor to ascertain whether, under observation for weeks 
or months, this limit remains at about the same height, whether it rapidly 
rises, or whether it shows any variations whatever. It is manifest that, in 
tumors of slight extent which increase chiefly in thickness, the intensity of 
the hypesthesia will be chiefly increased, but its extension upward in only a 
slight degree. When tumors rapidly grow upward, the anesthesia they cause 
must also rapidly ascend; but these are much more rare. In our case the 
determination of this upper limit of hypesthesia indicated that it chiefly existed 
in those striated portions of the skin covering the trunk which belong to a 
segment of the spinal cord, and opposite to which the vertebral column was 
especially sensitive to external pressure. For instance, there was hypesthesia 
in the cutaneous area of the 10th dorsal segment, and hyperalgesia in that 
of the 9th. The 10th spinal cord segment is opposite the 9th thoracic vertebra, 
and this is opposite the 8th spinous process, and just between the 8th and 9th 
sensitiveness to pressure was most distinct. The upper border of the tumor 
probably extended somewhat higher than this, for there was also hyperalgesia 
in the area of the 9th segment. 

It is more difficult to determine the lower boundary of the tumor. If 
the lumbar enlargement be exposed to pressure, the tendon and cutaneous 
reflexes of the legs are at least in part absent, and somewhat marked muscu- 
lar atrophy, possibly with DeR, may be present. How many dorsal segments 
are implicated cannot be determined, because the abdominal reflexes aid but 
little in the investigation. The upper, the middle, and the lower may be 
absent even when the tumor lies above the dorsal segment ; since in our patient 
some of the abdominal reflexes were present, particularly the upper ones, it 
seemed likely that the lower dorsal segments were implicated. 

As the abdominal muscles reacted normally to electricity, were not atrophic, 
and were still innervated, a tumor projecting downward was excluded. Simple 
paralysis might, of course, exist from pressure upon the spinal cord high up. 

Experience has shown that, in cases quite similar to ours, a circumscribed 
tumor existed which could be removed by operation. Unfortunately, we are 
at present unable to determine whether the tumor is extradural or intra- 
dural, or, above all, whether or not it can be removed; this depends upon the 
possibility of adhesions, upon the relation of the tumor to the spinal cord> 
and particularly upon the fact whether the tumor has extended anteriorly or 
posteriorly. We are forced to suggest operation to the patient because we 
know of no other curative measure, and the sequels of complete compression 
of the spinal cord would cause death. Prior to operation it is always advisable 
to try antisyphilitic treatment, as we cannot absolutely exclude syphilis occulta, 
particularly in the early stages of spinal disease. 

DIFFERENTIAL DIAGNOSIS 

The diagnosis of circumscribed extramedullary tumors of the spinal cord 
therefore essentially depends upon the existence of a gradually progressive 



DIFFERENTIAL DIAGNOSIS 351 

motor and sensory spinal paralysis, which, in spite of increasing intensity, 
extends upward very slightly, at most about one segment. This paralysis is 
usually preceded by a neuralgic prodromal stage of varying duration, in which 
the seat of pain generally corresponds to the uppermost limit of the paralytic 
phenomena. Xot infrequently local sensitiveness to pressure in the vertebral 
column opposite the affected spinal cord segment is revealed by minute inves- 
tigation. Occasionally, as is proved by my own experience, there is neither 
local pain on pressure nor radiating neuralgia ; nevertheless, on account of the 
progressive paralysis, we must make a diagnosis of tumor. 

In a differentio-diagnostic respect, as I mentioned in the history of my 
patient, we must first consider meningomyeUtis — more rarely myelitis. With 
the former we must include chronic pachymeningitis, which, owing to the 
pressure of its exudate upon the spinal cord, as well as to the propagation of 
the inflammation, may produce the same symptoms. 

Many years ago (1869) Charcot described pachymeningitis hypertrophica 
cervicalis, in which, as a rule, the same symptoms are observed as in circum- 
scribed tumor of the membranes of the spinal cord. 'Thickening of the dura 
is a form of tumor which produces the same effects as that described at the 
beginning of this article. But pachymeningitis usually runs a much longer 
course than most tumors of the membranes of the spinal cord; it may last 
for two decades, and in these cases it is usually an accompaniment of syphilis. 
In hypertrophic pachymeningitis the cervical portion of the cord is com- 
monly attacked, while most tumors are found in the dorsal cord. I have seen 
them in the meninges of the dorsal cord ; and they have frequently been noted 
opposite the cauda equina and the lumbar portion of the spinal cord. 

Ordinary meningomyeUtis can readily be excluded, since, as a rule, it shows 
a progressive course only when it develops upon a syphilitic basis. Usually 
there are simultaneously other symptoms on the part of the cerebral nerves, 
of the meninges, and of the brain. Even when these are absent, the spinal 
symptoms are not so uniformly and gradually aggravated; above all, they 
are not so constantly limited in their upward course; finally they not infre- 
quently respond to syphilitic treatment. 

In pure myelitis there is usually no pain; this symptom, it is true, may 
now and then be lacking in tumor. After a more or less rapid onset, the 
intensity of the pain becomes stationary ; in many cases its causes are obvious. 

Multiple sclerosis, at least during a certain stage of the disease, may occa- 
sionally resemble transverse myelitis ; but during its course the uniform sensory 
disturbances developing from compression, and distributed upon the skin of 
the trunk from below up to a certain height, are absent ; in sclerosis the local- 
ization and periodicity of hypesthesia vary greatly. The ordinary picture of 
multiple sclerosis differs so greatly that no differentio-diagnostic confusion is 
possible. 

The differentiation from intramedullary tumors, with or without syringo- 
myelia, not to speak of the rare cases which are combined with intramedullary 
or extramedullary tumors, is more difficult and sometimes impossible. 

In intramedullary tumors there is usually no preceding stage of radiating 
pain, particularly at the onset, while partial sensory paralyses are often 
observed, especially when a glioma, or gliomatosis, simultaneously causes cen- 
tral cavity formation. 



352 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 

But dissociated sensory paralysis may now and then be the result of extra- 
spinal tumors — Brown- Sequard's semi-lesion may be noted with extramedul- 
lary and intramedullary tumors; in spinal tumors there may be radiating 
pain if chronic meningitis has simultaneously formed; or, for unknown rea- 
sons, even without this, probably because of intraspinal irritation of the sen- 
sory pain tracts before their destruction. Why there is not always such an 
irritation, and why inversely, and in exceptional cases, there is no pain in 
spite of the pressure of an extraspinal tumor upon the extraspinal portion 
of the sensory nerve roots, we do not know. 

Although the differential diagnosis is thus rendered perplexing, it is some- 
what facilitated by the fact that extramedullary circumscribed tumors are 
vastly more numerous than intramedullary ones. The most common form 
of the latter, tubercle, occurs in patients who reveal tuberculosis of other 
organs. 

Syringomyelia may be differentiated from circumscribed tumors in that 
the latter, according to their position, produce symptoms referable to a cen- 
tral and elongated focus, that they more rapidly spread upward, perhaps to 
the medulla, and that much more circumscribed muscular atrophies are ob- 
served, even when large muscles are involved. After a long time, ordinary 
syringomyelia without gliomata will usually produce terminal symptoms of 
spinal paralysis just as severe as those of extramedullary tumor. 

I have already referred to the differentiation from diseases of the vertebra 
and from tumors. It may be added that in most of these affections there is 
generally greater rigidity of the vertebral column than in tumors of the spinal 
meninges. It was astonishing to note that one of our patients, the upper 
portion of whose medulla spinalis was compressed by a tumor the size of a 
walnut, complained of stiffness in the nape only at the beginning of the dis- 
ease, while active and passive movability of the cervical vertebral column 
continued to be normal for a year afterward, and until violent spastic mus- 
cular spasms at last appeared. In tumors of the sacral and lowest lumbar 
regions we should always palpate the bones per rectum, especially since in this 
region X-rays do not aid us. In my case X-ray examination revealed nothing 
abnormal. 

THE SEAT, EXTENSION AND COMPOSITION 

Passing to our case, and to the diagnosis of the seat, extent, and composi- 
tion of tumors of the spinal meninges, the localization of certain irritative 
phenomena may give light. 

THE SEAT 

In a certain number of cases sensitiveness to pressure may be detected at 
the height of those vertebrae opposite to which the tumor is situated; as a 
rule, laterally from the vertebral processes, and most often opposite that side 
which is the seat of the neuralgia and of the most marked paralytic phenom- 
ena. Unfortunately this s} r mptom does not appear in the majority of cases. 
Upon what this variation depends, cannot be stated. 

Sometimes the patient reports that on coughing, sneezing, or laughing — 
therefore upon movements during which the pressure of the cerebrospinal fluid 
rapidly rises — he experiences pain, particularly in those areas which are sensi- 



THE SEAT, EXTENSION AND COMPOSITION 353 

tive to pressure. This symptom has the same significance as local sensitive- 
ness to pressure, which, if it appear on active movements of the vertebral col- 
umn, is accompanied by pain in the same area. 

If extensive sensitiveness to pressure is demonstrated, we cannot at once 
assume the existence of a tumor. In the first place there may be chronic men- 
ingitis above or below the tumor; secondly, increased pressure of the cerebro- 
spinal fluid may perhaps render the sensory nerves more sensitive; finally, in 
addition to hyperesthesia of the skin there may be a purely functional hys- 
terical disturbance. The second irritative phenomenon is circumscribed neu- 
ralgia. If combined with local sensitiveness of the vertebral column, neural- 
gic pain is of particular import when it is seated within the nerves belonging 
to those segments of the spinal cord which, according to our present knowl- 
edge, lie opposite the sensitive spinous processes and the vertebral bodies. 

Very significant, too, are the hyper esthetic zones which may extend above 
the hypesthetic ones. Since these are often absent we must depend upon the 
phenomena of absence of function, sensory as well as motor. We must ascer- 
tain accurately by various methods of examination how far upward these phe- 
nomena extend; this will determine the upper border of the tumor. 

The localization of the motor symptoms of absence of function aids us 
in locating the tumor in enlargements of the cord and of tin 1 cauda equina 
(of course when combined with sensory disturbances), while the sensory 
symptoms form a valuable indication if the tumor is situated in the dorsal 
cord. Among the motor symptoms we may note the possible disappearance 
or decrease of the various reflexes. Sometimes there are trophic disturbances. 
It must be admitted that at the present time the exact localization of tumor 
at the height of the lumbar enlargement and in the cauda equina occasions 
much perplexity. 

By the combined researches of English, German, and American investiga- 
tors, it has been quite accurately determined which individual segments of 
the spinal cord are connected with individual muscular and cutaneous regions, 
and the results of these investigations have been compiled in the form of tables, 
of which I shall mention particularly those of Sherrington, Head, Starr, 
Edinger, Kocher, Wichmann, and Seiffer. 

THE EXTENSION 

In these it was demonstrated that muscle nerves do not all come from one 
segment, nor are they all supplied by one root of the cord, but by several; 
in the same way the individual cutaneous areas are supplied by two, three, or 
even four segments situated one above the other, and by their nerve roots, 
so that the destruction of a single sensory nerve root causes but little dis- 
turbance. In the diagnosis we may therefore assume that the upper end of a 
tumor will be found opposite those segments of the spinal cord the cutaneous 
areas or muscles of which show disease; and, in regard to sensation, those 
segments in the area of which hyperesthesia can be detected. 

To explain this segment innervation and its apparently remarkable rela- 
tion to the muscles and skin, Edinger 1 compiled a diagram which is very 

i "Neue Darstelltmg der Segmentinnervation des menschlichen Korpers." (Zeitschr. 
f. klin. Medicdn, Bd. LIII.) 



354 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 



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THE SEAT, EXTENSION AND COMPOSITION 



355 



comprehensive, in which, however, as in all other diagrams, some defects are 
perceptible ; but it must be remembered that there are variations in individual 
cases. 




Fig. 151. — Segment Innervation of the 
Skin. (After Edinger.) 



Fig. 152. — Segment Innervation of the 
Skin. (After Edinger.) 



356 



NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 



In this table the proximal muscles nearest the vertebral column are upper- 
most; the distal ones are lower down, according to their distance from the 
vertebral column. The segment number is marked above, and without follow- 




Fig. 153. — Diagram. (After Seiffer.) 



THE SEAT, EXTENSION AND COMPOSITION 



357 



ing downward the lines between the individual segments we can plainly see 
which spinal cord segments supply the individual muscles. 

The relation of cutaneous innervation to isolated spinal cord segments is 




Fig. 154.— Diagram. (After Seiffer.) 



358 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 



also clearly shown; in former diagrams the individual cutaneous areas were 
not sharply defined, this being impracticable because of the innervation of 
individual cutaneous areas by several different segments, and only the direc- 
tion of the innervation was 
indicated by lines. But 
at either side of these 
lines lies the cutaneous 
area which is supplied by 
a definite spinal cord seg- 
ment. At the same time 
it becomes evident, as Ed- 
inger explains, that the 
otherwise obscure distribu- 
tion of cutaneous innerva- 
tion in the extremities 
does not actually repre- 
sent the case, because the 
diagram is based on em- 
bryology (Figs. 150 and 
151). However, we may 
also utilize the diagrams 
of other authors. For the 
sake of the findings I 
have included those of 
Seiffer, as well as those 
of Head; the diagram of 
the latter author has en- 
abled me exactly to localize 
many spinal cord tumors — 
at least their upper boun- 
daries (Figs. 152, 153, 154, 
155, and 156). 

By the aid of these 
diagrams we can usually 
ascertain exactly the upper 
boundaries of a tumor. 
It is only necessary to 
bear in mind the relation 
of the individual spinal 
cord segments to the ver- 
tebral bodies and the 
spinous processes belong- 
ing thereto. For this pur- 
pose Gowers' table is valu- 
able; for it shows that the 
cauda equina begins op- 
posite the first lumbar 
vertebral bodies and their 
Fig. 155.— Diagram. (After Head.) spinous processes, and 




THE SEAT, EXTENSION AND COMPOSITION 



359 



that the greater portion of the lumbar vertebral column does not lie opposite 
the lumbar portion of the spinal cord (Fig. 158). 

After determining by the aid of these diagrams the upper boundary of a 
tumor, we must ascertain 
its longitudinal extent and 
its lower boundary. This 
is often impossible, because 
the local phenomena due to 
absence of function which 
appear at the upper limit 
generally overshadow those 
produced by pressure of 
the tumor upon the total 
transverse section of the 
spinal cord. It is easier 
to ascertain the longitu- 
dinal extension of the tu- 
mor within the cervical 
enlargement, for when the 
muscles of the lower cer- 
vical segments remain un- 
involved there is no strong 
pressure upon these. When 
the tumor is located op- 
posite the lower portion of 
the dorsal cord, and when 
the muscles belonging to 
it continue to show no 
degenerative atrophy nor 
DeR, we may conclude 
that it does not press upon 
the upper portion of the 
lumbar enlargement. How 
many segments are com- 
pressed within the dorsal 
cord cannot be stated 
from our present knowl- 
edge. Examination of 
the abdominal reflexes 
permits no positive con- 
clusions because these re- 
flexes may be abolished 
even when a circumscribed 
tumor is in a high loca- 
tion. Their appearance, 
even if only partial, 
would contraindicate the 
extension of a tumor 

downward. Fig. 156.— Diagram. (After Head.) 




360 



NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 



The absence of the tendon reflexes must be cautiously utilized, for this is 
observed even when there is no direct pressure upon the segments belonging 
thereto. When there is an acute transverse interruption of the spinal cord 

above the lumbar enlargement, the 
patella and Achilles tendon reflexes 
D.l 



Ictisk 



D.2- 



ai 



V; 



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D.l 



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, - 



S.3" 



L.l 



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10 



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Fig. 157. — Diagram. (After Head.) 



Fig. 158. — Diagram. (After Gowers.) 






THE SEAT, EXTENSION AND COMPOSITION 361 

usually disappear. 1 ( )u the other hand, their presence or increase shows that 
there is no pressure in the corresponding segments. 

Little is revealed by pain and paresthesia remote from the upper border 
of the tumor in deeper situated segments of the spinal cord and the cutaneous 
areas belonging thereto (for example, in the legs from tumors of the cervical 
cord) ; for these abnormal sensations may likewise appear when there is pres- 
sure much higher up, on the deeper situated centripetal intraspinal tracts, 
but not directly upon the segments. For instance, there may be a bilateral 
" girdle sensation " around the lower portion of the abdomen when a tumor 
presses upon the cervical segments. 

Fortunately circumscribed tumors are much more common and grow more 
slowly than extensive ones, so that operative measures are not contraindicated 
when we find a very large tumor. Circumscribed multiple tumors which 
grow slowly are more rare than solitary ones, and they do not produce pres- 
sure phenomena. If they lie close to each other, they will of course produce 
the same symptoms as elongated connected tumors. When far apart (for 
instance, one in the cervical, and the other in the lumbar, enlargement), the 
exact location may be readily diagnosticated if multiple tumors also can be 
detected, or if there are metastases from malignant tumors which cannot be 
cured by operative removal. 

When these can be excluded the local diagnosis of tumors of the dorsal 
cord and cervical enlargement can usually be made with exactness. For the 
middle and lower dorsal portions the following process is advisable: We 
ascertain as exactly as possible the upper boundary of the hypesthetic area 
on the trunk, and by referring to Head's tables we note which segment is sup- 
plied by the upper portion of the hypesthetic area that has been found; then 
by the aid of Gowers' tables we determine which vertebra and which of the 
spinous processes adjoin the damaged spinal cord segment. The surgeon 
must make his incision opposite this vertebral process, which is often sensi- 
tive to pressure. Usually the mode of operation is as follows : if, for exam- 
ple, hypesthesia is found pointing to the 10th dorsal segment, incision is made 
opposite the thoracic vertebra lying immediately above (the 9th), and also 
at the vertebral process next above (the 8th). 

Local diagnosis is much more difficult at the height of the lumbar enlarge- 
ment, of the conus terminal is and of the cauda equina. This is due to the 
fact that the anterior and posterior roots in this region do not at once leave 
the vertebral column when they emerge from the medulla spinalis, but they 
pass alongside a number of vertebral bodies before they disappear. There- 
fore, with a corresponding location of the tumor, and if there is pressure at 
the middle of the conus terminalis, the same symptom-complex may some- 
times arise as when the tumor is located in the region of the last lumbar 
vertebra or the sacral vertebra. With such an exceedingly difficult operation 
as an extensive one in the region of the lumbar vertebral column, it is wise 
to make as exact a local diagnosis as possible. 

i I recently saw a case of incomplete transverse lesion due to external compression 
by a metastatic sarcoma at the height of the sixth spinous process of the dorsal verte- 
bral column, and five days after the appearance of pressure phenomena the tendon 
reflexes of the lower extremity were abolished. Autopsy revealed a tumor at the height 
mentioned, but not in the lumbar enlargement. 



362 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 



V.X1 



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Fig. 159 shows distinctly that a focus at the height of the 12th thoracic 
and the first lumbar vertebra— this point is indicated by shaded lines — 
may spare the crural nerve situated laterally to it equally as well as if it 
were situated below the exit of this nerve further downward, and that the 
same symptoms may be produced in other nerve re- 
gions. For this reason great pains have been taken 
to establish differential factors, and it has fortunately 
been shown that in those lesions which act slowly, as 
tumors usually do, the differentiation between tumors 
of the lumbar enlargement and caudal tumors is not 
so perplexing as when disturbances appear suddenly; 
for example, those of traumatic nature. In the latter 
a distinct change in the individual vertebrae not in- 
frequently indicates the point of lesion, a change 
which is even more perceptible under X-ray examina- 
tion. Moreover, experience teaches us that in certain 
kinds of trauma (for example, a fall upon the but- 
tocks from a great height) only the fracture of cer- 
tain bones is found. Large tumors may now and 
then produce a decided and visible prominence of 
some portions of the lumbar vertebral column or of 
the sacrum. But this is rare, and in circumscribed 
tumors even local sensitiveness to pressure may never 
appear. 

We must, therefore, resort to other methods of dif- 
ferentiation. This is evident from the fact that on 
pressure in the lumbar enlargement bilateral paralytic 
Fig. 159. symptoms (at first of a motor nature) appear more 

rapidly — even if the tumor at first makes lateral pres- 
sure — than if it compresses the cauda equina. Dis- 
sociated sensory paralyses may develop more readily 
than in caudal tumors, while irritative phenomena are secondary (especially 
pain which in tumors of the cauda equina is usually very severe). On slight 
pressure upon the motor ganglion cells of the lumbar enlargement, irritative 
phenomena in the form of fibrillary and fascicular contractions are more 
common. Finally, a fact which Raymond noted in regard to the reflexes, 
namely, that when one of the deeper reflexes is lost those immediately higher 
may be increased favors disease of the lumbar cord. If the entire lumbar 
cord is not compressed but only its terminal portion, the conus terminalis, 
the following peculiar symptom-complex gradually arises: paralysis of the 
Madder and rectum, absence of the sexual reflexes as well as the Achilles tendon 
reflex, without paralysis of the muscles of the lower extremities. Simultane- 
ously a varying degree of anesthesia is noted in the anus and its surround- 
ings, the perineal region, the posterior skin of the scrotum, the mucous mem- 
brane of the urethra and bladder, and the posterior and internal surfaces of 
the thighs. 

Since the nerve fibers originating in the conus extend far down into the 
sacral portion of the vertebral column, and at a certain point lie next each other, 
pressure far below the conus terminalis may produce the same phenomena. 



THE SEAT, EXTENSION AND COMPOSITION 363 

Only a possible sensitiveness to pressure in the region of the corresponding 
vertebrae will then facilitate the decision. If the symptom-complex described 
be absent, it may be utterly impossible to make an exact topical diagnosis. 

The difficulty of determining the exact point for operation Avill be better understood 
by the details of a case I recently observed ( in which, unfortunately, exact diagnosis 
was impossible) than by long descriptions. It was the case of a man, aged 45, with no 
history of preceding infectious disease, certainly not of syphilis, but merely of pro- 
longed alcoholism. There was no hereditary predisposition of any kind, nor had there 
been any trauma. In the winter of 1900-1 pain appeared in the right thigh. He felt 
this both when in the recumbent posture and when standing, but not while sitting or 
walking. He was even able to make many walking tours in the Schwarzwald — exhaust- 
ing walks through deep snow and mountain climbing for as much as eight hours a day 
— without any pain. In the summer of 1901 the pains disappeared completely, but 
recurred the following winter. This time they were less severe but more continuous, 
and made sitting extremely difficult, while in the recumbent posture they were not felt, 
and they appeared in walking only when exhausted by several hours' marching. Shock 
from careless running down hill also produced them. 

His condition remained stationary until the winter of 1902-3. In January, 1903, 
the pains increased so much as to render long walks impossible. The patient did not 
consult a physician until the spring. He was placed upon potassium iodid and sodium 
salicylate. Warm carbonated baths were employed without improvement. In the course 
of the year the pain attacked the right leg and right foot, finally the left leg. Treat- 
ment at Oeynhausen for five weeks in September, 1903, as well as treatment by aspirin 
in the following October, did not benefit him. 

Pausing for a moment, we ask the question. What could have been diagnosticated 
during this time? Only sciatica, perhaps muscular rheumatism, was possible. But the 
course of the disease was opposed to such an opinion, since ordinary sciatica appears 
suddenly and disappears gradually. It is true relapses may occur, but they do not 
progress so slowly nor do they attack the other side, as in our case. Slight tenderness 
was at first also conspicuous, and this led us to believe that somewhere in the course 
of the sciatic nerve or its plexus or its roots compression had gradually developed, 
either in the bone, in the soft parts, in the sciatic nerve itself (perhaps in the form of 
neuroma ) , or in the vertebral canal. This view seemed to be substantiated when pain 
appeared in the left leg also. 

Toward the end of January, 1904, the patient was admitted to the hospital in 
Bonn. Brief examination revealed the following: A robust man whose internal organs 
gave no evidence of change, and whose bones and joints, including those of the vertebral 
column, presented no demonstrable anomaly. There was no sensitiveness to pressure 
in the vertebral column nor in the bones of the pelvis. Digital examination of the 
rectum disclosed nothing abnormal. A waddling gait, due to atrophy and weakness of 
the medial gluteal muscles, especially the right, was conspicuous, but the larger gluteal 
muscles were also atrophic, and this made the ascent of steps very difficult. There was 
no DeR in these muscles, but a marked diminution of electric contractility on the left 
side and total absence on the right side with all the different currents was demonstrated. 
In the course of the right sciatic nerve there was marked hypesthesia of the external 
malleolus and of the external border of the foot. The pain and temperature senses were 
also decreased, and in the toes the sensation of position was defective. The leg could 
be flexed by the exercise of very slight power. The right Achilles tendon reflex was 
absent, and the left was weak. Babinski's reflex was abolished on the right side but 
distinct on the left. Nothing really anomalous was noted in the region of the left 
sciatic nerve except the so-called sciatic phenomenon (pain in the sciatic nerve upon 
raising the extended leg). 

In the course of the crural nerve there was no demonstrable pathologic change. 
But it was difficult to raise the limb toward the trunk. Sensation about the anus, the 
perineum, and in the skin of the genitalia was absolutely normal, as were the bladder 
and rectal functions. The abdominal reflexes were good, the right cremaster reflex being 
well preserved, but the left very weak. There was pain in the right sciatic region which 
was especially distressing when lying upon the back. 



364 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 

This symptom precluded our designating the affection as a progressive disease of 
the sciatic nerves alone, because the gluteal nerves and those of the ilio-psoas were also 
implicated. We at first thought that a tumor was present, either in the lumbo-sacral 
plexus, within the sacrum itself, or pressing upon the roots in the vertebral canal. 
Chronic meningitis at the height of the cauda equina was contraindicated by the absence 
of syphilis, of tuberculosis, and of trauma, as well as by the circumstance that the 
affection did not more rapidly become bilateral but progressed with long pauses and 
gradations, although there was at first a decided and prolonged remission. 

Of course, there might have been disease of the sacrum itself, or of the lumbar 
vertebrae, or of both, yet no sensitiveness to pressure and no change in form ; even the 
X-rays revealed nothing abnormal. Nevertheless, such disease could not be absolutely 
excluded, although inability to find a primary focus (for example, in the prostate) 
was against the view of metastatic tumors. Normal conditions in the urine contra- 
indicated rare myeloma. A tumor of the vertebral column could not have caused pres- 
sure over the lower half of the lumbar enlargement and the conus terminalis because 
the pathologic phenomena developed much too slowly. There were neither bladder nor 
rectal disturbances, neither motor irritative phenomena, dissociated sensory paralyses, 
nor the peculiar anesthesia previously described. 

Compression of the fibers of the cauda equina, which, after excluding tumors with 
higher seat, finally comes under consideration, Avas favored by the severe pains so fre- 
quently observed in this condition ; it was contraindicated by the absence of bladder 
and rectal phenomena. It was difficult to understand how the ilio-psoas fibers originat- 
ing from the upper lumbar segments could be pressed upon, as well as the roots of the 
sciatic and gluteal roots, while the fibers of the crural and obturator nerves remained free. 

The possibility, and even likelihood, of multiple tumors of individual roots and 
nerve trunks was suggested ; of course the possible tumor or multiple tumors could 
not be exactly located, hence it was impossible to point out to the surgeon those lumbar 
vertebras below the first and the sacral vertebrae ( with the exception of the lowest which 
did not come under consideration) over which he was to operate. Opening the greater 
portion of the lumbar and sacral vertebral column simultaneously is such a serious 
operation that I did not think it advisable, particularly as the affection appeared to 
be arrested in the following months, and up to May there was decided improvement, 
especially a relief from pain. 

Then the constipation increased, and about the middle of June marked sensitiveness 
in the buttocks reappeared, as well as in the distribution of the sciatic. Atrophy of 
the gluteal muscle simultaneously increased, and there was loss of weight in the pre- 
viously strong and well nourished body. Slight difficulty in urination now appeared, 
and at the beginning of August circumscribed analgesia to the right of the anus ; the 
sphincter ani no longer contracted. The weakness in the sciatic increased; paresis of 
the right peroneal region appeared, and the Achilles tendon reflexes were abolished 
bilaterally, while the left plantar reflex was still distinctly preserved. The patella 
reflexes were feeble. About the middle of December a small bed-sore appeared, and the 
general debility increased. 

The pathologic picture now simulated a tumor at the height of the conus terminalis, 
and it seemed quite possible that the supposed growth might have gradually extended 
this far; but one or several tumors might also make pressure lower down in an inde- 
finable position. 

Considering this and the gradual decrease in weight, which could not well be 
accounted for, it seemed reasonable that a rapidly growing tumor, probably malignant 
and inoperable, might have developed from the sacrum or from the plexus and its 
intervertebral continuations. Opposed to this was the continuous absence of local pain 
on pressure of all the osseous structures which came into consideration, and the absence 
of deformity. Notwithstanding several attempts, no X-ray examination could be made 
on account of the great accumulation of feces which it was impossible to remove, and 
because of the great pain. 

Even in this stage it might be deemed advisable to make incision at the level of 
the sacral vertebrae, and, if no tumor were found there, either at once or subsequently 
at the height of the second lumbar vertebra; but since the tumor is as likely to be 
opposite the 3rd, 4th, or 5th lumbar vertebra, and because the operation would prob- 
ably be fatal, I cannot advise this method. At all events, it seemed certain that the 



THE SEAT, EXTENSION AND COMPOSITION 365 

operation might at first aggravate the bladder weakness and the bed-sore, and thus 
weaken the patient ; hence it was deemed wise first to try to heal the bed-sore, to say- 
nothing of the fact that a local sensitiveness to pain might arise and give us more 
definite indications. Unfortunately, after September the disease progressed with ex- 
ceeding rapidity, the patient constantly growing worse. Tne bed-sore rapidly enlarged, 
and finally necessitated the use of a permanent bath. The fever rose, the strength 
rapidly declined, the sciatic paresis increased, the patella tendon reflexes could no 
longer be evoked, and upon the loth of February, 1905, the patient succumbed to 
septic fever. 

The autopsy revealed the presence of a tumor 19% cm. long, beginning about 1% 
cm. below the lower end of the conns terminalis, and compressing the entire cauda 
equina. Its greatest breadth above was 1% cm.; below, 2% cm. An illustration of it 
is given in Fig. 107. The upper end of the preparation is somewhat sunken in conse- 
quence of gravity while in an erect position. It was loosely adherent to most of the 
roots of the cauda equina, and was detached without difficulty. Microscopical exami- 
nation showed it to be a sarcoma. 

If, therefore, operation had been attempted a few months earlier, it is likely that 
even in August or September the tumor would have attained such a size that many 
vertebrae must of necessity have been opened, and death would just as certainly have 
resulted — as in a similar case which I observed. How extensive the tumor was when 
the patient came to us could not be absolutely determined, but it was probably then 
quite large. The autopsy revealed nothing accurate as to its point of origin. It is 
just as likely to have started opposite the 4th or 5th lumbar vertebra as in the upper 
half of the cavity of the sacral vertebrae; therefore at the operation luck would have 
been a prominent element. 

For a long time no diagnosis of tumor of the cauda equina could positively be 
made. Our method of procedure was justified by the autopsy, especially as the pro- 
longed absence of bladder weakness in the light of similar experiences warranted us 
in assuming that it was not a caudal tumor. 

Fortunately the diagnosis is not always so difficult as in this case. Cir- 
cumscribed sensitiveness to pressure (which we searched for in this patient 
with negative results) usually points to a possible tumor within the sacrum 
or opposite the lumbar vertebral column, although it permits no absolute 
conclusions as to its length. In a case reported by Laquer x of Frankfort- 
on-the-Main, tumor formation within the sacral canal was diagnosticated on 
account of " strictly localized pain " in the middle of the sacrum which had 
existed for two years; there was also pain on pressure in the same area. 
^Yhen the surgeon made incision at the corresponding point, an extradural 
tumor was found which extended to the vertebral canal, and had compressed 
the cauda equina, but could easily be removed. The patient had suffered from 
almost unbearable pain, as well as disturbance in gait and transitory paresis 
of the bladder and rectum, and a bed-sore had already formed in the region 
of the trochanter; he was almost entirely cured by the operation. 

The local diagnosis is easier when, as in a case recently published by 
Engelmann, 2 a gunshot wound in the region of the sacrum indicates the 
location. In this case there was severe local and radiating pain. The hall 
had entered between the 5th lumbar and the first sacral vertebrae, and in this 
region also there was pain. Moreover the peculiar anesthesia previously de- 
scribed was noted, as well as cystitis and constipation, while motor paralytic 
phenomena were absent in the lower extremities, and of all the cutaneous and 
tendon reflexes in the legs only the Achilles tendon reflex was present. 

i Neurolog. CentralM., 1S91. 

2 Munch, medic. Wochenschr., 1904, Nr. 51. 



366 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 

These disturbances might have been attributed to an injury much higher 
up — in the lower portion of the conus terminalis — but the location of the 

wound indicated the region of 
the sacrum. The bullet, which 
was accurately located by X-ray 
examination, was found at the 
height of the first sacral ver- 
tebra, and removed. Four 
months after the operation the 
condition of the injured patient 
was almost normal. There 
was no pain; except for a 
periodic and slight dribbling 
of urine the bladder was nor- 
mal, and there was only a 
small hypesthetic zone around 
the anus. 

If, in this manner, we at- 
tempt to diagnosticate a tumor 
with certainty or even likeli- 
hood, we must also ascertain 
whether its location is extra- 
dural or intradural', and, if 
the latter, whether it lies be- 
tween the pia and dura, or 
between the pia and the spinal 
cord. Fortunately the latter 
is very rare. I say fortunately, 
because in this case the differ- 
entiation between acute trans- 
verse myelitis and pressure 
from tumor can scarcely be 
made; for, on the one hand, 
there are few sensory irritative 
phenomena, and, on the other, 
a general inhibition of conduc- 
tion, an interruption within 
the spinal cord, very rapidly 
takes place. 

We are still unable to dif- 
ferentiate between extradural 
and intradural tumors, al- 
though great attention has 
been devoted to this distinc- 
tion. Nor can we determine 
(important as this may be in 
deciding the question of oper- 
ation for tumor) whether a 
Fig. 160.— Sarcoma op the Cauda Equina. tumor grows anteriorly or pos- 




THE SEAT, EXTENSION AND COMPOSITION 367 

teriorly, or presses upon the spinal cord. We might suppose that in posterior 
pressure irritative phenomena and radiating pains would from the onset pre- 
dominate, and that in anterior pressure these might be absent or only slight. 
But this is by no means always the case. I have been convinced that, even 
with posterior pressure, the initial and neuralgic stage of the disease or pain 
may be almost wholly absent. 

It is easier to determine that a tumor grows laterally, or (at least chiefly) 
from one side. This for a time produces the familiar picture of semi- 
lesion, or Brown-Sequard's paralysis; i. e., motor paralysis predominates on 
that side of the spinal cord in which there is most marked pressure, on 
the other sensory paralysis, especially involving the pain and temperature 
sensations. 

Furthermore it is important to demonstrate whether the cause of com- 
pression is disease of the vertebral column or not; above all, whether tuber- 
culosis is present. This may be ascertained by an accurate history and close 
search for tubercular foci in the body, by the demonstration of fever, distinct 
changes in the form of certain portions of the vertebral column, and, after 
a long period of observation, by X-ray examination. Syphilitic disease of the 
vertebral column causing pressure upon the spinal cord is very rare, and, as 
a rule, is favorably influenced by antisyphilitic treatment. Exostoses develop 
with exceeding slowness, and in a given case can probably be recognized by 
radioscopy. 

Carcinomata and sarcomata of the vertebrae are most common. Since, in 
our experience, the former only secondarily develop in the vertebral bodies, 
therefore usually in elderly persons, wc should endeavor to ascertain whether 
there had been a prior operation in which a malignant tumor was removed, 
this fact being sometimes concealed. Above all, a possible primary malignant 
focus must be sought for, and unusual points of origin must not be excluded 
from consideration; for example, the prostate, the ovary, the thyreoid gland, 
the bronchi, etc. The primary cancer may have been strictly local. Persistent 
and severe pain on motion as well as during rest, particularly if extensive, is 
an especially noteworthy symptom. Vertebral cancer is usually multiple. Not 
rarely it is widely proliferated in the vetebral column, and may lead to 
sudden rupture with immediate symptoms of compression. Abnormally arched 
curvatures of the vertebrae may develop, while in tuberculosis of the vertebrae 
the angular kyphosis is much more likely. Sarcomata of the vertebral column 
may be primary or secondary. The former is perhaps more common. In the 
great majority of cases the secondary form is multiple, the primary less 
often so. 

In the diagnosis of secondary sarcomata the same considerations influence 
us as in eases of carcinomata. All regions of the body must be minutely 
examined for primary tumors. Multiple primary sarcomata often produce 
distributed and various kinds of pain. Pain in the bones on pressure and 
motion prior to the appearance of root symptoms, particularly neuralgic ones, 
favors an osteogenetic origin. Of course a positive diagnosis is not always 
possible, nor can we exclude tumors which originate from the spinal mem- 
branes. As in rare enchondromata, X-ray examination may in some cases 
aid us ; while extremely rare osteomata, exostoses, and chondromata are prob- 
ably always recognizable by this means. 



368 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 



THE COMPOSITION 

The diagnosis of the exact composition of tumors not originating from 
the bone but from the membranes cannot at present be made. Fibromata and 
fibrosarcomata are the most common, and, if the growth is not too rapid, can 
be diagnosticated. If other primary tumors, such as carcinomata, sarcomata, 
melanomata, are anywhere detected, and the previously described symptoms 
are present, we will not err in assuming metastases of these growths. Since 
in rare cases cysticerci and echinococci may be found, these also should be 
searched for. 

In regard to the course of the pathologic phenomena produced by such 
tumors and their possible cure, it may be stated that syphilitic neoplasms are 
amenable to treatment. It is, however, unfortunately true that nearly all 
others enlarge, they increase the pressure upon the spinal cord, and finally 
prove fatal, although often only after the lapse of years. Even the so-called 
benign tumors may thus have a malignant effect. Some tumors, especially 
neurofibromata, may remain stationary; in an interesting case reported by 
Henschen this author assumed with good reason that retrogression had oc- 
curred. 

Usually the pathologic phenomena gradually increase, although by no 
means uniformly. Comparatively often, especially in the first or neuralgic 
stage of the disease, there is prolonged or permanent amelioration of the pain. 
In the later stages when the symptoms of compression are prominent remis- 
sions may occur, probably because the tumor extends posteriorly, and there 
may be a temporary decrease in size, perhaps in consequence of softening, 
or because the spinal cord tract becomes accustomed to a steady, uniform 
pressure. If iodin is administered during such a period there may be 
an apparent improvement which may lead to an erroneous diagnosis of 
syphilis. 

It is of great significance in the diagnosis that, in spite of this advance of 
the pathologic phenomena (certainly in ordinary tumors which extend but 
slightly upward) , the height of the region involved in the pathologic phenomena 
remains approximately the same ; even these tumors do not extend upward, and 
they produce only the picture of a constantly increasing transverse lesion. 
This peculiarity enables us to differentiate circumscribed tumor formation 
from other spinal cord diseases — above all syphilitic meningomyelitis and the 
various forms of transverse myelitis. 

The fact, often so conspicuous, that pain is increased by sudden shock 
(for example, by coughing or sneezing) is not without value; but this is 
also observed in other diseases of the vertebral column, the vertebral joints, and 
the muscles of the back. Spastic rigidity of the muscles with tonic and clonic 
reflex spasms, often exceedingly painful and markedly developed, is not char- 
acteristic, although it frequently occurs; for these disturbances may also be 
observed in other diseases of the spinal cord. 

As a rule, the disease is fatal, and terminates after extreme suffering in 
consequence of cystitis, pyelitis, or bed-sores. 



TREATMENT 369 



TREATMENT 



Except for antisyphilitic treatment in the rare gummatous tumors, our 
only hope is in timely operative removal of the compressing tumor. Of course, 
this treatment should be tried before operating, as syphilis cannot be excluded 
with absolute certainty. Even if the patient is syphilitic, and gummata are 
found in other portions of the body, pressure upon the medulla spinalis may 
nevertheless be due to other tumors. 

While the diagnosis of tumors of the spinal cord as well as operative inter- 
ference for the purpose of removal appeared impossible until a few decades 
ago, yet a neoplasm of the spinal cord membranes was for the first time cor- 
rectly diagnosticated in 1887 by Gowers and Horsley. They localized it, 
and successfully removed it by operation. Others have since followed, and 
much more satisfactory results have been obtained than in analogous opera- 
tions for tumor of the brain. Statistics up to the beginning of 1905 which I 
collected, and including a case not yet published which terminated unsuc- 
cessfully, show 62 such cases, of which 24 were cured or greatly improved, 
therefore 38 per cent. Here it must be remarked that the patients who 
improved were saved from certain death by operation. In 7 cases of my own 
in which operation was performed I was so fortunate as to reckon 3 recover- 
ies and one permanent improvement. In an eighth case, the one not yet 
published, death followed the operation. In a ninth case, also not yet pub- 
lished, there was a metastatic tumor which had not been recognized as such; 
operation did not aggravate the case, but, after the wound had healed, death 
occurred in consequence of a sarcoma of the thyreoid gland. 

The conditions are most favorable when tumors are situated at the height 
of the dorsal vertebra, less so when in the cervical and lumbar regions 
including the cauda equina. Nevertheless Putnam, Krause, and Park have 
successfully operated on tumors at the height of the 3rd cervical segment, 
while Bardenheuer failed with a tumor diagnosticated by Minkowski and 
myself. On the other hand, Henschen and Lennander in Upsala, and Harvey 
Gushing in Baltimore, have successfully removed tumors in the lower portion 
of the cervical enlargement and at the height of the oth and 6th cervical 
vertebral arches. 

A tumor opposite the sacrum, extending from its middle almost to the 
lowest lumbar vertebra, diagnosticated in France by Laquer, was successfully 
removed in Frankfort by Eehn. One of our patients with a large tumor 
of the sacrum which extended upward to the first lumbar vertebra succumbed 
in spite of an operation performed by Schede. Extensive operations in this 
region, especially in bony persons, are very serious on account of the large 
and deep osseous wounds and the unavoidable aggravation of the preceding 
pathologic symptoms, while the opening of the sacrum and the removal of indi- 
vidual lumbar vertebrae are much less dangerous. 

The teclinic of the operation is not especially difficult although the utmost 
caution is necessary. Too much pressure upon the spinal cord must be avoided, 
for there is but slight possibility of its regeneration, and the paralysis becomes 
profound and incurable. Especially in operations on the upper portion of the 
medulla spinalis and in intradural tumors, we must prevent the outflow of the 
cerebrospinal fluid ; according to Sick this may be done by carefully tying the 
25 



370 NEOPLASMS OF THE SPINAL CORD AND OF ITS MEMBRANES 

dural sac with a thread prior to opening the dura. Finally, we must guard 
against severe hemorrhage during the operation; this will give the patient a 
better chance to recover from the aggravation of the paralysis and other symp- 
toms of his disease immediately after the operation. I cannot enter into the 
details of these conditions which belong to my surgical colleagues. They have 
a particularly difficult case when it is ascertained that the tumor is not situ- 
ated behind or alongside the spinal cord, but has grown anteriorly to the 
medulla spinalis, and is very difficult to detach. Sometimes the tumor cannot 
be entirely removed, or it may happen that in spite of all precautions there 
are severe secondary injuries. 

But in the successful cases Ave always have the satisfaction of having 
saved the patient from great suffering and certain death. It is the duty of 
the internist to make a diagnosis as early as the data at hand will permit — 
chiefly to ascertain that there is neuralgic pain in the trunk and extremities, 
as well as increasing motor, spastic, or sensory paralysis with or without pre- 
ceding neuralgia, constantly to consider the possibility of tumor, and to seek 
for its first symptoms. It is very humiliating to discover at the autopsy that 
we have failed to diagnosticate a tumor which might readily have been re- 
moved, and that in consequence of our lack of knowledge or carelessness the 
patient has succumbed to a painful death. 



MYELITIS 
By E, v. LEYDEX and P. LAZARUS, Berlin 

HISTORY 

A glance at the development of the pathology of the nervous system dur- 
ing the last century will reveal the wonderful and complete reconstruction 
that has taken place. This is especially true of diseases of the spinal cord, 
for a century ago the knowledge of the normal and diseased spinal cord was 
exceedingly scant; but to-day this is one of the best cultivated realms of our 
science. At the beginning of the preceding century all pathologic processes 
of the cord and its membranes were included under the same designations, 
" rachialgitis " and " spinitis " (Brera, Jos. Frank), also as notomyelitis (Hil- 
debrandt). The first attempt at a separation of disease of the cord from that 
of its membranes, occurred in a dissertation by Harless (Erlangen, 1814) 
and by Klohss ( LEalle, 1820) ; but only in the celebrated writings of Ollivier 
( Edition I, which was awarded a prize by the Academy of Paris) and in the 
works of Abercrombie (1828) did myelitis attain a substantive position and 
a clinical description which was a classic for that period. 

These researches contain a good description of the macroscopic changes 
of the inflamed cord. The altered consistence of the spinal cord was differ- 
entiated as softening (myelomalacia) and hardening {sclerosis), the latter 
according to the views of pathology at that time being regarded as the starting 
point of an inflammatory process. In reference to softening in the cord, and 
also in the brain, the red, yellow, and white variety were differentiated. 
White (central) softening was soon shown to be a post mortem change and 
without pathologic significance. Eed, and white softening, almost up to the 
present time, were regarded as true representatives of spinal cord inflammation. 

The next advance we owe to microscopy and in connection with this the 
improved methods in hardening and staining (M. Midler's chromic acid fixa- 
tion, L. Clarke's clearing with turpentine, Gerlach's carmin staining), by 
which processes the normal and pathologic anatomy of the spinal cord were 
revealed. 

The first important discovery in pathologic histology of the cord was due 
to Gluge in Halle (1850). He described, what was called after him, the 
" inflammatory globules " corresponding to our present granular cells. To 
demonstrate them a spread of tissue, to which a little dilute caustic soda has 
been added, is placed under the microscope and even with moderate mag- 
nification they are readily seen; by their black, granular consistence granular 
cells are sharply defined from the pale medullary substance (Plate I, Fig. 3 a). 
The great simplicity and rapidity of this method of examination renders it 

371 



372 MYELITIS 

still useful, provided it is followed by a minute microscopic investigation after 
hardening and staining. Granular cells are found in great numbers within 
the inflamed substance of the cord in myelitis, but they are also seen in other, 
pure, degenerative lesions in the brain and spinal cord. 

This simple method led to one of the most important advances in the 
realm of diseases of the spinal cord; we refer to the discovery of ascending 
and descending columnar degeneration, by one of the most original medical 
investigators, L. Ttirck, in Vienna (1851), 1 who was also the founder of laryn- 
goscopy. It is true Cruveilhier, in his celebrated Atlas on Pathological Anat- 
omy (Tom. II, 1835-1842), had described and depicted columnar degeneration 
as " Degeneration fasciculaire," without, however, pointing out its special 
significance. Leyden, in one of his first publications 2 upon diseases of the 
spinal cord, connected gray degeneration of the posterior columns in tabes 
with Tiirck's ascending, secondary degeneration and thus constructed his 
theory of the pathogenesis of tabes (1863). Tiirck's discovery further led 
to the separation of column or system diseases from focal diseases of the cord, 
which is still in force to-day. Focal diseases to a great extent are included 
with acute and chronic myelitis, maladies with which we are particularly 
concerned. 

PATHOLOGICAL ANATOMY OF MYELITIS 

For a long time myelitis was regarded as identical with softening {myelo- 
malacia). The inflamed spinal cord focus, especially in advanced cases, may 
be recognized on palpation by its decreased resistance, from a distinct swell- 
ing, as well as from an active injection of the meningeal vessels. On section 
of the focus the softening becomes even more marked, for the medullary sub- 
stance protrudes upon the cut surface; the color is whitish-yellow or reddish, 
not rarely speckled red, in consequence of disseminated hemorrhagic foci 
or capillary apoplexies. In more advanced cases the pia mater surrounds the 
completely softened cord substance, which has a pappy consistence upon the 
cut surface, like a loose sac. In other cases, in which the softening is not 
well defined, the normal structure of the gray and white substance is more or 
less obliterated. 

Not every softening is of inflammatory nature; there is also necrobiotic, 
ischemic and traumatic softening without inflammation. On the other hand 
there are not infrequently spinal cord inflammations without softening, to 
which Leyden called attention in his book upon " Diseases of the Spinal Cord " 
(1872-1876). In myelitis without softening there may be no visible 
macroscopic change; only the succeeding histologic examination, either in 
smear preparations or after hardening the cord, decides the diagnosis of mye- 
litis. Mostly in this form, which belongs to the early stage, the histopathologic 
lesions of myelitic softening are found to an attenuated extent, i. e., only 

i L. Tiirck, " Ueber ein bisher unbekanntes Verhalten des Biickenmarks bei Hemi- 
plegien." Zeitschr. d. Aerzte zu Wien, 1850. — " Ueber secundare Erkrankungen einzel- 
ner Riickenmarksstrange und ihrer Fortsetzungen zum Gehirn." Wiener Sitzungs- 
berichte 1851, VI. u. XI. Heft. 

2 Leyden, " Die graue Degeneration der hinteren Ruckenmarksstrange." Berlin, 
1863 und Deutsche Klinik, 1863, 23, 



PATHOLOGICAL ANATOMY OF MYELITIS 373 

swelling, or at most beginning destruction of the ganglion cells, medullary 
sheaths and axis cylinders, as well as disseminated round cell infiltration and 
more or less numerous granular cells. Plate I, Figs. 1, 2, and 3 gives an idea 
of the pathologic lesions in myelitic softening. 

The alterations of the parenchyma in myelitis are of a destructive nature; 
those of the interstitial tissue, that is of the neuroglia, are of productive kind, 
and the lesions of the vessels are of congestive, i. e., of exudative nature. 

The ganglion cells, according to the stage of inflammation, present degen- 
erative pictures of varying kinds. At the onset of inflammation the ganglion 
cells swell, the multipolar anterior horn cells particularly become clumpy, 
they do not permit the recognition of Xissl's bodies, or when present they are 
cloudy, finely granular and often show hyaline swelling. The cells stain poorly 
and are subject to chromatolysis. that is achromatosis. The nucleus moves to 
the periphery of the cell, it contracts, and finally can hardly be differentiated 
from the body of the cell, which has been converted into a homogeneous, 
structureless clump. In the terminal stages of the inflammation the ganglion 
cells may perish and be completely destroyed under conditions of pigment 
degeneration and vacuole formation, or they contract including their proc- 
esses (atrophy). According to Marinesco. proliferated glia cells are said to 
penetrate the protoplasm of the ganglion cells and analogous to phagocytosis 
these glia cells are able to take up the disintegrated substance ("neurono- 
phagous glia cells "). 

The nerve fibers are also destroyed in a manner parallel to the cellular 
changes. In the beginning of acute myelitis the axis cylinders swell, they 
show varicose thickening and sometimes fine granulation, which indicates the 
threatened destruction. The medullary shcatlis show globular-shaped swollen 
or contracted areas ; they are finally destroyed with the signs of fatty degen- 
eration (myelin globules) and of vacuole formation, so that the space occu- 
pied by the nerve fibers presents a cavity. In a transverse section these cav- 
ities appear like vesicles; they are larger than the normal transverse section 
of a nerve fiber and still contain a few axis cylinder fragments, without medul- 
lary sheaths or medullary sheath particles, which are changing into myelin 
globules; very often also granular cells are present. Fig. 161 (myelitis after 
gonorrhea) illustrates this change which was described and named by Leyden 
a "vesicular condition." (Compare also Fig. 162, syphilitic myelitis.) 

This specimen was obtained from Paul E., a workman, aged 27. who was admitted 
to the Clinic on March 2, 1890, and died on March 9th. There was complete flaccid 
paraplegia, which had developed almost suddenly, 2 days prior to admission to the 
Clinic. The patella reflexes were abolished, and up to the navel, in a horizontal line, 
sensation was altered (hypalgesia, hypesthesia) . The sphincters were incontinent. 
The purulent urethral secretion contained gonocoeci. 

The necropsy revealed a hemorrhagic, ulcerative, gonorrheal cystitis, a phlegmonous 
para- and pericystitis, which had distributed itself to the retroperitoneal tissue, besides 
there was also a fibrinous peritonitis. 

In the lower portion of the dorsal cord there was a moderately marked myelo- 
meningitis, that is, a leukomyelitis to the longitudinal extent of several vertebras. 
The "vesicular" condition of the diseased area (Fig. 168) is easily noted; with greater 
magnification the broadened transverse sections of the swollen axis cylinders and a 
few granular cells may be seen. The pia reveals great cellular infiltration, however, 
there are no vascular changes, nor bacteria. It could not be decided whether the 
myelitis, in this case, was due to a toxic metastasis or to a propagation of the gonor- 



374 



MYELITIS 



rheal inflammation, from the bladder to the spinal cord (by contiguity) as an ascend- 
ing neuritis ; in favor of the latter view are the phlegmonous pericystitis and the 
retroperitonitis (compare Zeitschr. f. klin. Med., 1892, Bd. XXI). 

The products of decomposition of the nervous tissue sometimes have the 
appearance of corpora amylacea. The destroyed masses of the medullary sub- 
stance are taken up by the granular cells and removed; these cells are found 




Fig. 161. — Inflammation of the Dorsal Cord (Vesicular Condition). 

in every inflammation and also in non-inflammatory spinal cord softening, 
for instance, after tying the abdominal aorta (Stenon's experiment). We 
shall not discuss the point whether these granular cells, which serve as trans- 
porters, consist of leukocytes (wandering cells) or of fixed connective tissue 
cells, that is, products of transformation of glia cells (Senator). 

In place of the destroyed parenchyma (in conformity with Weigert's law) 
a substitute proliferation of the intermediary connective tissue takes place. 
At the onset, the vesicular stage, the glia meshes appear dilated or irregu- 
larly lengthened, due to the swollen and destroyed medullary sheaths and 



PATHOLOGICAL ANATOMY OF MYELITIS 375 

axis cylinders. The glia cells and fibers swell, especially in the vicinity of the 
vesicular spaces, with moderate increase of their nuclei. The protoplasm of 
the glia cells may further also lose its structure and their processes may 
undergo varicose enlargement. 

In the stage of softening the glia stroma may finally break down, so that 
only a detritus of granular cells, medullary globules, fat drops, red blood cor- 
puscles, leukocytes, pigment layers, as well as destroyed axis cylinders and 
medullary sheaths, remain. These fragment masses are taken up by the gran- 
ular cells and are removed. To this a proliferation of the interstitial tissue 
is added, which begins in the periphery of the focus of softening, gradually 
proceeding toward the center and finally by retraction leads to a " sclerotic 
cicatrix." Here there are often hyperplastic connective tissue cells having 
numerous processes, " spider cells or Deiter's cells " ; further atrophic ganglion 
cells and nerve fibers, as well as degenerated vessels; occasionally a cyst-like 
space remains in the center of the sclerotic focus. 

Sometimes a few axis cylinders (in multiple sclerosis) in the fibrous cica- 
tricial tissue may retain the power of conduction and thereby regain their 
functional activity. "Whether besides this form of restitution a regeneration 
of ganglion cells and nerve fibers takes place, is very questionable. In the 
lower animals a regeneration of the central nervous tissue has been demon- 
strated by the formation of new fibers with certainty. Fickler (Deutsche 
Zeitsclir. f. Nervenhk., 1899, Bd. XYI), maintained that the nerve fibers of 
the spinal cord, also in man, are capable of regeneration even up to a complete 
recuperation of function, so long as the blood vessels of the cord are still 
intact. Fickler, in a case of compression of the cord, describes these newly 
formed fibers which were in connection with ganglion cells below the area of 
compression. 

The lesions of the vascular apparatus are sometimes only trivial in mye- 
litis, in other cases these may dominate the anatomical picture. In pure, 
toxic, myelitis the inflammation is said to be entirely limited to the paren- 
chyma, while in the infectious form the hemorrhagic, that is, the exudative 
variety predominates. 

The circulatory system reveals the following lesions: congestive hyperemia, 
vascular dilatation, embolism, thrombosis, and rupture of the medullary 
vessels with capillary or even larger hemorrhages. Hemorrhagic myelitis can 
not be positively differentiated, macroscopicallv from hematomyelia. In con- 
nection with inflammatory spinal cord hemorrhages, as in the brain, there 
is serous infiltration of the surrounding medullary substance and further on 
a colliquative necrosis. The extravasated blood is absorbed, larger or smaller 
portions of the inflammatory focus undergo fatty degeneration and are trans- 
formed into yellowish masses (yellow softening). Then there is resorption 
of the fatty products of degeneration and further a proliferation of the neu- 
roglia tissue with succeeding cicatrization. Included in the contracted cica- 
tricial tissue, finally we find as the residue of former hemorrhages, either 
amorphous pigment or hematoidin crystals (compare Plate I, Fig. 3 a, Expla- 
nation of the plate). 

Fig. 3 a, Microscopic picture of the softened masses (magnified 300 times) from a 
case of hematomyelia during confinement (published by E. v. Ley den, Zeitschr. f. klin. 
Med., Bd. XIII, p. 225). The patient, Mrs. K., aged28, during the first days of the 



376 MYELITIS 

puerperium developed total sensory and motor paralysis of the legs with paralysis of 
the sphincters, loss of the tendon reflexes, girdle pain and bed-sores. The patient died 
of sepsis upon the 51st day of the disease. At the autopsy, there was found in the 
lower thoracic cord, a soft, pappy, almost fluctuating, focus of softening 5 cm. long. 
Histologic examination revealed that this soft, yellowish mass consisted of the follow- 
ing elements: (1) Numerous large globules and granular cells; (2) fragments of nerve 
fibers with myelin drops and swollen, partly granular and fatty degenerated axis cylin- 
ders; (3) a few red corpuscles; (Jf) numerous, rhombic hematoidin crystals; (5) large 
cells with granular, rusty colored contents {pigment granules) ; (6) medullated nerve 
fibers, the axis cylinders of which are stained yellowish brown and reddish brown 
(hematoidin) , while the medullary sheaths remain unstained. 

Fig. 3 b-g, Sections through the spinal cord; the green color is due to the chromic 
stain. Abbreviations: s. D. = secondary degeneration (ascending and descending); 
R. d. = border degeneration ; H. — hemorrhagic focus ( Fig. 3 b, c, d, transverse section 
above the focus [cervical and thoracic cord], e, f, g = transverse section below the 
focus [lumbar cord] ) . In the cervical and thoracic cord the ascending degeneration of 
the posterior columns is recognized from the lighter color; in the vicinity of the focus 
of softening the posterior columns are attacked in toto, upward the degeneration 
passes to GolPs columns. Downward from the focus of destruction, in the latero- 
posterior columns, traces of descending degeneration can be demonstrated. The border 
degeneration is very marked above as well as below the focus. Further, a hemorrhage 
is noted passing through the entire cord, from the left GolPs column in the cervical 
cord to the posterior column in the lumbar cord, which originated from the focus of 
softening (central tube hemorrhage) and which has distributed itself in the vault of 
the posterior columns, upward and downward. The white medullary mass has suffered 
more than the gray. 



Of further lesions in the vessels, apart from the congestive hyperemia of 
the initial stage, infiltration and inflammation of the vascular wall itself is 
noteworthy. This is characterized by small cell infiltration of the vessel 
sheaths, further by serous transudation (inflammatory edema), or leukocytic 
exudation into the surrounding medullary tissue. Further hyaline degener- 
ation and finally even obliteration of the vessels may take place; then by 
ischemic processes either a necrotic inflammation or a cuneiform focus of 
softening develops, the apex being directed toward the center of the trans- 
verse section of the cord. 

It must, however, be emphasized, that this perivascular leukocyte infiltra- 
tion may not take place and that there are inflammations of the cord in which 
also the other circulatory disturbances are absent and only an (acute) degen- 
eration of the nerve parenchyma can be demonstrated. Lubarsch regarded 
these cases as degenerative inflammations, in contrast to the exudative forms, 
which run their course with small cell infiltration. 

The central canal is sometimes dilated in myelitis, occasionally even filled 
with a fibrino-purulent exudate. In central myelitis the vascular lesions and 
the nuclear increase are most marked in the commissures. 

If the inflammation begins in the white substance of the cord (UuJco- 
myelitis) there are usually lesions in the membranes of the cord. A primary 
meningitis may distribute itself along the vessels or the connective tissue septa, 
to the substance of the cord (border myelitis, perimyelitis, meningo myelitis) . 
In this complication the meningeal vessels are dilated, sometimes thrombosed, 
the pia mater showing small cell infiltration, thickened, being turbid and 
adherent to the medullary substance. Not rarely hemorrhages arise from the 
inflammatorily altered pia blood vessels. 



PATHOLOGICAL ANATOMY OF MYELITIS 377 

Myelitis may, especially in disease of the cervical and lumbar enlarge- 
ments, attack the nerve roots and even involve their extraspinal continuations; 
in the latter we recognize histologically, swelling of the axis cylinders, destruc- 
tion of the medullary sheaths and proliferation of the interstitial tissue, par- 
ticularly around the vessels. These degenerative and neuritic lesions, in dis- 
ease of the anterior horns, for example in poliomyelitis anterior, follow the 
affected nerve to its terminal distribution in the muscle and this structure 
presents atrophy. 

The bacteria found in the cerebrospinal fluid in myelitis will be described 
later on. 

The pathologic lesions that have been described may occup} T the entire 
transverse section in focal myelitis, or may be limited to a special area. Small 
foci usually do not show softening or only to a very slight extent. If the 
areas in the vicinity of the myelitic focus are microscopically investigated, we 
will find that 'the pathologic change always extends further than can be ob- 
served with the naked eye. To these, after myelitis has existed for a few 
weeks, Turck's secondary degeneration is added, which distributes itself upward 
in the sensory, and downward in the motor, fiber systems. 

Descending degeneration takes place in the motor tracts, such as the pyram- 
idal-anterior and lateral column tracts, and those from the cerebellum, the 
optic thalamus, the corpora quadrigemina and the red nucleus (v. Monakow's 
rubrospinal tract) and in the descending conduction tracts in the anterior and 
lateral columns. Ascending degeneration, on the other hand, occurs in the 
sensory conduction tracts, and especially all posterior column tracts (includ- 
ing Lissauer's zone), whose cells of origin are to be sought in the extradural 
spinal ganglia ; further the cerebellar lateral column tracts, whose trophic cen- 
ter is situated in Clarke's columns, finally Gowers' bundle and a part of the 
anterior column bundle. Only exceptionally is a retrogressive degeneration 
observed in the motor and sensory tracts opposite to the direction of conduc- 
tion, which at most only amounts to a few centimeters. An idea of the altered 
form and distribution at various heights of the cord, of ascending and descend- 
ing degeneration, is shown by the illustrations on Plate I, Figs, 1, 2, and 3 
(compare also Fig. 168). The form thus described corresponds to focal 
{transverse) myelitis, the first and most typical variety of spinal cord inflam- 
mation. 

The second form in which myelitis appears is acute disseminated or mul- 
tiple myelitis, in which a number of inflammatory foci are distributed through- 
out the cord. This has much similarity to multiple sclerosis; the latter may 
be regarded as a frequent termination of acute disseminated myelitis, such 
as occurs especially after acute infectious diseases. We are justified in assum- 
ing that sclerosis may originate from a spinal cord disease of long standing, 
and for this reason the condition belongs to chronic myelitis; according to 
Schmaus's recent investigation multiple sclerosis cannot be denied an inflam- 
matory character. 

The individual foci of disseminated myelitis, as a rule, have but moderate 
size and extent, they rarely include more than half a transverse section of 
the cord and one half the height of a vertebra. Their distribution varies 
greatly ; sometimes they are found in several small " plaques " in the vicinity 
of a larger focus. In other cases they are found all through the cord, even 
26 



378 MYELITIS 

up to the medulla oblongata, to the pons, mid-brain and brain. Generally 
their number is greater in the upper part of the spinal cord (cervical enlarge- 
ment) ; rarely are the foci more numerous in the mid-brain and bulbus (symp- 
toms of bulbar paralysis). 

Besides focal and disseminated myelitis we also recognize a third form, 
inflammation of the gray substance {poliomyelitis), which from its localiza- 
tion and symptoms differs materially from the two others. It presents the 
anatomical condition of " essential infantile paralysis " first described by 
Heine (1840), as well as of "essential atrophic paralysis of adults" (Du- 
chenne) ; the clinical picture was accurately investigated by Eoth (Basle), 
L. Clarke, Hay em, Charcot, and Kussmaul, the last author naming the disease 
anterior poliomyelitis; Leyden in a number of rapidly fatal cases of infantile 
paralysis was able to demonstrate the inflammatory character by discovering 
cellular infiltration in the anterior horns (Westphal's Archiv, 1864). Multi- 
ple myelitis and poliomyelitis present such a totally different clinical picture 
from transverse myelitis that they require a special description. (This will 
be found in another chapter of this book.) 

The following questions are justified from the description of the pathology 
of myelitis : 

(1) From which tissue elements does myelitis take its starting point? 

(2) How do we recognize that we are dealing with an inflammatory 
process ? 

(3) Row do we differentiate myelitis from other degenerations or from 
changes which occur in connection with hemorrhages or compression of the 
spinal cord? 

The answer to these questions is quite indefinite. Thus the starting point 
of the inflammation was referred in part to the ganglion cells (Charcot, Riss- 
ler, Kahlden), 1 partly to the circulatory apparatus (P. Marie, Schmaus, 
Mayer), and partly in the glia tissue (Marinesco, Goldscheider). Accordingly 
a parenchymatous, vascular and interstitial form of myelitis was recognized. 
In our opinion one and the same inflammatory irritant may attack the gan- 
glion cells and the nerve fibers, at one time or the neuroglia, that is the 
interstitial tissue, or the blood vessels with greater severity and correspond- 
ingly lead to a predominance of parenchymatous, interstitial or vascular 
lesions; all three are, however, so coordinated as in inflammations of other 
organs, for example, of the heart or of the kidney, in which the inflammation 
is not exclusively limited to the interstitial tissue, that is to the parenchyma 
alone, but constant^ attacks both, although not always to the same extent. 
In this the circumstance must be included that in myelitis an irritation to 
the supporting structure and upon the walls of the vessels acts differently 
than upon the specific nervous tissue; while the first react to irritation with 
increased activity — hyperemia, exudation and cell segmentation — the specific 
nervous elements are destroyed as soon as the irritation has attained a certain 
degree (Rissler). 

Nor can the boundary between acute degeneration and inflammation of 

i An accurate review of the literature will be found in Redlich's article Centralbl. 
f. allgem. Pathologie und pathol. Anatomie, 1898, Bd. IX, Heft 3 u. 4, and in W. Mayer's 
article " Myelitis acuta " in the " Arbeiten aus dem Institut fur Anatomie u. Physi- 
ologic des Centralnervensy stems," 1900, Heft VII. 



PATHOLOGICAL ANATOMY OF MYELITIS 379 

nerve fibers and ganglion cells be sharply defined. According to Ziegler's defi- 
nition, inflammation is to be regarded as a primary, degenerative tissue lesion 
which is only secondarily succeeded by changes of the vessel walls and further 
on by exudative processes. Thus, in disseminated myelitis Kahlden was able 
to discern foci of varying intensity and of different ages. The initial signs 
are exclusively marked by degenerative cell changes; then comes destruction 
of the axis cylinders, and finally the lesions of the vascular apparatus. Also 
in myelitis experimentally produced in animals, by the injection of bacteria 
or their toxins, all grades of inflammation were found, from simple degenera- 
tion to hemorrhagic softening. 

While according to this view the different changes of the individual tissue 
constituents in myelitis are coordinated and only represent varying forms of 
the same fundamental process, other authors regard as the essential signs of 
inflammation the "inflammatory circulatory disturbance" (Schmaus). This 
consists of congestive hyperemia, extravasation of blood, increased lymph 
transudation, and migration of leukocytes, which especially infiltrate the adven- 
titia and the perivascular lymph spaces. Finally the walls of the vessels may 
become thickened and show hyaline degeneration. A recent author, Douglas 
Singer, even refers the majority of cases of myelitis, not to inflammation, 
but to a thrombosis of the spinal arteries and necrosis in their area of dis- 
tribution. 

The view of the vascular character of myelitis in our opinion is only 
proper in infectious or toxic myelitis which arises through the circulation, 
especially in poliomyelitis, as well as in the disseminated forms, in which the 
inflammatory changes of the vascular apparatus are particularly prominent. 
In a similar manner primary disease of the walls of the vessels (syphilitic 
endarteritis, arteriosclerosis, thrombosis), further emboli in the region of 
the spinal cord arterioles, terminal arteries (Adamkiewicz, Kadyi), lead to 
nutritive disturbances and multiple degeneration. 

In contrast to these there are undoubtedly myelitides, even of the most 
acute type, in which the vascular lesions are secondary. On the other hand, 
the previously described lesions of the ganglion cells and nerve fibers, as well 
as of the neuroglia, further the epithelioid and fatty granular cells, can not 
be regarded as absolutely characteristic of spinal cord inflammation, as these 
are also observed in other non-inflammatory degenerations; even inflamma- 
tory degeneration can not be differentiated from the descending form. Fur- 
ther in disseminated myelitis one focus may only present the signs of acute 
degeneration of the nerve fibers and ganglion cells, while another focus shows 
interstitial inflammation. In this condition, as Obersteiner and Eedlich quite 
justly remark, there are only quantitative differences, so that a positive dif- 
ferentiation of acute inflammation from acute degeneration is impossible. 
For this reason Virchow's opinion is also applicable to myelitis, that inflam- 
mation is no t a uniform process presenting constant factors. 

Finally the differentiation of primary spinal cord hemorrhage from hemor- 
rhagic myelitis is very difficult especially in old foci. Ollivier separated hem- 
orrhages in the spinal cord (hematomyelia) from true acute myelitis. His 
reports are based upon the results of autopsy, while the clinical observation 
of the cases was not always clear in .regard to their development and course. 
According to Leyden's investigation and also to our present experiences, 



380 MYELITIS 

primary hemorrhages of the spinal cord are exceedingly rare, excepting those 
due to concussion. Post-infectious myelitis, in the transverse section, may 
present itself as hematomyelia. Thus Leyden saw a case of myelitis of this 
kind after the patient had suffered from influenza, the disease rapidly termi- 
nating fatally on account of the ascent of the process to the medulla oblongata 
which gave rise to respiratory paralysis. Almost throughout the entire length 
of the spinal cord numerous smaller and larger hemorrhages, in part between 
the pia and medullary substance and in part around the spinal cord vessels, 
were found. Careful investigation, however, unquestionably revealed the signs 
of an infectious myelitis (Plate I, Fig. 1). 

On the other hand, after serious traumatic lesions, a disease of the spinal 
cord with a rapidly fatal course may appear, in which at the post mortem 
hardly the traces of an effusion of blood will be noted, but, on the other hand, 
the distinct signs of myelitis. Leyden saw this condition in a case of com- 
pression of the cervical cord due to a detached odontoid process in a man, 
who broke his neck from the results of a fall. It has also been demonstrated 
in other organs, that in connection with traumatic hemorrhage, inflammatory 
lesions may appear. 

Larger effusions of blood, analogous to apoplectic cerebral foci, are rare 
in the spinal cord. Such conditions have almost always been seen only after 
rupture of the hemorrhage from the pons or the medulla into the fourth 
ventricle. In regard to the distribution of hemorrhage in the spinal cord, 
the blood gradually forces itself to the point of least resistance, and this is the 
top of the posterior columns. In this region the hemorrhage may take the 
shape of a red thread, which traverses the cord in this area in a longitudinal 
manner. Leyden has reported a case of this kind (Zeitschr. f. klin. Med., Bd. 
XIII). This was one of secondary dorsal myelitis occurring during the puer- 
perium (compare Plate I, Fig. 2). 

Such observations lead us to the conclusion that anatomically a sharp 
separation between primary hemorrhage with subsequent myelitis from pri- 
mary hemorrhagic myelitis is only possible in the rarest cases, so that this 
differentiation can not be maintained for clinico-practical purposes. 

The conditions are similar with another form of spinal cord softening, 
namely, compression softening, that is the much discussed compression mye- 
litis, which H. Oppenheim regards as more frequent than primary myelitis. 
Several authors have separated this form from true myelitis and have desig- 
nated it as stasis edema of the spinal cord. Leyden has included compression 
softening with myelitis on account of the similarity of the clinical picture. 
Prominent histologists, such as Schmaus, also consider it identical with mye- 
litis. In pressure paralysis we must, however, differentiate two processes: 

(a) Pure compression from tumors, which, as a rule, are situated within 
the spinal canal, forming upon the dura or pia mater (endotheliomata, fibro- 
sarcomata, fibromata) . They may compress the spinal cord to a narrow band 
and occlusion of the vessels produces ischemic processes, in which inflamma- 
tory lesions hardly arise. This is simple compression of the spinal cord, 
which, as is well known, may be relieved by surgical measures. On the other 
hand 

(b) In compression due to caries of the vertebras the inflammation may 
pass to the membranes of the cord (pachymeningitis tuberculosa, epidural 



PATHOLOGICAL ANATOMY OF MYELITIS 



381 



exudates and fungous proliferations) and may even permeate the cord itself. 
Secondary myelitis also occurs after fracture of the vertebras, causing com- 
pression and hemorrhage to which Fr. Kraus has particularly called atten- 
tion (Zeitschr. f. Hin. Med., Bd. XVIII, p. 360). The compressed medullary 
substance is often greatly softened and contains numerous granular cells. 
Eeactive inflammatory changes arise in the immediate vicinity of the focus 
of compression and beyond these Tiirck's ascending and descending degen- 
eration. Schmaus quite correctly emphasizes, in his recent article upon myelitis 
(Deutsche Zeitschr. f. Nervenheilhunde, Bd. XXVI, 1904), that the pressure 
upon the spinal cord may lead, not to stasis edema, but to true, secondary 
inflammation. 

Also according to Schmaus, shock or compression of the cord, as well as 
hemorrhage, may give rise to distinct softening, to which secondarily an 
inflammation, with leukocytic and granular cell infiltration, may be added, 
entirely identical with the anatomical picture of genuine myelitis. 

From a clinical standpoint we also maintain that a sufficiently distinct 
separation between compression softening and myelitic softening cannot be 
substantiated, that, therefore, pressure 
paralyses of the cord may oe included 
with myelitis, as well as that we cannot, 
clinically or anatomically, separate hemor- 
rhagic softening from true myelitis. 

Syphilitic myelitis also presents pecu- 
liarities. It occasionally arises in connec- 
tion with syphilitic meningitis or a gum- 
matous nodule-, according to Erb, the 
apparently simple, primary degenerations 
and atrophies (sclerosis, column degener- 
ation, inflammation) may be of syphilog- 

enous Origin. Syphilitic myelitis often FlG> i 6 2.— Syphilitic Myelitis of the 

arises in Small foci, which OCCUr in COn- Lumbar Enlargement, Showing 

nection with luetic vascular disease ap- Cicatricial Atrophy and Sclerosis, 

., . ,, i -i , -II ti Right Half (Relapse) in the Stage 

pearing unilaterally or bilaterally, solitary OF SuBACIJTE i NFLAMMATION (Vesic- 

or multiple (Fig. 162). vlar Stage). 




As an example of this form, a case reported by Leyden may serve (Charity — 
Annalen, III. Jahrg. ). The course of the disease as well as the anatomical investiga- 
tion permitted the recognition of three periods of development. It was the case of 
Mrs. Emma K., set. 30, who 15 years previously had passed through a luetic infection. 
Three years ago she was attacked with acute paraplegia with thoracic girdle sensation, 
and pain in both legs which rapidly emaciated. This condition, in the next few months, 
improved to such an extent, that the patient Avas able to take long walks. A relapse 
suddenly appeared 2 years later, the left leg being completely paralyzed; after treat- 
ment for 2 months incomplete restitution occurred, which, however, became aggravated 
after a prolonged journey and was succeeded by a complete, flaccid, atrophic paralysis 
of both legs with sensory disturbances, loss of reflexes and paralysis of the sphincters 
( 2d relapse ) . The patient died 4 months later, the result of sepsis which took its 
starting point from a bed-sore. 

Corresponding to the 3 " relapses " of the clinical course, the histologic examina- 
tion of the myelitic focus, which affected the lumbar enlargement, revealed 3 stages of 
development. As the first a fibrous cicatrix at the periphery of the left lateral column 
may be regarded; in this cicatricial tissue not a trace of nerve fiber could be found, 




382 MYELITIS 

on the other hand, a completely obliterated arterial vessel thickly permeated with 
yellow pigment granulations could be seen. We are justified in regarding this process 
as the starting point of softening in consequence of an arteriitis obliterans syphilitica. 
The remaining portion of the left, completely sclerosed and atrophic half of the cord, 
corresponds to the second period of the disease, while the right half was in a condition 
of subacute inflammation (vesicular stage) and coincides with the second relapse, which 
occurred 4 months prior to death. The remainder of the spinal cord revealed ascend- 
ing and descending degeneration; the pia mater thickened over the extent of the focus, 
was rich in cells and adherent.. 

The foci which only attack one-half of the cord are also of great interest. 
Fig. 163 shows the transverse section of a syphilitic myelitis with preponderant 

destruction of the left half. Unilateral 
disease leads to the form first described 
and named after Brown-Sequard; this is 
rarely of inflammatory origin (unilateral 
myelitis), it is usually the consequence 
of a unilateral division of the cord (from 
a stab wound in the cord or the like). 

A few words must be devoted to a 
quite rare, but nevertheless remarkable 
form of myelitis, caisson paralysis. This 
appears in persons working under high 
atmospheric pressure (in building tun- 
nels and bridges), when they come at 

Fig 163. — Syphilitic Myelitis, Pre- once from the caisson filled With COlll- 

dominant Destruction (Dark) of pressed air into the open air> TJ n a e r the 

the Left Lateral and Posterior . n „ ., . ■-, -, • » 

Columns, Partial Destruction of influence of this rapid lowering of pres- 

the Right Posterior Column. sure, various disturbances arise in the 

internal organs, among which the lesions 
in the nervous system are of particular interest. Such observations have been 
reported by v. Schr otter, Mayer and Heller in their " Handbuch der Caisson- 
krankheiten." Besides cerebral paralysis, spinal paralysis of the more or less 
complete paraplegic type is noted. The sudden appearance of these paralyses 
may be referred to rupture of the cord substance in consequence of the exit of 
air from the blood vessels, with or without extravasation of blood, accordingly 
to traumatic influences; from the embolic occlusion of the air vesicles of the 
arterioles of the spinal cord, circumscribed necroses may appear. The autopsy 
reveals circumscribed foci with the typical lesions of myelitis. 

We, therefore, do not err if we include, clinically, these various forms 
of spinal cord lesions and designate them myelitis. Clinical description will 
entirely justify this procedure. The etiology will often enough present points 
of support to render a differential diagnosis of the various forms possible. 



THE CLINICAL PICTURE OF ACUTE MYELITIS 

The starting point of the clinical diagnosis of acute myelitis is always 
formed from the more or less rapid and complete development of paraplegia. 
This type has been known for a long time. First described as the result 
of injury to the vertebral column and experimentally produced in animals 



THE CLINICAL PICTURE OF ACUTE MYELITIS 383 

by division of the spinal cord, it was subsequently observed as a spontaneous 
disease. In the most developed cases there is a complete functional separation 
of the upper and lower body portions, so that the latter are entirely beyond 
control of the will and devoid of sensation. Although we adhere to this type 
of myelitic transverse paralysis there are nevertheless manifold differences; 
thus the degree of the paraplegic paralysis may vary in an equal or unequal 
implication of the lower extremity, according to the height, the transverse 
extent, and intensity of the pathologic process. The clinical picture of 
myelitis is especially modified according to the involvement of the central 
gray, and the peripheral white, substance; it further depends upon the impli- 
cation of the anterior or posterior horns, as well as of the motor and sensory 
fiber columns, upon one or both sides. For this reason symptoms may appear 
singly or in combination in myelitis, conditions which are also observed in 
other diseases of the cord, for example, spastic spinal paralysis. 

In general the inflammation attacks both sides, not rarely even the entire 
transverse section of the cord; this is easily understood from the narrow 
extent of the cord; hardly thicker than the circumference of the little finger, 
only exceptionally may one-half be affected (unilateral myelitis). For this 
reason — in contrast to cerebral monoplegia and hemiplegia — motor and sen- 
sory paraplegia combined with paralysis of the sphincters remains the type 
of spinal cord paralysis. 

Before proceeding to the special description of the clinical pictures, ac- 
cording to the region of the cord involved, it appears advisable to enumerate 
briefly the common features of transverse myelitis. 

Transvprsr myelitis in its typical form, no matter at what height it is 
situated, produces the following symptoms: 

(1) In the muscles supplied by the cord segments below the lesion there 
is, as a rule, spastic paralysis without disturbance of electric contractility, 
frequently, but not always, with increased reflexes (descending pyramidal tract 
degeneration) ; 

(2) In the muscle regions of the diseased spinal cord segments, flaccid 
paralysis with DeR and atrophy of the muscles, as well as loss of reflex con- 
tractility ; 

(3) In the area of innervation of the diseased, as well as of the entire 
spinal cord below, anesthesia of the skin and also of the deeper parts (mus- 
cles, bones, joints). To this anesthetic or hypesthetic zone there is often an 
adjoining narrow, hyperesthetic band (girdle pain, see Fig. 166) ; 

(I) Bladder and rectal disturbances; 

(5) Vasomotor (edema, cyanosis) and trophic disturbances (bed sore, 
cystitis ) . 

Transverse myelitis most often affects the dorsal cord, which represents 
the longest and simultaneously the narrowest portion of the cord; for this 
reason disease of the entire transverse section in the dorsal cord is much more 
frequent than in other portions. 

The transverse size of the section of the dorsal cord, according to Stilling, 
amounts to 28.7-36.2 mm., while in the cervical cord it varies between 44.7 
and 62.49 mm. ; in the lumber cord between 35.2 and 42.9 and in the three 
upper sacral segments from 22.4 to 8.6 mm. (measured in a child). To this 
must be added the circumstance that the dorsal cord is situated in the long- 



384 MYELITIS 

est and most exposed portion of the vertebral column. The dorsal cord ex- 
ternally corresponds to a line from the seventh cervical spinous process to the 
tenth thoracic vertebra. 

We shall, therefore, describe 

THE CLINICAL PICTURE OF DORSAL MYELITIS 

somewhat minutely and in this connection the variations in disease of other 
portions of the cord, particularly emphasizing segmental localization of the 
spinal functions. 

We may differentiate four stages in the course of myelitis : 

(1) The prodromal stage; 

(2) The stage of paralysis, in which the motor and sensory paralyses, 
ascending and descending secondary degenerations and the degeneration in 
the anterior horns take place to the fullest extent depriving the muscles and 
nerves of their nutrition (neuroparalytic stage according to Goltz). 

In connection with these is, 

(3) The regenerative stage and finally 

(4) The stage of arrest (stationary stage, sequels). 

I. THE PRODROMAL STAGE 

The signs which signalize a threatening myelitis, apart from preceding 
trauma or infection, are in part sensory, in part of motor kind. The patient 
drags the member attacked by paralysis, and has a permanent or transitory 
sensation of leaden weight, muscular tremor, of stiffness and of rapid exhaus- 
tion ; the legs temporarily refuse to function and there is a " kink " in one or 
the other knee-joint. Among the sensory symptoms there are various disturb- 
ances of sensation, formication, circumscribed anesthesia and dysthesia, fur- 
ther a tormenting girdle sensation and lightning-like pains, which appear 
particularly at night and are often combined with involuntary muscular con- 
tractions. These pains can not be referred to disease of the cord, for this 
structure, like the brain, is but little or not at all sensitive; on the contrary 
they indicate an implication of the membranes of the cord or of the nerve 
roots. Myelitis not infrequently begins with crises-like gastric pains, nausea 
and vomiting. Sometimes even in the prodromal stage of myelitis pain or 
symptoms of retention appear on the part of the bladder and rectum. 

Often fever and chills open the scene, especially in inflammation of the 
cord the result of acute infectious diseases, for example, enteric fever, influ- 
enza, gonorrhea, malaria; rise in temperature is noted especially often in 
acute inflammation of the anterior horns (poliomyelitis) and in acute dis- 
seminated myelitis. The height of the fever varies, in some cases it rises to 
104° F. ; usually, however, the rise is moderate and in a few days drops to 
normal. 

The appearance of fever and chills in the further course of myelitis indi- 
cates a complication, either of sepsis (usually originating from a bed-sore or 
from cystopyelitis), or hypostatic pneumonia or a disturbance of the func- 
tion of the bowels, more rarely a complication on the part of the cord (menin- 
gitis, abscess of the cord) . 



THE CLINICAL PICTURE OF DORSAL MYELITIS 385 

The symptoms that have been described, in varying degrees and in mani- 
fold arrangement, constitute the prodromal stage, and according to the rapid- 
ity with which myelitis develops, may be of briefer or longer duration. Some- 
times the disease appears in the midst of health; in a few hours the signs 
of paralysis attain such a grade that the patient almost collapses. Such para- 
plegias and anesthesias with sudden onset resemble in their appearance spon- 
taneous apoplexy of the cord; there may even be a combination of both proc- 
esses, a hemorrhage into a focus which up to that time has been latent. As 
a rule, however, in these forms of myelitis with an apoplectiform onset, there 
are symptoms in the history, in contrast to primary hemorrhage of the cord, 
such as have been described previously, to which but little attention had been 
given. Further the paralysis does not advance in primary hematomyelia, on 
the contrary — the pressure symptoms, the edema of the cord improve, and 
there remain, similarly as in cerebral hemorrhage, only the symptoms due 
to the anatomical destruction of the cord. 

What in this myelitis acutissima s. apoplcctica is the work of destruction 
of but a few hours, in the acute variety requires a few days, in the subacute 
form a few weeks, and in chronic myelitis an insidious development of a few 
months. This principle of development proposed by Leyden x for the 4 forms 
only refers to the onset and the rapidity of development of myelitis, however, 
not to the further course. The disease varies in regard to the rapidity of its 
onset; when the paralysis has reached its acme it is usually chronic, lasting 
for months or years, excepting those rare cases which terminate fatally during 
the early period. 

The apoplectiform development has been observed especially in central 
hemorrhagic myelitis, which preferably attacks the gray substance. Caisson 
paralysis also develops acutely; the patient coming out of coma in a para- 
plegic condition. 

In the subacute and chronic forms the signs of paralysis are sometimes not 
uniform, but are paroxysmal, presenting remissions and intermissions; the 
paralysis may even improve until a relapse brings out the entire clinical 
picture (myelite a rechutes). This paroxysmal course occurs especially in 
metasyphilitic inflammations of the cord (compare the previously described 
history). In another case of this kind under our observation the paresis 
of the left leg developed suddenly to a paraparesis, which again improved 
under mercurial treatment. After some time paralysis reappeared and 
finally led to complete paralysis of both legs and was followed by paresis of 
the left arm. 

A beginning myelitis can, therefore, not be taken too seriously and 
particularly the transitory improvements in its course are by no means a 
source of unalloyed joy. During the entire course of the malady a new attack 
may occur, especially as the spinal cord is much more sensitive than the brain 
and an intercurrent disease, or carelessness on the part of the patient, or in 
the treatment, may readily rekindle the inflammatory process. 

i Article upon myelitis, Verhandlung des Congresses fur innere Medicin in Berlin, 
XIX. Bd., 1901. 



386 MYELITIS 

2. THE STAGE OF PARALYSIS 

Dorsal myelitis produces paralysis of both legs, the muscles of the trunk 
up to the height of the pathologic focus, as well as of the bladder and the 
rectum. 

In the most marked cases the paralysis is complete and implicates all of 
the sensory and motor qualities: the legs no longer obey the impulse of the 
will, they are anesthetic, immotile and only by dysesthesia, pain and involun- 
tary contractions do they show signs of life. The sphincters are insufficient, 
cystitis and bed-sores appear, the consequences of which (pyelonephritis, sep- 
sis) are frequently sufficient to cause death. Fortunately these severe cases 
with fatal paralysis are rarer than those in which some movement is present. 

Motion is usually earlier and more severely affected than sensation. Motor 
paralysis may even be complete, while sensation is but little or not at all in- 
volved. For this incongruence of sensory and motor paralysis at the present 
time we cannot give a well-founded explanation. The paralyzed muscles do 
not obey the will, but by no means are they always incapable of stimulation ; 
on the contrary, in the majority of cases they reveal increased reflex excita- 
bility. The hypertonic muscles soon become rigid, probably a sign of a begin- 
ning secondary pyramidal degeneration; perhaps also a sign of an extra- 
pyramidal, beginning regeneration. Only those muscles remain flaccidly 
paralyzed whose anterior horns are destroyed, while the others, centralized below 
the medullary disease, may even show increased tonus, increased reflex irrita- 
bility and even spasm. The last named is sometimes spontaneous but more 
frequent after attempts at motion, for example, when the patient endeavors 
to sit up or after various reflex stimulations, stroking the sole of the foot or 
the popliteal space, from the effect of cold, shaking the bed and the like. The 
legs are spasmodically raised or drawn up with marked muscular tremor so 
that the heels approximate the buttocks, the toes being spasmodically flexed. 
In other cases the legs are alternately flexed and extended, which in children 
may jerk the limbs about (flying legs). This spasmodic condition is followed 
by painful muscular rigidity, which produces a cadaver-like rigid condition. 
The clonic spasms have been called by Brown- Sequard spinal epilepsy. It is 
the result of increased reflex irritability of the normal muscle. Contrac- 
tion of the legs indicates the seat of the lesion above the lumbar cord; if 
this latter region is also attacked or destroyed, the reflexes disappear, the 
paralyzed legs are flaccid and do not react to external stimulation. 

In the medium severe cases of inflammation of the thoracic cord, by a 
minute investigation of the power of movement, the implicated muscle groups 
may be determined and we may thus inform ourselves in regard to the height 
and transverse distribution of the lesion in the cord (see the section on 
regional diagnosis). Most frequently slight flexure movements are retained 
in the toes, ankle, knee, and hip joints; but it is most difficult actively to 
raise the extended leg from its support. Attempts at movement are slow and 
tiring, the legs appear heavy as lead to the patient and he is soon exhausted. 

In milder cases the patients are still able to walk with the support of a 
cane or even without, the legs being held stiff like stilts. Raising the tip of 
the foot is difficult, so that a drawing of the trail of the foot shows an almost 
continuous but irregular path. This spastic gait renders the ascent of stairs 



THE CLINICAL PICTURE OF DORSAL MYELITIS 387 

very difficult as well as jumping and rapid walking ; tremor soon appears, the 
patient is quickly exhausted (myasthenic symptoms), he stumbles and threat- 
ens to fall ; sometimes one or the other leg fails and the patient actually falls. 
The sensory symptoms in these milder cases are actually slight but on the 
other hand there are frequently pains in the distribution of the sciatic nerve. 
The bladder is almost always affected. 

A common outcome of spastic paralysis is in contracture, which in contrast 
to hemiplegic extensor contracture may also induce a flexure position. The 
hip and knee joints may be flexed in such a manner that the heels approxi- 
mate the buttocks; the knee joints are sometimes contracted in a position 
of adduction so that they bore into each other sufficiently to produce pressure 
ulcers and can only be held apart by producing active pain. The feet in con- 
sequence of contraction of the gastrocnemii assume a pes equinus position ; the 
contracted muscles finally stand out like fibrous bands so that relief of the con- 
tracture is no longer possible by manual redressment but only by operative 
means. 

Atropliy attacks the over-extended antagonists to a greater extent than the 
contracted muscles ; according to the extent of the anterior horn lesion it may 
at one time attack this, at another time that, muscle to a greater extent, 
so that various deformities and contractures may develop. In the paralyses 
which are flaccid from the onset, from external mechanical influences paralytic 
contractures may result, for example, from pressure of the bed clothes upon 
the dorsum of the foot pes equinus may develop. 

In dorsal myelitis, besides the muscles of the legs those of the trunk and 
of the abdominal wall may be involved. Faralvsis of the intercostal muscles 
renders respiration difficult and occasionally leads to dyspnea; usually, how- 
ever, the vicarious function of the diaphragm and the auxiliary muscles of 
respiration compensate for this defect. 

A lesion of the 5th-12th dorsal segments paralyzes the abdominal mus- 
cles. Paralysis of abdominal pressure renders expiration and expectoration 
difficult, as well as contraction of the abdomen. Bronchitis, hypostatic pneu- 
monia, hindrance of the act of vomiting, meteorism, constipation and the 
retention of urine are the consequences. \Yagner and Stolper report a case, 
in which the muscles of the abdomen, in consequence of a partial injury of 
the lumbar cord were only paralyzed on the right side and were flaccidly 
protruded. Stolper even observed ileus-like symptoms after spinal cord hem- 
orrhage. Paresis of the abdominal muscles may be noted from the inability 
of the patient to rise from the recumbent position unaided. The rectus ab- 
dominis may present segmentary paralysis; Kocher reports the case of a 
paraplegic, following dislocation and fracture of the 11th and 12th thoracic 
vertebra?, in whom the rectus abdominis did not contract below the umbilicus 
but responded well above this point. 

When the inflammation attacks the upper thoracic cord from the second 
thoracic vertebra upward, then to the paralyses of the trunk and legs there 
are also added pareses of the flexors of the fingers, of the thumbs and the 
ball of the little finger, and of the interossei, as well as of the pronator quad- 
ratus. In disease of the first dorsal segment there are also oculopupillary 
symptoms (see cervical myelitis). 

The behavior of the paralyzed muscles to the electric current is of great 



388 MYELITIS 

diagnostic, prognostic and therapeutic import. Electricity in a certain respect 
is a test for the condition of the cord and of the muscles. A permanent, nor- 
mal, electric reaction indicates that profound damage of the muscles and 
nerves, that is of the anterior horns which innervate them, is extremely un- 
likely. In general, therefore, with the lesion high, the electric contractility 
of the lower members is retained. Only in lesions of the lumbar cord and of 
the cauda equina, may electric alterations appear, and especially after destruc- 
tion of the gray anterior horns. The appearance of DeR indicates a profound 
destructive lesion, with degeneration of the muscle and nerves, as may be 
readily determined macroscopically. 

As is well known complete DeR consist in the loss of electric response of the 
nerve and of faradic contractility of the muscle; contraction of the muscle 
to galvanism is all that is retained, and at first may even be increased. This 
increase of galvanic irritability is often accompanied by an increase of mechan- 
ical irritability; it is not to be confounded with the increase, which is regarded 
as a sign of general heightened irritability occurring with both currents in 
spastic, cerebral, and spinal paralyses. Besides, an alteration in the form of 
contraction and in the law of contraction is characteristic of the DeR; the 
first is sluggish, crawling, the latter shows an increase of the AnCC over 
the CaCC. 

DeR may take place during the first weeks of myelitis and points with 
great likelihood to the arrest of the trophic influence of the anterior horn cells 
as well as to a severe course for the muscular paralysis. In favorable cases 
the increase of galvanic irritability gradually decreases, the formula of con- 
traction again becomes normal and faradic contractility reappears. In the 
unfavorable cases the contractions remain sluggish and the electric formula 
transposed; galvanic contractility may gradually decrease until in the termi- 
nal stage of atrophy, neither from nerve nor from muscle, by the strongest 
faradic or galvanic currents, can a contraction be obtained. It is self-evident 
that complete DeR in myelitic paralysis only occurs in those muscle bundles 
the related anterior horns of which are destroyed. If a portion of the anterior 
horn is unaffected, the muscle fibers pertaining to it are retained, so that a 
muscle in one fiber bundle may show normal electric reaction and in another 
DeR, as may be the case also in spinal progressive muscular atrophy (Du- 
chenne-Aran type). 

Simple, quantitative decrease of electric contractility with a normal elec- 
tric formula is less unfavorable; this condition is particularly observed in 
inactivity atrophy, as in the late stage of those paraplegias, in which the lesion 
is above the leg center, and also above the lumbar cord. 

The disturbances in the sensory field are even more varied than those of 
the motor. In the paralyzed lower extremities, sensation (conduction of sen- 
sory stimulation from consciousness) may be more or less reduced; and in 
all areas of distribution, upon one or both sides or even only in one part 
of a lower extremity, as upon the posterior surface of the legs or the sexual 
organs and the perineum, etc., these conditions may prevail. The individual 
sensory qualities (pain, cold, heat, vibration, and localization) may be in- 
volved to a varying extent. If the inflammation is limited to the central gray 
of the cord, pain is mostly absent, as the spinal cord substance in itself is 
insensible. Only when inflammation or edematous swelling is distributed to 



THE CLINICAL PICTURE OF DORSAL MYELITIS 389 

the spinal membranes which are richly supplied with sensory nerves or to the 
posterior roots do pains appear. For this reason, in uncomplicated myelitis, 
in contrast to meningitis, the vertebral column is not held stiff nor is pain 
present upon movement or pressure. 

Among the sensory irritative symptoms different forms of paresthesia 
appear in the paralyzed limbs. Some patients complain of numbness, tingling, 
furry sensation in the toes, others of tormenting heat and cold, burning, 
itching, of spasmodic sensations in the bladder and rectum. In the region 
of the spinal nerve roots sensations of this kind are experienced as girdle 
pains or as a painful constriction, which may even lead to respiratory difficulty. 
This girdle sensation may often be referred to an irritation of the nerve roots 
at the upper boundary of the lesion and then indicates its height. Externally 
this girdle sensation often shows the boundary of the anesthetic and normal 
cutaneous zone (see Fig. 173). Sometimes pains are early symptoms of 
myelitis, especially of syphilitic perimyelitis and meningomyelitis. The pains 
are aggravated at night and are sometimes unbearable; thus many a myelitic 
becomes a morphin habitue. The pain is especially severe in the legs, it is 
experienced as " shooting " sciatica, or as a feeling of constriction around the 
knee and ankle joints. 

The anatomical cause of these pains may be sought in an irritation of the 
posterior nerve roots, either by an encroachment of the inflammation, by 
pressure of the tissue swelling, by the edema or inflammatory exudate, or from 
adhesion of the spinal membranes to the cord. This pressure is most decided 
upon the nerve roots in the case of disease of the vertebrae; the pain being 
most severe in carcinoma of the vertebras {paraplegia dolorosa) ; they are not 
so intense in spondylosis rhizom clique, less, even absent, at least during rest, 
in spond}ditis tuberculosa. 

This pain may be produced or increased by pressure, palpation over the 
vertebrae, by bending forward or by jumping. Occasionally the localized ver- 
tebral pain, which is momentarily absent, may be provoked by passing a hot 
sponge over the region or by the contact of a vibrating tuning fork. Accord- 
ing to an ingenious observation of W. Gull's the severe neuralgic pains, in 
cancer of the vertebra, are almost always experienced close to the vertebral 
column, while in caries of the vertebra they are situated laterally in the wall 
of the thorax. Here the patient notes a painful burning or tearing or a com- 
pressing girdle sensation about the thorax. In utilizing these sensations of 
pain, in a diagnosis of the height of the lesion, it must be remembered that 
the nerve roots which pass out of the cord, in the thoracic and lumbar regions, 
run obliquely from above downward. The focus in the cord, therefore, is 
always higher than the point of exit of the nerve roots which belong to it in 
the canal of the vertebral column. 

In myelitis the internal organs may also give rise to pain; thus in dorsal 
myelitis there are attacks of angina pectoris and gastric crises. It is quite 
possible that the internal organs, in diseases of the spinal cord segments that 
supply them, react by sensory disturbances in a similar manner to the skin. 
Both are linked with nerve tracts, which was made clear by Head's investi- 
gations upon the hyperalgesic zones of irradiation of the skin in disease of the 
internal organs. Head refers the spinal innervation of the heart to the 1-3 
thoracic segment, of the lung to the 1-5 thoracic segment, of the stomach 



390 MYELITIS 

from the 6-9 thoracic segment. According to Kocher 1-4 dorsal segments 
send sympathetic nerves to the head and neck as well as to the heart and 
lungs; the 5-9 dorsal segments to the glands of the intestines and abdomen 
(splanchnic nerves), and the 10-12 dorsal segments to the testicles, the blad- 
der and the rectum (inferior splanchnic nerves; internal spermatic plexus; 
inferior mesenteric plexus). These reports of the cord innervation of the 
heart, the stomach and the intestines, of the spinal course and the location 
of the conduction tracts for the intestine, require further investigation. 
Probably spinal vasomotor innervation plays an essential part in these intes- 
tinal reflex processes. 

In addition to these attacks of pain there are also hyperesthesia, hyper- 
algesia and spontaneous muscle contractions in the paralyzed members. This 
may go so far that even slight contact or pressure may produce painful formi- 
cation (Charcot's dysesthesia), and even contractions. The bones and muscles 
are often hyperesthetic. This hypersensitiveness, in contrast to the neuritic 
pains, is not in connection with the pressure points of the peripheral nerve 
trunks, but with the segment zones. 

The combination of these spontaneous pains with objective anesthesia in 
the region of the paralyzed limbs is peculiar. This condition known as anes- 
thesia dolorosa is referred to a process of irradiation due to a central irritation 
of the posterior roots, which have simultaneously become incapable of conduc- 
tion for peripheral irritations. This may be due to compression of the poste- 
rior roots from masses of exudate or contracting cicatricial bands; the symp- 
tom-picture of paraplegia dolorosa is especially common in compression of the 
spinal cord due to carcinoma of the vertebra (Charcot, v. Leyden). 

The appearance of sensory paralysis indicates a profound destruction of 
the cord. The extent of the sensory disturbance is of the greatest importance 
in regard to the height of the lesion in a dorsal myelitic focus because motor 
paralysis in all diseases of the dorsal cord, from the third segment downward, 
presents very nearly the same picture of paraplegia. In dorsal myelitis anes- 
thesia may extend from the tip of the toes to the clavicles, varying in height, 
according to the location of the focus (compare Seiffer's diagram of sensory 
topography). 

In disease of the lowest dorsal cord segment the anesthesia is limited to 
the legs, anteriorly it reaches to Poupart's ligaments, posteriorly to the height 
of the 5th lumbar vertebra. With each higher segment that is diseased the 
border of anesthesia rises. In disease of the 9th and 10th dorsal segments the 
anesthesia reaches to the umbilicus, of the 8th and 1th dorsal segments to the 
line of the xyphoid, of the J±th, 5th and 6th dorsal segments to the breasts 
and in disease of the 3d dorsal segment to the clavicles. In an affection of the 
upper thoracic segments the anesthesia reaches posteriorly to the first thoracic 
vertebra: a hypesthetic line reaches to the axilla and to the ulnar inner sur- 
face of the arm including the ulnar half of the forearm and the second to the 
fourth finger, similar to the condition found in ulnar nerve paralysis. 

As the dorsal segments are situated higher than the vertebrae which corre- 
spond to them, and as besides a completely anesthetic zone always points to 
an implication of adjacent segments, the focus in the cord is always to be 
sought for higher than would correspond to the actual anesthetic zone. Thus 
in total anesthesia of the mammae (border limit of the 5th dorsal segment) 



THE CLINICAL PICTURE OF DORSAL MYELITIS 391 

the medullar}- lesion is to be sought at the height of the 4th thoracic vertebra 
or even of the next higher vertebra. 

The lower boundary of the lesion is very difficult to estimate, as the motor 
and sensory paralysis implicates the entire body below the focus. Only the 
retention of the reflex arc below the point of lesion, as well as the behavior 
of the paralyzed muscles and nerves upon electric testing, and the condition 
of the sphincters furnish points for the recognition of the lower boundary of 
the lesion. 

Often enough the extent of the sensory disturbance does not correspond 
with the motor paralysis, because the inflammation may at one time attack 
the sensory, at another time the motor, portions in the cord more intensely. 
The height of the focus is naturally determined by the highest segment that 
is diseased. If the myelitic focus is oblique, or if the disease is more marked 
on one side of the cord, then also the leg upon the side of the more severely 
involved cord lesion shows the paralysis to a greater extent. In inflammation 
limited to only one-half of the cord Brown- Sequard's paralysis will be pro- 
duced; this occurs more frequently in unilateral spinal cord injuries, for 
example from a stab wound or traumatic dislocation of the vertebrae. Motor 
paralysis (spastic paresis with increase of the tendon reflexes, arrest of the 
muscle sense, the tract running in the posterior column of the same side) is 
situated upon the side of the injury, while sensory paralysis (analgesia) affects 
the opposite side, as the sensory tracts first cross in the spinal cord. 

These peculiar sensory disturbances are shown by a case under our observa- 
tion. It was one of Brown- Sequard's paralysis that had existed for 25 years; 
remarkable besides the prolonged duration, for tormenting hyperesthesia and 
a spastic contracture of the lumbar muscles upon the paralyzed side, as well 
as for the favorable outcome (Figs. 164 and Hi.")). 

The case (I) was that of a carpenter, Paul H., aet. 50, who 25 years previously 
had suffered from a stab wound in the thoracic vertebral column. Immediately after 
this injury there was paralysis of the right and analgesia of the left leg. The wound 
healed rapidly. Patient was in the hospital for 7 months and was finally able to walk" 
with the aid of a cane. Functions of the bladder and rectum were intact. Upon the 
paralyzed side there was often severe hyperesthesia and paresthesia (burning, numb- 
ness, formication). To this were added pain and stiffening in the hip, knee, and ankle 
joints upon the right side ; occasionally, for several seconds, there were painful spas- 
modic extensions of the right leg, in consequence of which the patient suffered for years 
from insomnia. Upon the 8th of June, 1904, he was admitted to the Charite. 

In this powerfully built patient, there Mas a cicatrix 3 cm. in length, somewhat to 
the right of the median line (Fig. 105). between the spinous processes of the 2nd and 
3rd thoracic vertebrae. The vertebral column was nowhere sensitive to pressure. There 
was spastic paresis of the right leg with slight atrophy (inactivity). The circum- 
ference of the right thigh was 46.5 cm. and 39 cm., on the left 47.5 cm. and 41 cm. 
The muscles of the calf were equal upon both sides (circumference 33 cm., each). The 
patient, like cerebral hemiplegies, dragged the right leg scraping the floor with the tip 
of the foot in a semicircle. The flexor muscles were more markedly paralyzed than the 
extensors. In the lumbar muscles and those of the leg on the right side, there were 
spontaneous contractions. The patient limped — in consequence of contracture of the 
right sided lumbar muscles — as the right half of the pelvis was higher than the left; 
the right leg, therefore, appeared shorter and the right malleolus tibce was 2 cm. higher 
than the left. The length of the legs, measured from the iliac spine to the ankle was 
equal upon both sides. 

Pupillary reflexes normal. Patellar and Achilles tendon reflexes were greatly in- 
creased upon the right side, normal upon the left. Babinski's reflex was pathologic 




Fig. 164. — Arrangement in Brown-Sequard's Paralysis in a Case op Stab Wound to 
the Right Half of the Spinal Cord (between the Second and Third Thoracic 
Spinous Processes) in Seiffer's Diagram. 

C, cervical, D, dorsal, L, lumbar, S, sacral, segment. Upon the side of the injury (right) 
there was spastic paralysis with hyperalgesia (transverse shading) and diminution of sweat 
secretion, upon the left side there was analgesia (longitudinal shading) and thermo-anes- 
thesia (crosses). The sensations of contact and position were intact. 
392 




Fig. 165. — Posterior View of Fig. 164. 
(For caption see page opposite.) 



393 



394 MYELITIS 

upon the right, normal upon the left, side. Cremaster reflex normal upon both sides. 
Abdominal reflexes absent upon both sides. 

Electric contractility in the muscles and nerves of the right leg was increased. 
Secretion of sweat in the right leg was almost arrested. The sensory conditions may 
be seen from the diagram (see Figs. 164 and 165). The sciatic points upon the right 
side were extremely painful. 

The treatment consisted in methodic exercise, bath-gymnastics, and carbonated baths, 
vibration massage, inunctions of iodin-vasogen, application of menthol salves and the 
administration of veronal and trigemin. The pain and paresthesia in the right leg 
almost disappeared, and walking improved to the extent that the patient was able to 
ascend stairs unaided. He was discharged from the hospital July 16th, greatly im- 
proved and was able to follow his occupation. 

Symptoms on the part of the bladder and rectum may be entirely absent 
in unilateral myelitis, as the corresponding tracts run bilaterally (according 
to Kocher in both cervico-dorsal-lumbar tracts) ; these bilateral tracts act 
synergistically and may function vicariously for one another. The appearance 
of bladder and rectal symptoms, therefore, renders a bilateral spinal cord 
disease likely. 

Anesthesia, in a complete transverse interruption, involves all qualities 
including deep sensation (position, muscle and locality senses). In a partial 
disease of the cord the individual qualities of sensation (tactile, pain, tem- 
perature, vibration sensations) may show all forms of transition, from an 
absolute to slight loss, in all variations of distribution; sometimes a slowing 
of conduction is noted. A case reported by J. Schnitzer is remarkable. It 
was one of spontaneous delivery in a woman suffering from compression 
myelitis (dorsal cord) ; there was anesthesia extending 3 cm. above the xiphoid 
line. Labor was rapid, without pain and without the aid of the abdominal 
muscles. Partial sensory disturbances are more marked as a rule toward the 
periphery, i. e., toward the toes and less so centrally, but even here there are 
exceptions to the rule. 

The transverse topography of the focus may be inferred from the nature 
of the sensory disturbance; thus a loss of pain and temperature senses, with 
retained contact sense, point to disease of the posterior horn adjacent to the 
central canal. In other cases the temperature sense may be alone involved or 
only deep sensation may be affected, with retention of the other sensory quali- 
ties. From these clinical facts we may conclude that every sensory quality 
has special conduction tracts. 

The condition of the reflexes varies according to the localization, intensity 
and duration of the disease ; sometimes the reflexes are normal, at other times 
increased to clonus, sometimes abolished. Normal reflexes permit us to con- 
clude that their reflex arcs in the gray substance are normal. Lesions outside 
of the reflex arcs, for example in the anterior columns, may be present without 
influencing the reflexes. Hence, absence of reflexes indicates a lesion of the 
reflex arc, such as may result from disease of the posterior roots (anesthesia), 
or of the anterior roots (motor paralysis), that is of the gray substance or of 
Burdach's tract. Exaggerated reflexes are due either to greater irritability 
of the gray substance, or to interruption of the pyramidal tract. 

The law that the reflexes are abolished in the innervation area of the 
diseased section of the cord, being retained above and below in consequence 
of the interruption of the cerebral reflex inhibition and increased below, is 



THE CLINICAL PICTURE OF DORSAL MYELITIS 395 

true of some, but by no means of all, cases of myelitis. Thus in dorsal 
myelitis, the patellar and Achilles tendon reflexes are usually active (their 
center being in the lumbar and sacral cord), often being even increased to a 
clonus, sometimes even to the extent that slight peripheral irritation, such as 
slight contact with or cooling of the legs, produces convulsive muscular con- 
tractions. The increase of reflexes may persist for years, even after restitu- 
tion of motion, as the last sign of recovery from a myelitis. Exaggerated knee 
jerk, naturally, may not be present, when the inflammatory process has involved 
the lumbar enlargement. On the other hand, in a high complete transverse 
separation of the cervical cord or of the upper dorsal cord, a loss of the patellar 
reflex has been observed, in spite of the anatomical integrity of the lumbar 
reflex arc. According to Kocher, the absence of the tendon reflexes in the 
paralytic area especially characterizes complete transverse lesion of the cord, 
while in unilateral or partial cord lesions the reflexes are retained and com- 
monly exaggerated. 

Various hypotheses have been proposed to explain this peculiarity of the 
reflexes; Bastian, Bruns, van Gehuchten believe that the loss of the reflexes 
is due to the absence of the muscle tonus tracts which originate from the cere- 
bellum. According to Hoche, perhaps the long, descending posterior column 
tracts are related to the abolishment of the patellar reflex. Other authors 
(Gerhardt, Sternberg) believe that there is a reflex inhibition due to irrita- 
tion originating from the point of lesion passing to the reflex centers. The 
early absence of the tendon reflexs at the onset of myelitis is regarded as 
a sign of shock, that is, an irradiation of the central irritation. Egger be- 
lieves in a functional paralysis of the gray substance, and Bischoff in a dam- 
age of the latter, as the result of vasomotor and consecutive circulatory dis- 
turbances. Xone of these views have as yet been supported by unquestioned 
anatomical findings. In many cases the total loss of reflexes (including those 
of the skin, tendons, vasomotor apparatus, bladder and rectum) in high, 
complete transverse lesions of the cord, must be regarded not only as the 
sign of a sudden action, an irradiation of the central irritation, but as the 
result of a disease descending in the gray substance to the lumbar cord. Thus, 
v. Leyden in 2 cases of tumor compression of the cervical cord, which ran 
their course clinically with flaccid paralysis and absent reflexes, found lesions 
in the anterior horn cells, extending down into the lumbar cord (Archiv 
fur Psychiatrie, 1878, Bd. VIII, and Berlin, klin. Wochen., 1878). 

In regard to the skin reflexes the theories also are far apart. The inter- 
ruption of the pyramidal tract, for example after hemorrhage of the internal 
capsule, increases the tendon reflexes, while the cutaneous reflexes are dimin- 
ished or abolished. In disease of the pyramidal lateral column, the cutaneous 
and tendon reflexes are usually increased. In diffuse inflammation of the 
cord, according to the implication of the gray or white substance, the condi- 
tion of the cutaneous and tendon reflexes will vary. Thus the skin reflexes 
disappear in cutaneous anesthesia, while the tendon reflexes may be increased. 
On the other hand in disease of Burdach's columns, the tendon reflexes may 
be abolished and the skin reflexes retained, as the tracts of the latter radiate 
directly into the posterior horns and thence into the anterior horns. 

To this must be added the physiologic differences in regard to number 
and strength of the cutaneous reflexes in the normal person. The pathologic 



396 MYELITIS 

reaction of Babinskr's toe reflex, i. e., sluggish primary extension of the large 
toe upon stroking the ball or the external border of the foot, is not absolutely 
significant of a pyramidal degeneration. Babinskr's reflex also occurs in other 
diseases of the spinal cord, in healthy nurslings, in sleep, and exceptionally 
even in some functional diseases of the nervous system (hysteria). From 
the absence of the skin reflexes, therefore, we can not draw such definite 
diagnostic conclusions in regard to the nature and seat of the pathologic focus, 
as from the loss of the tendon reflexes. Only rarely may we obtain an idea 
of the location of the cord lesion from the segmentary testing of the skin 
reflexes, for example, when Babinskr's and the cremaster reflexes are retained, 
while the abdominal reflexes are absent; a condition that may occur in a 
circumscribed myelitis of the lower dorsal cord. Occasionally the skin re- 
flexes in dorsal myelitis may be increased to such an extent that even slight 
cutaneous stimulation, such as the pressure of the bed clothes, gives rise to 
contractions, especially in the flexors of the knee joint and in the extensors 
of the great toe. 

Among the most important and at the same time most serious sequels of 
myelitis are the symptoms on the part of the bladder and rectum. The blad- 
der disturbance is often an early symptom of myelitis as well as of other cord 
diseases, especially those involving the posterior columns. At the onset there 
are usually spasmodic symptoms, tenesmus (spastic contraction of the de- 
trusor) retentio vesica? (sometimes also am). In the further course incon- 
tinence occurs. The retention may be spastic as the result of sphincter spasm 
or paralytic the consequence of paralysis of the detrusor vesicae. In this con- 
dition, especially if there be anesthesia of the mucous membrane, the bladder 
may be distended to the umbilicus and even beyond, until the over-distended 
sphincter vesicae relaxes (Landois) and dribbling of urine occurs (inconti- 
nentia paradoxa). In some cases from reflex irritation, for example, upon 
attempts at movement a discharge of urine, a spasm of the bladder takes 
place, sometimes also an erection of the penis. 

When the sphincter center in the sacral cord is affected, sphincter paral- 
ysis takes place, and may be recognized from the constant paralytic incon- 
tinence. If the lesion is above the sphincter center, strangury is often unnoted 
and the discharge of urine occurs involuntarily, but at long intervals and 
in a full stream. With an anesthetic mucous membrane of the bladder pro- 
longed retention of urine occurs, which, in consequence of the pressure, may 
give rise to circulatory disturbances in the wall of the bladder with subse- 
quent hemorrhage, necrosis and ulceration, especially in the vertex of the organ. 
Finally, especially at the acme of the disease, paralysis of the sphincters and 
of the detrusor, with permanent incontinence of urine, appears. 

Paralysis of the sphincter is of the greatest importance in diagnosis, prog- 
nosis and treatment of myelitis. In the diagnosis, paralysis of the sphincter 
not infrequently is decisive in differentiating neuritic and toxic paraplegias 
from myelitis. These maladies were formerly included with spinal paralysis 
or were regarded as " reflex paralysis." In neurotic paralysis, in contrast 
to disease of the cord, only exceptionally do bladder and rectal symptoms 
appear. 

The second point of importance in sphincter paralysis is in prognosis, as 
incontinence of urine and feces is a direct danger to the life of the patient. 



THE CLINICAL PICTURE OF DORSAL MYELITIS 397 

Evacuation of the bladder in spite of the constant dribbling of urine is not 
complete. In the stagnating contents of the bladder, partly from the urethra, 
especially in the female, partly from the lymph channels, by way of the rec- 
tum, microbes (bacterium coli) enter. Besides the normal bladder contains 
numerous germs, which in the healthy are evacuated without damage; in 
paralysis of the bladder, however, they are retained in the residual urine and 
produce cystitis. According to Charcot, catarrh of the bladder is analogous 
to the bed-sore and is to be regarded as a trophic disturbance, directly de- 
pendent on the spinal lesion. As a matter of fact cystitis may appear with- 
out preceding catheterization. Cystitis is diagnosticated from the presence 
of fever, chills, and the change in the urine. The further consequences are 
ammoniacal fermentation of the urine, precipitates of calcium phosphate, 
putrid, even ulcerative and necrotic catarrh of the mucous membrane, ascent 
of the inflammation to the ureters, to the pelvis of the kidney, and to the 
kidney itself, as well as a distribution to the perivesical tissues (pericystitis, 
paravesical phlegmons). Pyelonephritis is a common cause of death in 
myelitis; such patients become debilitated by fever, chills, metastatic abscesses, 
septic diarrhea, anorexia and insufficient ingestion of food, until finally col- 
lapse or uremia hastens the fatal issue. 

Another, quite as serious complication is due to disturbance in the dis- 
charge of the feces; this is due partly to weakness of abdominal pressure, 
partly to spastic or paralytic conditions of the sphincter ani. In the begin- 
ning the irritative symptoms are most prominent: tenesmus ani and retentio 
alri; tympanitis, in consequence of retention of intestinal gases and obstinate 
constipation, appears and may even continue for weeks. This condition be- 
comes even more disagreeable and dangerous from paralysis of the sphincter, 
for the patient often loses the sensation of tenesmus as well as of the passage 
of feces. The patient lies in the urine and feces which have been involun- 
tarily voided and in spite of the greatest care in nursing, in a brief time, 
ulcers form ad nates (bed sores), the sequels of which (erysipelas, sepsis) 
cause the death of many a myelitic. 

The male potency, the power of erection as well as of ejaculation, may 
sometimes be retained in dorsal myelitis. More often, however, this is lost; 
occasionally there is spermatorrhea without libido and without erection. More 
rarely there is a reflex erection of the penis or priapism. Sometimes there 
is anesthesia of the penis and testicles; the latter organs are innervated by 
the upper lumbar segments and, therefore, in isolated disease of the sacral 
cord, remain sensitive to pressure in the anesthetic scrotal skin (innervation 
area of the III sacral segment). 

Menstruation may be retained for a long time. In the terminal stages of 
myelitis it commonly ceases in consequence of the debility of the entire 
organism. Myelitis does not hinder the power of conception; but pregnancy 
usually aggravates the malady. 

Vasomotor and secretory anomalies also appear in the paralyzed members. 
The temperature of the skin may be raised at the onset of myelitis and in 
the further course may drop 2° F. below the normal. The limbs are cool and 
cyanotic. Very often edema is observed in them, which Vulpian refers to 
the decreased nutrition of the walls of the vessels. Edema of the legs may 
be the result of a sluggish circulation and of absence of motion. Thromboses 



398 MYELITIS 

have been repeatedly noted in paraplegics, in the femoral vein or its branches. 
Arterial thromboses in the paralyzed limbs are rare; these only develop in 
endarteritis that is, sclerotic processes. 

Arrest as well as increase of the secretion of sweat has been observed in 
the paralyzed areas as anomalies of secretion. Sometimes while the body is 
in perspiration, the paralyzed members do not sweat; in other cases the latter 
are bathed in perspiration and the remaining portion of the body is quite dry. 
The cause of this disturbance has not yet been made clear. 

Among the usual accompaniments of myelitis are trophic disturbances in 
the skin, the joints and the bones. In the skin there are often pemphigus- 
like or herpetic vesicles, bluish-black infiltrates and even necrotic inflamma- 
tions. These appear most readily in regions that have suffered from slight 
mechanical injury. We must, therefore, guard against the most insignificant 
lesions, for example, pressure of the bed clothes, prick of a pin, etc., for, 
on account of the loss of sensation in the skin, these are not noted and, there- 
fore, easily become infected and even gangrenous. Further, eruptions of 
various kinds are noted, urticaria, not infrequently petechia, phlegmons, ex- 
travasations of blood and eczema; but we cannot enter upon a description 
of these at this point. Trophic disturbances are further revealed in the fall- 
ing out of the hair, in desquamation of the epidermis and of the nails. 

The acute bed-sore (decubitus) is of especially serious import. It was 
described by Samuel (Kbnigsberg) and mentioned by Charcot in a masterful 
description, as the characteristic of the malignant form of myelitis. Charcot, 
and following him numerous other authors, recently also Dejerine, regard 
acute bed-sore as the result of a trophic disturbance of the cutaneous nervous 
system. There is much in favor of this view. In many other spinal diseases 
as in syringomyelia, characteristic trophic disturbances appear. Besides 
decubitus often appears in myelitis quite acutely, in the first few days, in 
fact very much more rapidly than in patients bed-ridden from some other 
cause, even in comatose persons. Thus we saw a case in a woman suffering 
from dorsal myelitis, in whom during the first week bed-sores appeared in 
various areas of the lower extremity; first on the heel, then upon the ankle; 
from the pressure of the bed clothes, then upon the trochanters and the 
sacrum. First there was a reddish discoloration of the skin which in a few 
hours became a blackish-blue. Soon the epidermis was raised in vesicles, 
which after desquamation gave place to ulcers, rapidly gaining in extent. 
Besides the areas that have been named the iliac fossae, the tuber ischii and 
the internal condyles of the femur are often the seats of bed-sores. 

Without treatment the bed-sore rapidly increases in extent and depth; it 
finally undermines the soft parts and attacks the periosteum until the bare, 
necrotic bone can be seen in the floor of the ulcer. The sacral bed-sore may 
attack the retrorectal tissue and give rise to an exceedingly painful, purulent 
periproctitis. 

An early, systematic and careful prophylaxis, that is, the treatment of the 
beginning bed-sore is one of the chief objects in the therapy of myelitis and 
here the importance of paralysis of the sphincters and of bed-sores must occupy 
the main attention of the physician ; this greatly influences the prognosis and 
very often even the question of life may depend upon this ; bed-sores and cysto- 
pyelitis are the most frequent causes of a fatal termination in myelitis. 



THE CLINICAL PICTURE OF DORSAL MYELITIS 399 

Trophic changes also occur in the bones and joints (osteoporosis, arthrop- 
athies), but with less frequency than in tabes, in which the osseous anes- 
thesia with retained muscle power favors the development of arthritic de- 
formities; while in myelitis early motor paralysis compels the patient to 
remain in bed. 

In connection with this description of the symptomatology of myelitis, 
we shall report a case of traumatic hematomydia, that is myelitis, which in 
many respects is quite remarkable (Case II). 

TRAUMATIC HEMATOMYELIA 

Man, act. 35, fracture of the XI thoracic vertebra, complete motor and 
sensory paraplegia with loss of reflexes and incontinence of the sphincters. 
Death after 15 months. Destruction of the cord from the 9th thoracic seg- 
ment downward without demonstrable compression. Tubular posterior col- 
umnar hemorrhage. 

A sailor, Kr., aged 35, suffered from an accident on October 27, 1896; he fell back- 
ward over a mast, while a falling spar struck him on the abdomen. Immediately after 
this accident his legs were paralyzed and without sensation, there were besides severe 
pains in the back and side, as well as incontinence of urine. Examination revealed 
total, flaccid paraplegia with complete loss of sensation from the soles of the feet to 
the umbilicus', above this region there was a narrow hvperesthetic zone (Fig. 16(5). 
Posteriorly the loss of sensation reached the upper border of the pelvis. The tendon 
reflexes in the legs as well as the abdominal reflexes were absent. There was incon- 
tinence of urine and feces. In the vertebral column, in spite of repeated examination 
even by a surgeon, no curvature nor dislocation of the spinous processes was found 
There was slight sensitiveness to pressure of the 11th and 12th dorsal vertebrae. Lum- 
bar puncture performed early after the trauma, produced 20 c.c. of a clear, watery 
fluid with but slight pressure. 

The paralysis and the sensory disturbances remained stationary during the follow- 
ing 15 months. Rapid atrophy occurred in the muscles of the legs (see Fig. 166), 
they lost their faradic contractility. All therapeutic attempts (extension, corset treat- 
ment, careful nursing and nourishment ) were futile. Soon putrid cystitis, gangrenous 
pressure ulcers, bronchitis and apathy appeared ; finally the patient refused nourish- 
ment and died upon January 7, 1898, 15 months after the injury. The necropsy 
revealed, besides general macies, a purulent inflammation of the bladder and kidneys, 
amyloid degeneration of the spleen and liver, a few bronchopneumonic foci in the left 
lung and a pelvic phlegmon. 

Xo abnormality was visible externally on the vertebral column at the autopsy. 
Only after sawing through the vertebral column in a median direction was a cuneiform 
deformity noted in the 11th thoracic vertebra, so that anteriorly it was 12 mm. and 
posteriorly 20 mm. in height. The spinous and transverse process showed no deviation, 
only the 11th thoracic vertebra protruded 2 mm. into the vertebral canal. Nowhere 
was there compression of the cord; at the height of the 8th and 9th thoracic vertebrae 
the cord was quite flat, contracted to a ribbon. The pia mater showed no changes; 
nowhere were there traces of hemorrhage. After hardening in formalin and Miiller's 
fluid, there were shown in transverse sections through the cord, that from the 8th dorsal 
segment downward to the conns medullaris the structure had disappeared (Fig. 167 f, 
g, and h ) . This portion of the cord was contracted, and stained a uniform light yellow. 
In the higher sections (Fig. 167 a-e) up into the cervical cord the usual picture of 
ascending degeneration in the posterior columns as well as in the periphery of the 
lateral columns was recognized and could be noted by the paler stain. Besides a focus 
of softening about the size of a millet seed (Fig. 167 e) was found from the 8th dorsal 
segment, extending downward in the left posterior column near the commissure being 
distributed to the dorsal cord, and at the 4th dorsal segment widening into a cavity 



400 



MYELITIS 



(167 d) thence extending over the middle of both posterior columns and filled with 
yellowish masses. This tubular focus terminated in the lower cervical cord. 

Leyden, upon histologic examination (according to Nissl, Marchi, Weigert, etc.), 
was unable to find nerve fibers or ganglion cells in the lower dorsal, lumbar or sacral 
cord; the tissue in the entire transverse section consisted of numerous granular cells, 




proliferated glia meshes, myelin granules and a few hematoidin crystals. Only in a 
few longitudinal sections were there scant fragments of destroyed axis cylinders and 
medullary sheaths. The pia mater was greatly contracted, the posterior roots were 
only in part destroyed, while the anterior roots were transformed into an almost com- 
plete dense, fibrillary tissue. This destruction of the spinal cord continued in the 
lateral columns to the 8th dorsal segment, while in the posterior columns it could be 
followed, in the form of a peg-like focus to the lower cervical cord. The largest focus 



THE CLINICAL PICTURE OF DORSAL MYELITIS 



401 



also situated in the 4th dorsal segment (Fig. 167 d) consists of granular cells, remains 
of medullary sheaths and glia fibers, the latter becoming denser around the focus. 
With this there is the usual ascending degeneration in the posterior columns (Fig. 
167 ci-e) and in the lower border zone (cerebellar lateral column, Gowers' bundle). 
The muscles of the leg were in a condition of degenerative atrophy; the transverse 




Cervical Enlargement 




Dorsal Segment I 
Above the Lesion 



Below the Lesion 








Conus 



Dorsal Segment 6 



Fig. 167. — Destruction of the cord from the 8th dorsal segment downward, Fig. /, g, h. Ascending 
degeneration in the posterior and lateral columns, Fig. a-e. Central tubular hemorrhage in 
the left posterior column (near the posterior horn of the 6th dorsal segment, Fig. e), which 
in the 4th dorsal segment, Fig. d, distributes itself over both posterior columns. Fig. 
c, d, and e are enlarged five times, the remaining ones about twice. 



402 MYELITIS 

striation had disappeared, the muscle fibers were narrowed and permeated by fat 
globules. Of the nerves which supply the muscle fibers and of the nerve terminations 
nothing could be seen; the interstitial tissue, and especially the nuclei were increased. 

The remarkable point of this case consists in the total destruction of the cord from 
the 9th dorsal segment downward without demonstrable compression. The autopsy 
showed that the 11th thoracic vertebral body was compressed into a wedge shape, but 
it only protruded 2 mm. into the vertebral canal. As, however, immediately after the 
injury the signs of a separation of the cord appeared, a transitory compression of the 
cord must have taken place whereby the vertebra, either spontaneously or by exten- 
sion, must have returned to its original position. As in the remaining vertebrae and 
arches no changes could be demonstrated, the view is justified that compression only 
occurred at the 11th thoracic vertebra. If nevertheless such a large portion of the 
cord was destroyed this can only be explained by a hemorrhage which permeated the 
substance of the cord and destroyed it. The elongated focus in the posterior column 
may also be referred to hemorrhage. Traces of blood were scant which, however, can 
easily be explained from the age of the focus (15 months). 

A similar case was reported by A. Westphal ; it was one of traumatic myelitis 
(paraplegia) after fracture of the bodies of the 1st and 2nd lumber vertebrae. At the 
autopsy ( patient died 7 days after the trauma ) , it was shown that the vertebrae no- 
where exerted pressure upon the cord ; the same condition was also revealed by the 
membranes of the cord, the external configuration being normal throughout. The his- 
tologic examination showed the gray, and in part also the white, substance destroyed 
in the sacral cord while the remaining portions presented marked myelitic changes, 
which were regarded as the direct result of shock to the cord. 

We must still devote a few words to the symptoms on the part of the 
brain, the eye, the heart and lungs which occasionally appear in myelitis. 

The brain, apart from encephalomyelitis, the disseminated multiple form 
of myelitis, is only indirectly involved by embolism from the heart; or 
by septic and uremic intoxication. A mental depression must not be 
underestimated which affects the entire condition, the appetite and with 
it the nutrition and sleep thereby increasing the exhaustion and loss of 
strength. 

In myelitis, symptoms on the part of the optic nerve have, been observed 
(Erb, Bielschowsky). Optic neuritis with turbidity of the retina, injection 
of the vessels and even hemorrhage have been reported. These changes are 
usually not severe but are of great importance in the diagnosis of disseminated 
myelitis. According to Henneberg, the lesion of the optic nerve may arise 
from the direct ascent of the meningo-myelitic process to the base of the brain, 
or to the distribution of the toxic products in the cerebrospinal fluid, or by 
a rise of pressure in the interior of the skull in consequence of an increase 
of the cerebrospinal fluid (stasis inflammation). 

The ear may be affected ; otitis media may be the cause of the development 
of a descending meningomyelitis. 

The general circulation and the heart do not suffer directly; but from 
insufficient nutrition, from mental depression, as well as by shock and by 
various infections, the heart may be damaged, apart from cardiac affections 
which may have existed previously and become aggravated in the course of the 
disease. 

Pulmonary complications appear without direct connection with myelitis, 
from embolism or other causes, as bronchitis, not infrequently as hypostatic 
pneumonia or as metastatic abscesses originating from bed-sores or from 
cystopyelitis. 



THE CLINICAL PICTURE OF DORSAL MYELITIS 403 

3. STAGE OF REGENERATION 

The period of regeneration is introduced by the gradual return of spon- 
taneous movement and by an improvement in sensation. The muscles become 
more tense and the previously absent reflexes gradually reappear. The ulcers 
take on a better appearance, the bed-sores heal, the urine clears; simultane- 
ously the general condition improves; the mental condition, the ingestion 
of food and the appearance of the patient (tonus of the facial expression) 
become more favorable, the power of the heart increases. But the patient 
is still in danger, for relapses and complications cannot be excluded for some 
time. The anatomical faculty of regeneration of the spinal cord, according 
to recent investigations, appears to be possible. 

The duration of the period of regeneration up to a permanent arrest of 
the symptoms of paralysis cannot be determined with certainty. The more 
severe the first depressive stage the longer are the advances of the second, 
the period of restitution. The time up to the arrest of the disease, in mild 
cases, must be calculated at a few months; in severe cases many months even 
1 or 2 years. 

4. TERMINATION OF MYELITIS 

The terminations of myelitis are the following: 

(1) Complete resolution of the pathologic lesion, as well as entire func- 
tional recovery of the patient is quite rare. \Yhen this does occur, the cases 
are very mild, the diagnosis not always positive; but restitution o*f slight 
anatomical lesions has been exceedingly likely judging from the results: 

(a) in mild cases of serous myelitis (edema of the cord) ; 

(b) in syphilitic myelitis; 

(c) in myelitis after infectious diseases, especially the disseminated form; 

(d) in traumatic and compression myelitis. 

(2) Partial recovery is more common than complete. A decided improve- 
ment in the anatomic lesion cannot be doubted in these cases. 

(3) Termination in typical sclerosis. This is especially seen in multiple 
sclerosis following infectious diseases. In connection with this are 

(4) The conditions in which the residual processes in the spinal cord 
(cicatrization, degeneration, cysts, contraction) must be regarded as incur- 
able. On the other hand the sequels which the patient presents (paralyses, 
contractures, muscular atrophy, pain, cystitis, bed-sores, etc.) are more liable 
to improvement from proper treatment, which we owe particularly to the 
advances of physical-orthopedic therapy. 

(5) The termination of myelitis in abscess is very rare: observations of 
this kind have been published by Nothnagel. These were mostly metastatic 
medullary abscesses, occurring in the course of pulmonary abscess. The ab- 
scess, with the rapid development of paralytic symptoms, soon causes death. 

(6) The lethal termination is relatively frequent in the severe cases of 
transverse myelitis, as well as in bulbar myelitis. The autopsy, according 
to the duration of the disease, reveals the lesions of the fresh process, or at 
the periphery contracting connective tissue strands (cicatricial tissue), per- 
haps with small cavities (cyst formation) are already present. 

In connection with these lesions in the spinal cord, those of other organs 
27 



404 MYELITIS 

must be considered and present more or less dependent complications or 
sequels. We must mention briefly (a) disease of the vertebra?, intraspinal 
tumors, (b) cystitis with pyelitis and nephritis, bed-sores and foci of general 
septic infection in the lungs, heart, or skin, (c) more rarely cardiac and pul- 
monary disease (hypostatic pneumonia), (d) muscular atrophy, changes in 
the bones and joints, (e) syphilitic changes in the internal organs. 

Before proceeding to the clinical description of the forms of cervical, lum- 
bar and sacral myelitis, it is necessary to describe the regional diagnosis of 
diseases of the cord. For this purpose we must first explain: 

THE SEGMENTARY LOCALIZATION OF THE FUNCTION OF MOTION 

Myelitis according to its height and transverse diameter in the cord may 
present a manifold clinical picture. An understanding of this varying physiog- 
nomy of myelitis is gained from an appreciation of the segmental arrangement 
of the spinal functions, which we must discuss somewhat minutely as it forms 
the foundation of the topical diagnosis of disease of the cord. Not only in 
the brain, but also in the spinal cord, are ganglion cells, which are functionally 
united for the motion, sensation and reflexes of the various members, grouped 
in definite parts, one above the other, that is in segments. Of these there are 
31 in the cord, corresponding to the nerve root pairs, 8 cervical, 12 thoracic, 
5 lumbar and 5 sacral segments and one in the coccyx. 

These segments do not correspond in location to the vertebrae of similar 
names, but are higher than the point of exit of the nerve roots belonging 
thereto. This difference increases caudally. It is the consequence of the 
relative shortness of the spinal cord, which is only 45 cm. (compared with the 
vertebral column which is 70 cm.), and therefore at the top of the first, occa- 
sionally of the second, lumbar vertebra (especially in women) finds its termi- 
nation. In the embryo the cord fills the entire vertebral canal; but in the 
further course of fetal development the longitudinal growth of the cord no 
longer keeps pace with that of the vertebral column, especially its lumbosacral 
portion, so that finally in the new-born the 4 lower lumbar vertebrae and the 
sacrum contain no cord but only the cauda equina. 

The knowledge of this upward shifting of the cord segments is of great 
importance in diagnostic and especially in surgical considerations. Thus a 
morbid focus located in the lumbar cord should not be sought for in the 
lumbar vertebral column, but in the lowest two thoracic vertebrae. On the 
other hand operations may be performed upon the lower lumbar vertebrae and 
upon the sacrum (resection, puncture) without danger of injuring the spinal 
cord. The cervical cord segments are situated about one vertebra higher than 
their corresponding vertebra. In the thoracic segments this difference amounts 
to from 2-3 vertebrae, so that finally the lumbar cord is at the height of the 
2 lowest thoracic vertebrae. The sacral cord is situated behind the first lum- 
bar vertebra and the conus medullaris behind the succeeding intervertebral 
disc, sometimes even behind the upper half of the second lumbar vertebra. 
It must, therefore, be remembered that the cord focus is always higher than 
the vertebra which numerically corresponds to it. 

Prior to a consideration of the various clinical pictures presented by dis- 
ease of the individual sections of the cord, we shall discuss the spinal cen- 



SEGMENTARY LOCALIZATION OF THE FUNCTION OF MOTION 405 

tralization of motor and reflex functions in a tabular form. The credit of 
having constructed the laws of segmentary innervation is due to a number 
of physiologists, anatomists, and clinicians, but especially to surgeons. We 
refer to Bolk, Bramwell, Bruns, Edinger, Goldscheider, Gowers, Head, Kocher, 
Fr. Kraus, Lapinsky, Oppenheim, Eoss, Seiffer, Sherrington, Allen Starr, 
Thornburn, Wagner and Stolper, Wichmann and others whose experiences we 
have utilized in this compilation. 

We shall not, as has been customary in previous compilations, regard each 
segment as an elementary central organ, but on the contrary attempt to pre- 
sent the localization of the muscle groups which functionally act together. 
One segment never acts alone, never does a single muscle contract, but always 
several segments are innervated simultaneously. Even the simplest move- 
ments are not brought about by the contraction of an isolated muscle, but by 
the harmonious coordinated effect of several muscles. Thus a simple cortical 
impulse necessary to flex the elbow employs 4 segments, in corresponding to the 
localization of the flexors of the elbow joint (brachialis internus, biceps, brachio- 
radialis, pronator teres, extensor carpi radialis longus) from the 4-7 cervical 
segments. No single one of these muscles can voluntarily be contracted by 
itself. Besides every single muscle, as well as one and the same cutaneous 
area, especially in the limbs, is in connection with at least 3 nerve roots, that 
is, segments (compare P. Lazarus, "tJber die spinale Localisation der motor- 
ischen Functionen," Zcitschr. f. hlin. Med., Bd. 57, p. 99 et scq., 1905). 

Thus the biceps is innervated by 4 segments (4, 5, 6, and 7th cervical 
segments). Usually only one or 2 segments contain the center for the main 
function of the muscle in question, while the neighboring segments are mainly 
concerned in coordination. As at the present time we possess no definite 
knowledge in regard to the localization of muscle nuclei, in the following 
table all segments are included, which may be in direct connection with the 
respective muscle groups. Subsequent investigation will be required to modify 
or correct our tabulation. 

In connection with this multi-segmentary localization and innervation is 
the fact that one and the same muscle may be involved in various functional 
movements. Thus the biceps may elevate the arm anteriorh', flex the fore- 
arm and finally supinate it without it being possible for us at the present 
time to state definitely, which ganglion cells of the segments concerned sup- 
ply one or the other function. Nor does the stimulation of a single anterior 
root of a multi-segmentary muscle lead to the contraction of the entire mus- 
cle, but only to a definite portion. Neither does section of a single root pro- 
duce complete paralysis, but the muscle is rendered useless only for certain 
movements, in which it was correlated with other muscles (Sherrington, Eus- 
sell and others). 

In conformity with this we note in some spinal diseases, such as Du- 
chenne- Aran's paralysis, that only some portions of the muscle atrophy, while 
others remain normal ; consequently the muscle may be paralyzed only in one 
function, while in other respects it acts normally. The loss of function, 
therefore, of a single segment or root produces a partial functional disturb- 
ance, which may be compensated by the vicarious action of retained adjacent 
segments. 

The joints are also supplied by several segments or nerves. Thus upon 



406 MYELITIS 

the extensor surface, the knee joint is innervated by the crural, and upon 
the flexor side by the sciatic, nerves. Every cutaneous area beside being in 
connection with its own segment is to a certain extent also influenced by the 
adjacent segments, above and below. Only the loss of function of at least 
3 segments produces complete paralysis. In contrast to this, paralysis pro- 
duced by lesions of a peripheral nerve implicates the entire area of distribu- 
tion of the nerve. For this reason segmental paralysis has a different limit 
than that brought about by disease of the peripheral nerve. 

In the following tables the separate motor functions and the reflexes, 
with the segments that supply them, are placed together. This grouping 
we employ in the examination of spinal lesions. From a methodic and de- 
tailed investigation of the power of motion, of sensation, and of the reflexes, 
or from the absence of definite functions, we are enabled to conclude which 
components of the muscle and nerve are involved and thus determine the seg- 
ment that is affected. 

Further it may be noted from the table, that one and the same segment 
may contain the fibers for the antagonists as well as of the agonists (both 
acting together in every coordinate movement), and that* the functional area 
of the segment does not coincide with the region of innervation of the periph- 
eral nerve trunk. The spinal cord segment like the brain supplies no single 
muscle or nerve, but only power for a definite kind of motion. • 

This segmentation is even more marked in the lower animals than it is in 
man. Thus in the zebra, it is marked by the stripes of colored hair, in the 
ring snake by the roseate swelling of the spinal cord corresponding to the 
points of exit of the nerve roots (Luderitz). In man the individual medul- 
lary segments are not so sharply separated from each other; they are united 
by numerous " intersegmental and conjunction tracts." The enlargement of 
the cervical and lumbar areas (spinal arm and leg centers) merely correspond 
to the complicated functions of the arms and legs. 

Finally we must mention that up to this time we only know the projection 
of the spinal cord segments into 2 organ systems, into the skin and muscles, 
while in regard to the spinal innervation of the internal organs, especially 
of the heart, of the vessels, of the intestinal tract (with the exception of the 
sphincters) and even of the bones and joints, we possess no positive knowledge. 

SPINAL LOCALIZATION OF THE MOTOR FUNCTIONS 

Abbreviations : C, D., L., S. = Cervical, Dorsal, Lumbar, Sacral. 
S. = Segment. N. = Nerve. Plex. = Plexus. 

Head Movements: I.-VIII. C. S. 

Flexors of the head : I.-V. C. S. Extensors of the head : I.-VIII. C. S. 

(Nervi cervical.-Nerv. accessorius. ) (Nervi cervical.-N. suboccipital, 

N. accessorius, N. occipital, ma- 
jor-) 

Rotators of the head and lateral movements: I.-VIII. C. S. 
(N. suboccipital.-N. accessorius.-N. cervical.) 



SEGMENTARY LOCALIZATION OF THE FUNCTION OF MOTION 407 

Xeck Movements: I.-VIII. C. S. 

Flexors: II.-VIII. C. S. Extensors: I.-VIII. C. S. 

(Plex. cervical, et. brach.) (Plex. cervical, et. brach.) 

Rotators and lateral movements: I.-VIII. C. S. 
(X. suboccipit.-X. cervical. I.-VIII, Plex. brach., X. dorsalis scapulae.) 

Movements of the hyoid bone : I.— III. C. S. 

Elevators : I.-IL C. S. Depressors : I.-III. C. S. 

(Ramus mylohyoideus X. trigemini (Ram. descend. X. hypoglossi.) 

ram. III., X. facialis et hypo- 
glossus.) 

Back and Trunk Movements: I. D. S.-I. S. S. (perhaps also with the 
action of the I.-VIII. C. S.) 

Retracting flexors (extensors) I. C. Erectors: V. D. S.-IV. L. S. 

S.-I. s. s. 

(X. cervical.-thoracal.-lumbal-sa- (X. intercostal. V.-XII., X. ilio- 

cral, X. thoraco-dorsalis, X. glu- hypogastricus - X. ilioguinalis, 

taeus inf.) plex lumbalis.) 

Rotators and lateral movements : I. C. S.-I. S. S. 
( X. cervical.-thorac.-lumbal.-sacral. ) 

Abdominal Muscles: V. D. S.-I. L. S. 
(X. thoracal. V.-XII. X. iliohypogastr.-X. iliolinginalis. ) 

Respiratory Movements: (Medulla oblong, up to XII. D. S.) 

Inspiratory muscles: .Med. oblong.- Expiratory muscles: II. D. S.-I. 

XII. D. S. L. S. 

(X. facialis, X. vagus, X. phreni- (X. intercostal. II.-XI.-X. ilio- 

cus, X. accessorius, X. intercos- hypogastr. et X. ilioinguinalis. ) 
tal. I.-XIL, Plex. cervic. et bra- 
chialis.) 

Abdominal press: VII. D. S.-I. L. S. 

(X. intercost. VII.-XII, X. iliohypogastric, et ilioinguinal.) 

Upper Extremity 

Shoulder girdle and also shoulder joint: II. C. S.-VIII. C. S. 

Elevators: II. C. S. Depressors: V. C. S.-VIII. C. S. 

(X. accessorius, Plex. cervical., X. (X. subscapularis X. thoracal, an- 

dorsal. scapul., X. thoracic. ter.) 
long.) 

Adduction: II. C. S.-VIII. C. S. Abduction: V. C. S.-VII. C. S. 

(X. accessor.-X. subscapularis, X. (X. thoracal. long.-X. subscapu- 

dorsal. scapul.) lar.) 



408 



MYELITIS 



Shoulder joint : IV. C. S.-VIII. C. S. 



Abduction: IV.-VI. C. S. 

(N. axillaris, N. thoracic, long., N. 
suprascapular. ) 
Forward: IV. C. S.-VII. C. S. 
(N. axillaris, N". musculocutaneous, 
N. thoracic. long.-N", thorac. 
ant.) 
Inward rotator: V. C. S.-VII. C. S. 
(N. subscapularis, N. thoracal, 
ant.) 



Adduction: 1V.-VIII. C. S. 

(N. thoracal, ant., N. suprascapu- 
lar.-]^, subscapul., N. radialis.) 
Backward: IV. C. S.-VII. C. S. 

(N. axillaris, N. subscapular.) 



Outward rotator: IV. C. S.-VI. C. S. 
(N. suprascapular, N. axillaris.) 



Elbow joint: IV.-VIII. C. S. 



Extension: V. C. S.-VIII. C. S. 

(N. radialis.) 

Supination : V. C. S.-VIII. C. S. 
(N. radialis, N. musculocutane- 



Flexion: IV. C. S.-VII. C. S. 

(N. musculocutan.-N. median.-N. 
axillaris. ) 
Pronation: VI.-VIII. C. S. 

(N. medianus.) 



ous.) 



Wrist joint: VI. C. S.-I. D. S. 



Extension: VI. C. S.-VIII. C. S. Flexion: VI. C. S.-I. D. S. 

(N. radialis.) (N. median, ulnaris et radialis.) 

Radial flexion: VI. C. S.-VIII. C. S. Ulnar flexion: VI. C. S.-I. D. S. 

(N. radial, et median.) (N. radial, et ulnar.) 

Finger movements: VI. C. S.-I. D. S. 

Extension: VI. C. S.-VIII. C. S. Flexion: VII. C. S.-I. D. S. 

(N. radialis.) (N. median, et N. ulnar.) 

Abduction: VII. C. S.-I. D. S. Adduction: VII. C. S.-I. D. S. 

(N. ulnar.-N. radial.) (N. ulnaris.) 



Thumb movements: VI. C. S.-I. D. S. 



Extension: VI.-VIII. C. S. 

(N. radialis.) 
Abduction: VI. C. S.-I. D. S. 

(N. radialis.) 
Opposition: VI. C. S.-I. D. S. 

(N. radial., median et ulnar.) 



Flexion: VI.-VII. C. S. 

(N. ulnar, et median.) 
Adduction : VII. C. S.-I. D. S. 

(N. ulnaris.) 



Movements of the little finger: VII. C. S.-I. D. S. 



Extension : VII. C. S.-I. D. S. 

(N. radial, et ulnar.) 
Abduction: VII. C. S.-I. D. S. 

(N. ulnaris.) 
Opposition : VII.-VIII. C. S. 

(N. ulnaris.) 



Flexion: VII. C. S.-I. D. S. 

(N. ulnar, et median.) 
Adduction: VIII. C. S.-I. D. 

(N. ulnaris.) 



SEGMENTARY LOCALIZATION OF THE FUNCTION OF MOTION 409 



Lower Extremity 
Hip joint: XII. D. S.-I. S. S. 



Extension : IV. L. S.-I. S. S. 

(X. glutseus inf.-X. peron. com- 
mun.-X. tibialis.-Plex. ischiad- 
ic.) 
Abduction: IV. L. S.-I. S. S. 

(X. glut, sup.) 
Outward rotators: II. L. S.-I. S. S. 
(X. tibial.-Plex. ischiadic.-X. ob- 
turat.-Plex. lumb.-X. femor.-X. 
glut, inf.) 



Flexion : XII. D. S.-I. S. S. 

(Plex. lumb.-X. femor.-X. glut. 
sup.-X. obturat.) 

Adduction: III. L. S.-I. S. S. 

(X. femor. X. obtur.-X. tibial.) 
Inward rotators : II. L. S.-I. S. S. 

(X. glut. sup.-X. obturat.) 



Knee joint: II. L. S.-II. S. S. 



Extension : II.-IV. L. S. 
(X. femor.) 



Flexion : III. L. S.-II. S. S. 

(X. tibial.-X. peron. com.-X. 
f emor.-X. obturat. ) 



Ankle joint: IV. L. S.-II. S. S. 



Dorsal flexion : IV. L. S.-I. S. S. 

(X. peron. prof.) 
Adduction: IV. L. S.-II. S. S. 

(X. tibial.-X. peron. prof.) 
Pronation : IV. L. S.-I. S. S. 

(X. peron. superfic. et prof.) 



Plantar flexion : IV. L. S.-II. S. S. 

(X. tibialis, X. peron. superfic.) 
Abduction: IV. L. S.-I. S. S. 

(X. peron. superfic. et prof.) 
Supination: IV. L. S.-II. S. S. 

(X. tibialis, X. peron. prof.) 



Toe movements: :' IV. L. S.-II. S. S. 



Dorsal flexion : IV. L. S.-I. S. S. 
(X. peron. prof.) 

Adduction: I. et II. S. S. 
(X. plant, lateral.) 



Plantar flexion : V. L. S.-II. S. S. 

(X. tibial.-X. plantar, med. et lat- 
eral.) 
Abduction: I. et II. S. S. 

(X. plant, lateral.) 



Great toe: IV. L. S.-II. S. S. 



Extension : IV. L. S.-I. S. S. 
(X. peron. prof.) 



Flexion : V. L. S. I.-II. S. S. 

(X. tibialis, X. plant, med. et 
later. ) 



SPINAL LOCALIZATION OF THE REFLEXES 

Tendon reflexes : 
Achilles tendon reflex : III. L. S.-I. S. S. 
Patellar tendon reflex : II.-IV. L. S. 
Extensor reflex in the upper and lower arm : VI. C. S. 
Flexor reflex (closure of the fingers upon tapping the palm) : VII. C. S. 



410 MYELITIS 

II. Cutaneous reflexes : 

Sole of the foot reflex: IV.-V. L. S. (perhaps also I. II. S. S.). 
Babinski's reflex: dependent upon the intactness of the pyramidal- 
lateral column tract. 
Cremaster reflex: I. II. L. S. 
GlutsBl reflex : IV.-V. L. S. 
Abdominal wall reflex 

epigastric: IV.-VII. D. S.; 

meso- and hypogastric : VIII.— XI. D. S. 
Interscapular reflex : V. C. S.-I. D. S. 

III. Reflexes of the smooth muscles: 

Cilio-spinal center : VI. C. S.-III. D. S. 

Dilatation of the pupil (upon irritation of the nape) : III.— VII. C. S. 

Ejaculatory reflex center: III. S. S. 

Erection reflex center: II— IV. S. S. 

Bladder and rectal reflex centers : III.-V. S. S. 

(The rectal reflex center is situated below the bladder reflex center.) 

TRANSVERSE DIAGNOSIS 

Myelitis may principally attack the central gray matter — poliomyelitis 
already mentioned — which appears to be the case especially in disease of the 
cervical and lumbar enlargements. It is, however, more common for the in- 
flammation to affect the white substance more intensively: border myelitis, 
I euko myelitis. Ordinarily myelitis attacks the gray and white substance in an 
irregular arrangement. In the localization diagnosis of myelitis not only the 
height of the lesion but also the transverse distribution, and with this the 
complete topography of the focus, is to be determined. The topographical 
transverse diagnosis is only possible through a minute knowledge of the func- 
tions of each individual fiber system. For this purpose the adjoining figure, 
which presents a diagram of a transverse section of the spinal cord, contain- 
ing the motor and sensory functional regions, should be consulted (Fig. 168). 

A lesion of the gray substance leads, almost exclusively, to a loss of func- 
tion in the area of innervation to which it belongs, while destruction of the 
white substance also interrupts those tracts of conduction which pass the seg- 
ment in question on the road to and from the brain. In this manner a remote 
action is produced in all of the segments situated above and below the lesion, 
which are present in the direction of physiologic conduction (cellulifugal) 
leading to ascending and descending degeneration; this condition may be 
demonstrated during the second week, after the lesion has taken place. 

The anterior horn cells represent the motor, trophic and probably also the 
vasomotor centers, and isolated disease of this structure reveals the picture 
of atrophic muscular paralysis, such as we see in infantile spinal paralysis or in 
progressive spinal muscular atrophy. In both, flaccid paralysis with rapidly 
progressing muscular atrophy, with abolished reflexes and with DeE, are char- 
acteristic. Frequently vasomotor disturbances and anomalies in the secre- 
tion of sweat are added. All sensory and motor functions of the segments, 
situated above and below the point of lesion, are intact as well as those of 



TRANSVERSE DIAGNOSIS 



411 



the bladder and rectum. In case a few ganglion cells remain normal within 
the affected anterior horn then the muscle fibers supplied by them retain 
their function. Disease of the anterior root produces the same picture as a 
lesion of the anterior horn to which it belongs; both including the axis cylin- 
der which originates there form an anatomical unit, the spino-muscular neuron. 




- 17 



Fig. 168. — Schematic Representation of the Sensory and Motor Spinal Cord Tracts. 



Right: Descending tracts (transverse shading 
with circles). 



Left: Ascending conduction tracts (longitudinal 
shading and dotted). 

i, Anterior column-ground bundle; 2, Gowers' column; 3, Lateral column remains; 4, 
Clarke's columns; 5, Cerebellar lateral column tract; 6, Lateral boundary layer of the 
gray substance; 7, Posterior horn; 8, Lissauer's border zone; 9, Spinal ganglion with 
the posterior root fibers; 10, Burdach's column; 11, Goll's column; 12, Pyramidal 
anterior column tract; 13, Anterior root fibers; H, Motor anterior horn cells; 15, Mon- 
akow's rubrospinal bundle; 16, Pyramidal lateral column tract; 17, Ventral posterior 
column field; 18. Descending dorsal root fibers (Schultze's "comma field"); 19, Oval 
field (Flechsig). 

The shaded areas on the right characterize the most frequent distribution of descending, those 
on the left of ascending, degeneration after transverse lesions of the cord. 



In contrast to this, disease of the cerebrospinal neuron, the pyramidal tract, 
gives rise to entirely different symptoms. The origin of the pyramidal tract 
is in the large pyramidal cells of the cerebral cortex, its termination in the 
crossed anterior horn. Lesion of the pyramidal tract not only produces an 
increase of tension of the muscle tonus (hypertonia) in the area of innerva- 
tion of the destroyed segment, but also in those lying below; further there is 
an increase of the tendon reflexes, a positive Babinski toe reflex and some 
weakness of the muscles. Neither atrophy (apart from the atrophy of inac- 
tivity), nor complete paralysis occurs; automatic and reflex movements, as well 
as electric contractility are retained. A lesion of the pyramidal lateral column 
28 



412 MYELITIS 

tract may be compensated for, to a certain extent, by the vicarious action of 
the motor reserve tracts (pyramidal anterior column tract, the ganglion tracts), 
in contrast to the irreparable functional destruction after disease of the ante- 
rior horns. We must mention briefly that combined disease of the entire 
motor system (anterior horn and pyramidal tract) produces the well known 
picture of amyotrophic lateral sclerosis (paralysis with muscular atrophy and 
spasm). According to recent investigations it appears doubtful whether the 
spastic condition is due to a lesion of the pyramidal tract or that it is only 
the consequence of an irritation of the anterior horn cells. 

The symptoms of absence of function in the other descending tracts, from 
the cerebellum, the optic thalami, the corpora quadrigemina, the red nucleus 
and the olives which pass in part into the lateral columns, partly into the 
anterior columns, are as yet not absolutely determined. 

Sensory disturbances only appear in diseases of the posterior roots and in 
their area of distribution in the posterior horns and in the posterior columns. 
An affection of the posterior roots or of the posterior columns produces com- 
plete segmentary anesthesia and analgesia, with loss of the tendon reflexes. 
An irritative condition of the posterior roots gives rise to paresthesia and 
hyperesthesia, and also to lancinating pains and girdle sensations, from the 
position of which we may sometimes determine the height of the medullary 
focus. 

A partial sensory defect points to the intramedullary seat of the spinal 
paralysis. Dissociated sensory paralysis is characteristic of an isolated disease 
of the posterior horn, i. e., the arrest of pain and temperature sense with 
retained tactile sensation. In unilateral disease of the cord this sensory paral- 
ysis occurs in the area of innervation of the lesion upon the same side, on 
the other hand, in the area of innervation of the regions of the cord situated 
below upon the opposite side, for the sensory tracts cross within the central 
gray matter (compare the clinical picture of Brown- Sequard's paralysis). 

Disturbance of the muscle sense with decrease of the muscle tonus (hypo- 
tonia), and disturbance of the tactile and pressure senses with but slight loss 
of cutaneous sensation, are characteristic of an isolated disease of the posterior 
column. An affection of the posterior column produces the picture of tabetic 
ataxia, which at the present time is generally referred to a disturbance of sen- 
sation. The functional importance of the remaining ascending tracts, of the 
delicate sensory anterior column ground bundle, also of the cerebellar lateral 
column tracts and of Gowers' tract, have not yet been positively demonstrated ; 
possibly the cerebellar lateral column tract, which arises in Clarke's cells, pass- 
ing to the corpus restiforme and cerebellum, serves to regulate equilibrium; 
according to Gowers the tract named after him conducts the sensation of pain, 
while other authors include Gowers' bundle among the equilibrium tracts be- 
cause it terminates in the cerebellum. 

After having described the pathologic anatomy and clinical picture of 
myelitis, as well as the symptomatology of the motor, sensory, and reflex dis- 
turbances, according to the height and transverse distribution of the myelitic 
process, we shall present the individual clinical forms of myelitis; in this we 
shall include a description of the etiology, prognosis and treatment of the 
disease. 



TRANSVERSE DIAGNOSIS 413 



1. CERVICAL MYELITIS 



Inflammation of the cervical cord is the rarest and simultaneously the 
severest form of myelitis; it develops as the consequence of compression of 
the cord and may produce paralysis, as well as anesthesia of the trunk and 
of all 4 members ; further, a disturbance of the eyes and of the face ; and on 
account of paralysis of the diaphragm, the respiration may be so influenced 
that death from suffocation sometimes takes place. 

Complete transverse disease of the cervical cord is rarer than in the dorsal 
cord, as the former is larger than the latter; according to Stilling' s measure- 
ments the transverse segment of the cervical cord varies between 44.7 and 
62.4 cmm., in contrast to 28.6-36.2 cmm. in the dorsal cord (measured in 
the child). 

Diseases of the cervical cord may be divided into 2 groups; in the first— 
superior type — are affections of the 4 upper cervical segments, while to the 
second group — inferior type — belong the maladies of the 4 lower cervical seg- 
ments. The diseases of the upper dorsal segment may be included with those 
of the inferior type. It is located behind the seventh cervical vertebra and 
anatomically as well as functionally belongs to the cervical enlargement which 
reaches from the 2d dorsal segment to the 5th cervical segment inclusive. 

Disease of the -4 upper cervical segments directly endangers life. Affec- 
tions of the phrenic center which is mainly contained in the 4 cervical seg- 
ment are particularly serious ; this is situated behind the upper portion of the 
4th cervical vertebral body. In a total transverse disease at this height (typus 
quartos) symptoms of paralysis of the diaphragm appear: the epigastrium 
retracts during inspiration, the respiratory auxiliary muscles act energetically, 
but nevertheless little air enters into the pulmonary alveoli; on the other hand 
considerable blood enters the alveolar vessels; the dangerous symptoms of air 
hunger and of asphyxia (dyspnea, cyanosis) appear. Added to this, from the 
insufficient action of the diaphram, abdominal symptoms arise (downward 
displacement of the liver, meteorism, retentio alvi, engorgement of the cir- 
culation). The ordinary outcome of paralysis of tJie phrenic nerve is death 
from suffocation. Only in unilateral disease of the phrenic center, life may 
exist so long as the opposite half of the diaphragm continues to function. 
Unilateral paralysis of the phrenic nerve produces paralysis of the same side 
of the diaphragm; this half is elevated (cadaver position) and the lung 
above it takes no part in respiration. In this portion of the pulmonary system 
hypostatic pneumonia or a purulent bronchitis may readily develop and fre- 
quently enough terminates fatally. 

The symptoms of absence of function in disease of the upper cervical seg- 
ments (the first cervical segment is situated in the arch of the atlas) have 
not been accurately determined ; at all events life can exist only with a partial 
lesion or with a very slight compression of the upper cervical segments. In 
an implication of the upper cervical segments all four members may show 
spastic paralysis; muscular atrophy arises only when the lower cervical seg- 
ments are involved (Kahler). Besides there is usually paralysis of the deep 
muscles of the neck and nape, of the sternocleidomastoid and of the trapezius. 
Further there may be difficulty in the production of the voice and disturbance 
in deglutition, as well as paralysis of the muscles of the palate and tongue; 



414 MYELITIS 

the origin of the hypoglossal and spinal accessory nerves reach as low as the 
second and third cervical segment. These symptoms thus form a transitional 
stage to bulbar paralysis, which not infrequently is due to an inflammation 
ascending from the cervical cord to the bulbus medulla; and gives rise to the 
clinical picture of atrophic glosso-pharyngo-labial paralysis (see Ley den, 
Archiv fur Psychiatr. u. Nervenlch., 1870, Bd. II u. Ill u. VII : acute bulbar 
paralysis). 

Cerebral symptoms rarely arise in uncomplicated cervical cord inflamma- 
tion. Only when the inflammation attains the medulla and the brain, or 
is propagated to the membranes of the cord and thence to the meninges, or 
in cerebral and spinal disseminated inflammation, do cerebral symptoms ap- 
pear; such as headache, altered states of consciousness, paralyses of the cere- 
bral nerves and not rarely optic neuritis (Erb, Bielschowsky). 

In regard to sensory phenomena in cervical myelitis there may be anes- 
thesia distributed over the entire body from the toes to the back of the head. 
The occiput is innervated from the first and second cervical segments; the 
third cervical segment supplies the anterior and lateral skin of the neck, the 
fourth supplies the skin of the nape, the shoulder and the region of the 
clavicle to a point near the second intercostal space. The skin of the trunk 
below this is not supplied by the 4 lower cervical segments but from the 
dorsal cord. The 4 lower cervical segments and the first dorsal segment have 
no part in the sensory innervation of the neck and trunk; they supply the 
skin of the arms in the form of longitudinal striae, which, corresponding to the 
height of the segment, run upon the radial and ulnar sides. 

Symptoms of paralysis of the arms occur not only in disease of the 4 lower 
cervical segments but also with involvement of the 2 upper dorsal segments. 
The latter are situated behind the 7th cervical vertebra and the intervertebral 
disc beneath. In an inflammation of the 2 lowest cervical segments and of 
the 2 upper dorsal segments there is paralysis from the elbow joint down- 
ward (paralysis of the forearm). The ulnar muscles as well as the small mus- 
cles of the hand and fingers are most markedly affected and there is often 
atrophy and DeE. Ulnar flexion of the hand, all of the movements of the 
little finger, of the interossei and lumbricales, as well as adduction of the 
thumb are usually decidedly paretic, while the other movements of the thumb 
as well as of the long flexors and extensors of the fingers and of the hand 
are only somewhat weakened. Extension of the elbow joint, pronation, adduc- 
tion and the backward movement of the forearm may occasionally be paretic, 
while supination (brachioradialis) and flexion of the elbow joint are quite 
normal. 

The muscular atrophy appearing in the paralyzed members is character- 
istic of a lesion in the lower cervical cord; this is absent when the interrup- 
tion in conduction is situated higher up (Kahler, also F. Kraus, Die Bestim- 
mung des betroffenen Ruckenmarkssegmentes bei Erkrankungen der unteren 
Halswirbel, Zeitschr. f. Hin. Med., 1891, XVIII. Bd). 

In disease of the first dorsal segment, but especially in lesions of the first 
anterior dorsal root, besides paralysis of the small muscles of the fingers and 
anesthesia in the ulnar distribution, the symptoms of Klumpke's paralysis are 
observed: contraction of the pupil and of the palpebral fissure (miosis et 
ptosis spinalis paralytica) with softening and retention of the eyeball (paral- 



TRANSVERSE DIAGNOSIS 415 

ysis of Midler's muscle). Sometimes there is vascular dilatation and wast- 
ing of the same side of the face (flattening of the cheek). These symptoms 
are referred to an implication of the sympathetic nerve. The sphincter iridis 
is supplied by the oculomotor nerve, and the dilator of the pupil by the cervi- 
cal sympathetic. Its oculopupillary fibers arise in the oculopupillary center 
of the medulla; they pass down through the cervical cord and out in great 
part through the first pair of dorsal roots. Thence they pass through the 
communicating branches to the inferior cervical ganglion of the sympathetic, 
from here into the cavity of the skull and then along the sinus cavernosus 
to the orbit. Irritation of the first dorsal root dilates the pupil of the same 
side. This was experimentally proven by Oppenheim, in a case trephined by 
v. Bergmann, in which the cervicodorsal vertebrae were opened on account of 
a gun shot injury. Along with the dilator fibers, the nerve fibers for the 
smooth muscles of the orbits and lids pass from the medulla to the first ante- 
rior dorsal root; therefore an isolated lesion paralyzes nearly all sympathetic 
ocular fibers. But a lesion of the medullary segments above this, up to the 
medulla, may also produce pupillary disturbances, as all cervical segments 
are penetrated by the pupillary fibers. On the other hand, according to 
Klumpke's experience, oculopupillary symptoms never appear in plexus lesions. 

If the inflammation also involves the 5th and 6th cervical segments, which 
like the two lower segments are about 2-| cm. in length, the paralysis is dis- 
tributed to the upper arms. Most of the cell groups for the functions of the 
deltoid, brachioradialis, brachialis internus, coracobrachialis, teres minor, infra- 
spinatus, supraspinatus and of the rhomboidei are contained in the 5th cervical 
segment. In disease of the 5th cervical segment the arm hangs flaccidly, as if 
dead; besides there may also be paresis of the scaleni, as well as of the deep 
muscles of the neck and nape. The consequence is difficulty in rotary and 
flexure movements and in fixation of the head; the respiration, as the result 
of paresis of the auxiliary muscles of respiration, may be of pure abdominal 
type. According to Thorburn and Kocher, an outward rotation and abducted 
position of the arm with a flexed elbow joint, is characteristic of disease of 
the 6th cervical segment, as the muscles centralized in the 5th cervical segment 
retain their function and produce this posture. 

When the inflammation is exclusively limited to the anterior horns of the 
cervical enlargement, that is to the anterior roots, then a flaccid atrophic paral- 
ysis arises, restricted to the arms, if the inflammation is distributed to the 
long tracts in the white medullary substance, a spastic paralysis of the legs 
occurs in addition. 

A complication of myelitis with spinal meningitis is not rare, particularly 
in the region of the cervical enlargement, as the membranes of the cord are 
in immediate proximity. Spinal meningitis is recognized from the severe 
pains in the nape, which radiate to the tips of the fingers, by contracture of 
the muscles at the back of the neck, by sensitiveness to pressure and rigidity 
of the vertebral column, and by Kernig's sign (incapability of extending the 
knee in the sitting posture on account of contracture of the flexors of the 
knee), as well as by hyperesthesia of the skin and muscles. The spinal symp- 
toms, especially cervical neuralgia and sensory paralysis in caries of the cervical 
vertebra, may for a long time precede the direct vertebral ones (deformity, 
pain, gravitation abscess) (Kahler). 



416 MYELITIS 

In transverse myelitis of the 1th and 8th cervical segments and also in dis- 
ease of the first dorsal segment (lower arm type) the anesthesia extends to the 
ulnar region, inner surface of the arm, the back of the hand, the ball of the 
little finger, the entire extent of the two last fingers and to the second finger 
(only upon the dorsal surface). Compare the diagram of sensory topography, 
Fig. 176. The other fingers and the region of the hand may present mild 
sensory disturbance (hypesthesia), the thumb being least affected. Only in 
destruction of the 5th and 6th cervical segments is the anesthesia distributed to 
the radial side of the forearm which is characteristic of this condition; the 
anesthesia then involves the entire arm. The neck, nape and occiput, how- 
ever, remain free as these structures are innervated by the four upper cervical 
segments. If the cervical cord is only partially affected in its transverse sec- 
tion, the sensory involvement will vary according to the position of the lesion ; 
perhaps the legs may be unaffected and only the arms attacked. Sensation 
and motion cannot be so accurately limited in myelitis as in our physiologic 
diagrams, for myelitis does not present an anatomical sharply circumscribed, 
but a diffuse, inflammatory process. Only in complete transverse disease is 
it characteristic that the distribution of the sensory paralysis is present from 
the tips of the toes to the uppermost diseased segment area. 

In cervical cord myelitis paresthesia and lancinating, radiating pain, from 
the nape to the arms and tips of the fingers, are not rare. On the other hand 
in disease of the lower cervical cord the girdle sensation, at the boundary 
of the thoracic anesthesia in the second intercostal space, is absent. The lower 
four cervical segments only supply the arms with sensation, so that the sen- 
sory area of the 2d dorsal segment immediately adjoins that of the 4th cervical 
segment at the top of the thorax. Only in disease of the latter are the supra- 
clavicular nerves, which originate from it, involved. 

Headache is common in cervical myelitis. One of our patients complained 
of a tormenting neuralgic occipital pain. The large occipital nerve originates 
from the upper cervical cord. Hemicrania is also observed in this form of 
the disease and may be referred to the fibers in the cervical sympathetic origi- 
nating from the cervical cord. 

In lesions of the lowest portions of the cervical cord and of the first dorsal 
segment a fall in temperature sometimes appears, while in disease of the upper 
cervical cord there is said to be a rise (Thorburn). The latter condition has 
been observed by Wagner and Stolper even after simple contusions of the nape 
of the neck. On the other hand Kocher, from his experience with compres- 
sion of the cervical cord after injuries to the vertebrae, could not confirm this 
behavior of the temperature with the seat of the lesion in the cervical cord. 

The behavior of the arm reflexes, in inflammation of the cervical cord, 
is very difficult to estimate, because they may be absent in normal persons or 
very difficult to demonstrate. In a unilateral inflammation differences in the 
condition of the arm reflexes may be of value in the diagnosis. The reflexes 
of the lower extremities and of the abdomen, in partial involvement of the 
cervical cord, are mostly increased; only in a complete transverse lesion are 
the reflexes of the legs absent. Wagner and Stolper even maintain that the 
presence of the tendon reflexes in the legs excludes a complete transverse 
lesion. The arm reflexes are usually absent in an inflammation of the cervical 
enlargement and in this condition all of the muscles supplied by the brachial 



TRANSVERSE DIAGNOSIS 417 

plexus are usually flaccid, paralyzed, and atrophic. Sometimes motion is re- 
tained in the legs. Such a condition permits us to conclude that the central 
portions of the cervical cord are especially implicated, while the long tracts 
in the white substance of the spinal cord are spared. 

The condition of the bladder and rectum in cervical myelitis is the same 
as in dorsal myelitis. Occasionally the appearance of glycosuria has been 
reported in literature. Priapism has been repeatedly noted in cervical myelitis 
but may occur in other diseases of the spinal cord situated above the lumbar 
enlargement, in which the center of erection is located. Bramwell refers pria- 
pism to an irritation of the fibers which pass from the brain to the sexual 
reflex center. According to other authors it is to be regarded as a vasomotor 
disturbance. 

Bulbar myelitis (acute bulbar paralysis) is usually not due to very large 
foci, since life is destroyed by very small ones. The paralysis is of the degen- 
erative, atrophic form and implicates the region of the nerve nuclei situated 
in the medulla; it, therefore, attacks (completely or incompletely) the facial 
nerve, the hypoglossal, pneumogastric and spinal accessory nerves, also the 
respiraton r muscles; especially significant is the paralysis of the lips, the 
tongue, the pharynx and the organs of speech (anarthria). The paralysis 
of the pharynx, the threatening involvement of the vital centers of respiration 
(noeud vital, Flourens, 1842) and of the disordered act of deglutition con- 
stantly endanger the life of the patient. 

2. LUMBAR MYELITIS 

The lumbar cord has a length of 5.13 cm. (Liideritz). It begins at the 
top of the 10th thoracic vertebra and is about twice as long as the sacral cord. 
Distally the 5 lumbar segments decrease in size (15.6 mm., 13.0 mm., 10.25 
mm., 7.0 mm., 5.5 mm., according to Liideritz). Myelitis of the lower half 
of the lumbar enlargement (second lumbar segment to the second sacral seg- 
ment) paralyzes the motor and sensory area of innervation of the sciatic nerve, 
especially all of the muscles from the knee downward (excepting the tibialis 
anticus). In diseases of the 4th and 5th lumbar segments, which are situated 
at the height of the 12th thoracic vertebra and the succeeding intervertebral 
disc, besides the symptoms of loss of function in sacral myelitis (foot and toe 
paralysis) the following conditions are noted: paralysis of motion in the hip 
joint in the sense of inward and outward rotation, backward movement of the 
thigh, and flexion at the knee joint. The antagonistic movements (extension 
of the knee and flexion of the hip) are retained in isolated disease of the lowest 
part of the dorsal cord. Peroneal paralysis is particularly severe and pro- 
longed ; the same is true of paralysis of the extensor digitorum communis and 
of the tibialis anticus. Even in the cases of lumbar myelitis terminating in 
recovery a flaccid paralysis of the foot remains; such patients cannot walk 
because they cannot lift the tip of the foot from the ground. 

When the inflammation is distributed to the 3 upper lumbar segments there 
is complete paralysis of all of the muscles of the leg. 

Myelitis of the lumbar enlargement preferably attacks the central gray 
substance; the paralysis is, accordingly, flaccid with degenerative atrophy, 
electric reactions of degeneration, and loss of the reflexes. The paralysis, 



418 MYELITIS 

therefore, resembles the neuritic form; it differs, however, from the latter by 
the segmental arrangement of the motor and sensory symptoms, also by the 
early incontinence of the bladder and rectum, and the frequent presence of bed- 
sores. 

Added to this flaccid, atrophic paralysis there is soon rapid emaciation of 
the legs, paralytic contractures, osteoporosis and rarefaction of the bones. 

In disease of the 3 lowest lumbar segments anesthesia attacks the feet and 
the legs in toto, also the gluteal region, the perineum and striaform zones of 
the skin upon the posterior surface of the thighs, therefore, especially the sciatic 
region. Only in an involvement of the upper half of the lumbar enlargement 
will the area of the crural and obturator nerves be affected. The consequence 
is total motor and sensory paralysis of both legs; the anesthesia involves a 
line drawn through the symphysis, the crests of the ilia and the 5th lumbar ver- 
tebral process. 

In complete destruction of the lumbar cord all the tendon and cutaneous 
reflexes may be absent. The patella tendon reflex can only be present when 
its reflex center in the 2d to the 4th lumbar segments is intact. The cremaster 
reflex, and sensation in the testicles are abolished in disease of the , 2 upper 
lumbar segments. The Achilles and plantar reflexes can only be present when 
the lower lumbar and sacral cords are not affected. The functions of the 
bladder, rectum, and the sexual organs are for the most part paralyzed early 
in lumbar myelitis. 

3. SACRAL MYELITIS 

Myelitis of the sacral cord and of the conus terminalis (this reaches from 
the 3d sacral segment to the filum terminate) occurs particularly after trauma, 
such as falls upon the buttock (conus hemorrhage). All of the 5 sacral seg- 
ments, according to Luderitz, are only 2.11 cm. in length (measured in the 
cadaver of a female, aged 33) ; they are, therefore, shorter than any single 
one of the middle thoracic segments (5.-9.), their length varying between 
2.25-2.4 cm., while the length of a single sacral segment, according to Lude- 
ritz, is only from 0.35-0.42 cm. In the slight transverse and longitudinal 
extent of the sacral cord, which is included within the space of the first lumbar 
vertebra, there can hardly be question of an isolated disease of a single sacral 
segment from a clinical standpoint. We shall, therefore, consider together 
the motor, sensory, and reflex signs of loss of function, in discussing disease 
of the sacral cord. 

Motor symptoms appear only in disease of the second sacral segment and 
those above, and especially involves the ankle joints, while the knee joints are 
usually but little or not at all affected. The free, hanging foot shows the 
picture of peroneal paralysis, but all the other motor functions, dorsal and 
plantar flexion, pronation and supination, as well as extension and flexion of 
the toes, are rendered very difficult. With an involvement of the lowest lum- 
bar segment, the nerve root of which belongs to the sacral plexus, outward and 
inward rotation of the thigh, flexion of the knee joint (semi-muscles, biceps; 
their function should be tested in the lateral position or the abdominal posture) 
may be paretic. The extensor quadriceps, the sartorius, psoas, and the adduc- 
tor group retain their function. With support such patients may still walk, 
but their feet are dragged along like a prothesis, Rising upon the tips of 



TRANSVERSE DIAGNOSIS 419 

the toes, especially raising the external border of the foot, is exceedingly diffi- 
cult on account of peroneal paresis. The paralysis is flaccid, without or only 
with very slight muscle contractions; there is degenerative atrophy with abol- 
ished reflexes and DeR or even with entire loss of electric reactions. 

The sensory disturbance, in case only the last two sacral segments are 
attacked, includes the anus and the anal region. In a lesion of the 3d sacral 
segment the " riding breeches " form of anesthesia is characteristic (buttocks, 
perineum, posterior surface of the thigh). The skin of the scrotum is also 
anesthetic (see Figs. 169 and 170). The testicles retain sensation within the 
anesthetic scrotum, as these organs are innervated by the upper lumbar seg- 
ments. If the disease distributes itself to the 2 upper sacral segments anes- 
thesia of the sole of the foot and of the posterior surface of the leg arises. 
In a complete lesion of the sacral cord the anesthesia implicates the entire 
region of the buttocks, coccyx, the anus, perineum, the external portions of the 
genitalia, the mucous membrane of the rectum, the bladder and urethra (ab- 
sence of strangury, loss of sensation upon catheterization, and upon the pas- 
sage of feces) ; besides the anesthesia is distributed to the posterior side of 
the thigh, to the external surface of the lower leg and to the lateral half 
of the foot. The medial half of the dorsum of the foot and of the sole may 
be hypesthetic in disease of the sacral cord so that the entire foot presents 
motor and sensory paralysis. 

The Achilles tendon reflex and that of the sole of the foot are abolished 
early in sacral myelitis, while the patella and cremaster reflexes may be re- 
tained. Occasionally trophic disturbances develop very rapidly, especially bed- 
sores upon the heel, the ankle, and the sacrum. 

The bladder and rectum, as well as the sexual power, are early paralyzed 
in sacral myelitis. What is true of the sphincter ani is also true of the sphinc- 
ter vaginae, its center being situated in the 3d and 4th sacral segments. The 
centers for erection and ejaculation are situated in the 2d-4th sacral seg- 
ments, usually above those for the bladder and rectum. Therefore in sacral 
cord disease priapism does not occur, as is sometimes noted in affections of the 
cervical cord. 

A case reported by Neumann is remarkable (Virchow's Archiv, 1890, 122. 
Bd., p. 505), that of a man aet. 37 suffering from a stab wound in the left 
half of the sacral cord. The penis was paralyzed according to the Brown- 
Sequard type. Erection was unilateral occurring upon the uninjured side, but 
this side was less sensitive than the opposite. 

The following case (III) is an example of myelitis of the lumbar cord: Karl J., 
aet. 31, married, well until 6 years previously. At that time (1896) he suffered from 
suppuration of the inguinal glands without there being disease of the genitalia. At 
about the same time — without the patient being able to assign a cause — an ulcer 
appeared upon the lower lip and later enlargement of the glands of the neck took place. 
There was no eruption ; systematic inunction treatment. 

His present illness began in December, 1900. with constipation and lancinating pains 
along the course of the sciatic nerve, combined with numbness in the right leg. At that 
time there were, as yet, no disturbances of motion or of the reflexes. The pain dis- 
tributed itself to the right half of the sacrum and attained such a degree of intensity 
that it was relieved only by hypodermic injections of morphin and belladonna. In 
April, 1901, chills were frequent. In May, 1901, paralysis began, first in the toes of the 
right foot. In addition there was severe sciatica even upon the left side, as well as 
retention of urine and cystalgia. The paralysis in the succeeding 4 weeks involved both 




Fig. 169. — Distribution of Tactile and Thermic Anesthesia and Analgesia (Shaded, 
Lines Drawn in Seiffer's Diagram) in a Case of Lttmbrosacral Myelitis, 

420 




Fig. 170. — Posterior View of Fig. 169. 
(For caption see page opposite.) 



421 



422 MYELITIS 

ankle and knee joints. Since that period walking was impossible. The paralysis was 
flaccid, and atrophy of the muscles of the leg soon appeared (June, 1901). The fol- 
lowing 6 months the patient was in the hospital. Systematic use of the catheter, fara- 
dization, galvanism, massage, and another mercurial inunction treatment were without 
success. The pain increased in December, 1901, especially in the left leg, which became 
completely paralyzed. Spa treatment in Wiesbaden brought about no relief and for this 
reason the patient was admitted to the Charite upon July 21, 1902. 

Status prwsens. A powerfully developed man who can only move forward with 
great difficulty supported by two crutches. Internal organs normal as is also the tem- 
perature. Complete flaccid paralysis of the entire musculature of the left leg and of 
the muscles of the knee and ankle joints upon the right side. In the recumbent posture 
only slight flexion and adduction of the right hip joint are possible. The muscles of 
both legs, especially the left quadriceps and gastrocnemius, are very atrophic. The 
circumference of the thigh in the upper third upon the right side amounted to 46 cm., 
upon the left 39 cm. ; in the lower third upon the right 37 cm., upon the left 35 cm. 
The circumference of the calf upon either side was 28 cm. 

Faradic stimulation of the entire left lower extremity, even with maximum currents, 
produced no contraction neither of nerve nor muscle ; only the adductor longus revealed 
a faint contraction. The faradic contractility of the muscles of the right thigh was 
quantitatively diminished but from the knee joint downward it was completely abolished. 

There was no response to galvanism of the entire left limb, with indirect as well as 
with direct stimulation, even with very strong currents. In the right thigh only with 
the strongest currents (40-50 Ma.) with direct, as well as with indirect, applications 
could rapid contractions be obtained. The formula was normal. In the right leg only 
the extensors and the peronei muscles reacted to the same strength of current; the 
muscles of the calf would not contract. 

Sensation in all of its qualities (tactile, temperature, electric, and pain) was totally 
abolished. The loss of sensation was distributed almost symmetrically to the buttocks, 
scrotum, the posterior surface of both legs and to the feet in toto. In the last named 
area the patient complained of spontaneous lancinating pain ( ancesthesia dolorosa ) . 
The urethra was also anesthetic. Retentio urines et alvi. The left leg showed a slighter 
disposition to sweating than the right. 

The reflexes of the sole of the foot as well as the cremaster, of the patella and of 
the Achilles tendon were abolished bilaterally; the arthritic sense was lost upon both 
sides. Lumbar puncture, under low pressure furnished about 10 c.c. of clear fluid; 
microscopic and bacteriologic examination was negative. Bed-sores upon the sacrum. 
The spinal column was not sensitive to pressure. 

X-ray examination revealed 4 osteoporotic foci in the center of the 12th dorsal ver- 
tebra, and the 3 upper lumbar vertebrae, which could be recognized from the flaky, 
white clarification within the vertebral shadow. An X-ray photograph of the left hand 
and foot revealed a distinct difference in the structure of the bones. The bones of the 
hand were normal, while those of the foot showed osteoporotic changes in the bones of 
the paralyzed members. 

The treatment consisted in systematic exercise in the bath, and galvanism with 
strong currents. In addition carbonated baths, exercise with apparatus, sand baths, 
bloodless nerve stretching, injections of corrosive sublimate and iodipin were employed. 
The severe paroxysms of pain in the regions of the sciatic and peronei nerves, and in 
the left tendo Achillis only yielded to hypodermics of morphin. 

Under this treatment, to which the patient submitted for 6 months, the functional 
disturbances improved somewhat — although very slowly. The neuralgic attacks became 
rarer and less intense. The boundaries of the anesthetic zone narrowed somewhat, espe- 
cially upon the buttock and right thigh ; analgesia gave way to hypalgesia, the arthritic 
sense returned. The bed-sore healed. The movements of the hip joint (extension and 
flexion) especially of the right side, increased, and in the right knee joint slight active 
motion appeared. Galvanic contractility improved to the extent that all of the nerves 
and muscles — excepting the left crural and sciatic — reacted to strong currents ( 23 Ma. ) . 

There remained a flaccid, atrophic paralysis of the left knee and ankle muscles and 
of the muscles of the right ankle, complete anesthesia upon the posterior surface of both 
legs and also upon the dorsum and sole of the foot. Orthopedic apparatus (see Fig. 
178) adjusted to the 3 joints enabled the patient to walk in a walking-frame, later 



TRANSVERSE DIAGNOSIS 423 

upon 2 canes, and finally when he was discharged from the hospital (March 24, 1903) 
with one cane, or with support upon one side — although the gait was dragging — but 
nevertheless with surety, even in ascending steps. He has resumed his former occupa- 
tion and has been attending to his duty continuously for 2 years. 

The typical, segmentary, symmetric distribution of sensory disturbance, the 
bladder and rectal symptoms, the absence of the reflexes and the bilateral, flaccid, 
atrophic paralyses in our case present the clinical picture of a subacute-chronic 
disease of the lumbosacral cord. In regard to the difference in the implica- 
tion of the musculature-^-on the right side the region of the sciatic, on the 
left the sciatic and crural, nerves were involved — the focus was situated in 
the motor areas of the cord asymmetrically, and upon the left (to about the 
II lumbar segment) higher than upon the right side (to about the IV. L. S.). 
The distribution of the anesthesia corresponds to a lesion of the 4th lumbar 
segment downward. The history of the case just described, therefore, is that 
of a myelitic focal disease (of syphilitic origin), which had extended from the 
2d lumbar segment to the conus medullaris. 

4. DISEASES OF THE CAUDA EQUINA 

may also give rise to flaccid paralysis of the legs, anesthesia in the distribution 
of the sacral, the pudendal, and coccygeal plexuses and in the areas of the 
sciatic, crural, and obturator nerves. The bladder and rectum may be incon- 
tinent and the sexual power may be lost. A focus that has involved all of 
the nerves of the cauda equina — which pass downward from the 2d lumbar 
segment — may, therefore, produce the same clinical picture as a lumbosacral 
medullary focus. 

The differential diagnosis between a root and medullary lesion is, therefore, 
very difficult in this region. In favor of disease of the cauda equina is the 
unilateral appearance and the limitation of the symptoms of absence of func- 
tion to a nerve or functional area. In the cauda the sensory roots are dor- 
sally, and the motor, ventrally situated; only outside of the dura do they 
unite and form the mixed peripheral nerve. If the peripheral, caudal fila- 
ments are affected then there is paralysis of the crural and obturator nerves 
(paralysis of the adductors and quadriceps, extensor surface of the limb). 
If the deeper and more central fibers are attacked there is then functional dis- 
turbance in the distribution of the sciatic nerve (the foot and flexor surface 
of the knee joint). 

Further, in favor of disease of the cauda — corresponding to the more 
marked development of the sensory caudal roots — is the marked alteration of 
sensation and particularly the predominance of sensory, irritative phenomena: 
paresthesia, radiating pains (root neuralgia). Even analgesia liypermstlietica 
or anaesthesia dolorosa may appear, that is, pain in portions of the body that 
are objectively without sensation. In contrast to this, in medullary lesions, 
motion suffers sooner and to a greater extent than sensation. Only in lesions 
of the anterior root are there muscular twitchings, spasm and finally paralysis 
with rapid, progressive, degenerative atrophy. The destruction of a few roots 
may, however, occur without symptoms, as almost every muscle and every 
cutaneous region is supplied by at least 3 roots. While a lesion of the posterior 
cauda roots is distributed to the entire sensory functional area, in incomplete 



424 MYELITIS 

medullary lesions, sensation may be retained in some or even in all of its 
qualities. Dissociated sensory paralysis, for example, analgesia and thermo- 
anesthesia with retained tactile sense, and also the arrangement of the sensory 
symptoms in symmetric, segmental zones are in favor of the medullary seat 
of the disease. 

Finally we must mention that in trauma the cord is sooner involved than 
the nerve roots which are more resistant and that in the central medullary 
portions, rich in vessels and poor in connective tissue, extravasation of blood 
is more likely to occur than in the white substance. Central myelitis is, there- 
fore, very often more intense than inflammation of the white substance of the 
cord. Partial, transverse disease corresponds to an incomplete paralysis; sen- 
sation may be retained or only disturbed in part, while motion is completely 
abolished. 

ETIOLOGY OF ACUTE MYELITIS 

Spontaneous development of acute myelitis without a demonstrable etiol- 
ogy, as was formerly believed, is now generally rejected. According to recent 
investigations of the spinal cord, especially bacteriologic, and of the cerebro- 
spinal fluid, infections and intoxications play the most important role among 
the etiologic factors in acute myelitis (compare the references of Strumpell 
and Eedlich upon acute myelitis at the 19th Congress on Internal Medicine, 
Verh. d. Congr. 19. Band, 1901). In this disease accordingly there may be 
either a primary entrance of bacteria into the cord, or the lesion is due to the 
action of their toxins, or the effect of chemical or metallic poisons. 

The paraplegias occurring in the course of infectious diseases belong to the 
first group in which the pathogenic agents are found in the myelitic foci. 
Microorganisms have been repeatedly demonstrated in the inflamed spinal cord, 
such as the staphylococcus albus et aureus, streptococci, pneumococci (Fiirst- 
ner), anthrax bacilli (Marinesco), typhoid bacilli (Curschmann), and tubercle 
bacilli (in tuberculous myelitis or in tuberculosis of the spinal cord). 

More often bacteria have been found in the spinal fluid especially pyogenic 
organisms and tubercle bacilli. In the spinal fluid of infectious poliomyelitis, 
Schultze found the Jager-Weichselbaum meningococcus. Myelitis may arise 
after whitlow, as reported by Strumpell, and in this case the staphylococcus 
albus could be demonstrated in the cerebrospinal fluid. 

The microorganisms find their way into the spinal cord partly from the 
central canal, partly from the subarachnoid space (Marinesco), that is, from 
the cerebrospinal fluid, by way of the blood and lymph (Babes). The capil- 
lary spinal cord emboli described by v. Leyden also belong to this category; 
these were noted in the course of ulcerative endocarditis. In acute marginal 
myelitis, in the course of epidemic cerebrospinal fever, Leyden was able to 
demonstrate the point of entrance of the cocci along the septa in the medullary 
substance. 

We must also remark that in general septic processes, bacteria for exam- 
ple, streptococci have been found in the cerebrospinal fluid, without pathologic 
lesion being present in the cord or in the membranes. The bacteria are present 
in the circulation and find their way into the secretions and excretions, as into 
the urine (bacteriuria), without producing changes in the kidney through 



ETIOLOGY OF ACUTE MYELITIS 425 

which they pass. Only upon the demonstration of products of inflammation 
(increase of lymphocytes and leukocytes, intracellular bacteria) is the view 
justified that the spinal cord or its membranes are directly affected. 

It has been possible experimentally by the introduction of bacteria (espe- 
cially streptococci, staphylococci, pneumococci, typhoid bacilli and those 
of diphtheria), into the subarachnoid space of animals or into the circulation, 
to produce meningitic processes. The changes in the ganglion cells are worthy 
of note (clearing and swelling of the cells and their processes, as well as 
atrophy of Xissl's bodies) which were produced experimentally by Goldscheider 
and Flatau in superheated animals. F. Brasch was able to show the same 
lesions in man. with a pre-mortal continuous rise of temperature to over 
105^° F. Langworth Xiehols observed pathologic changes (central and 
peripheral chromatolysis) in the anterior horn cells, as well as in the spinal 
ganglion cells in cases of enteric fever and also in rabbits infected with typhoid 
bacilli ; the chromatic substance in the diseased cells may return to the normal 
condition. 

Myelitis may be a sequel of almost all infectious diseases and all three 
forms may appear: the myelitic focus (transverse paralysis), poliomyelitis 
and not rarely disseminated myelitis, i.e., sclerosis. 

Among the most frequent infectious diseases in which during, or after the 
febrile course has passed, myelitic processes may be observed are variola (West- 
phal, Leyden and others), enteric fever (Salzwedel, Leyden, Ebstein, Cursch- 
mann, Schiff, Lepine) diphtheria, influenza, pneumonia, dysentery, sepsis and 
pyemia, puerperal fever, malaria, erysipelas, gonorrhea ; these are mostly com- 
bined with meningitis (Leyden, Tllmann, Debove, Hayem, Spillmann, Her- 
zog). Angina tonsillaris may, as in the case reported by Hoche. give rise 
to myelitis. As is well known the tonsils are a common port of entrance for 
different infections ; we need only refer to suppurations and tuberculosis, acute 
articular rheumatism, peliosis rheumatica, plague, nephritis, endocarditis and 
influenza. 

Among the infectious diseases just enumerated from which spinal cord 
disease originated, the pathogenic agent could very rarely be demonstrated. 
These negative findings render it very likely, that not alone the bacteria, but 
the toxins produced by them, are the agents which produce inflammation of 
the spinal cord. The spinal cord inflammations, evoked experimentally in 
animals, are in favor of this view. The forms of myelitis produced experi- 
mentally in animals by the introduction of bacteria into the subarachnoid 
space, upon examination often failed to reveal the presence of bacteria; they 
often disappeared in the course of a few days (Marinesco, Hoche) ; on the 
other hand, by the injection of toxins, such as diphtheria toxin (Henriquez 
et Hallion, Crocq), streptococci toxin (Homen), spinal cord inflammation 
was produced. Occasionally myelitis may appear as a primary disease and 
this is favored by the observations of Oppenheim and others. 

Tuberculosis and syphilis (the hereditary and the acquired form) are rela- 
tively frequent causes of myelitis; these diseases being the most important 
ones among the chronic infections. Pochhammer calculates the frequency of 
syphilis in the history of myelitis at 64 per cent. Syphilitic myelitis occurs 
either as primary hyperplastic pachymeningitis or as a chronic cerebral or 
spinal cord disease (encephalomyelitis luetica). In the latter form there is 



426 MYELITIS 

commonly spastic paraplegia, paralysis of the ocular muscles, and of the optic 
nerve, as well as other cerebral symptoms (aphasia). Syphilitic myelitis is 
not always of an inflammatory nature; often there is a gumma of the cord 
or its membranes; further a primary syphilitic endarteritis of the spinal 
vessels is also capable of giving rise to myelitis. Kepeatedly the appearance 
of diffuse myelitis has been noted after an attack of acute poliomyelitis which 
had occurred years previously. This is due to a rekindling of the inflamma- 
tion in the cicatrized anterior horns (W. Hirsch). 

Next to bacterial, chemical and metallic poisons produce inflammatory 
changes in the cord. Thus circumscribed and disseminated, especially chronic, 
forms of myelitis have been noted after lead, chloroform, carbonic oxid and 
illuminating gas poisonings, the last especially in firemen. Here also myelitis, 
due to pellagra, must be included which occurs among the people of Italy and 
South Eussia who eat much maize. 

Of great importance, particularly in regard to accident cases, is myelitis 
occurring after trauma. All injuries and diseases of the vertebral column, espe- 
cially tuberculous spondylitis and carcinoma of the vertebral column, as well 
as scoliosis and kyphosis with marked deformity, may give rise to lesions of 
the cord (compression, hemorrhage, laceration, inflammation). The micro- 
organisms present in the circulation may colonize upon the extravasated masses 
of blood. In addition to these traumatic disturbances, as well as to the ischemic 
necroses of the cord (after embolism, thrombosis, compression), reactive proc- 
esses may occur (round cell infiltration, fatty granules), so that there is no 
difference between the lesions arising in acute myelitis, traumatic myelitis 
and compression myelitis (Ft. Kraus, Zeitschr. f. Min. Med., Bd. XVIII, 
1891, Case I, cervical focal myelitis after fracture of the 5th cervical ver- 
tebra). 

It is important to know that hemorrhage, as well as acute degeneration, 
and inflammation, may arise from shock to the vertebral column without 
injury to the vertebrae (contusion myelitis). The post traumatic development 
of progressive degeneration, such as chronic myelitis, syringomyelia, multiple 
sclerosis, has been repeatedly reported after blows, railroad accidents (railway- 
spine), without there being external signs of injury. Changes develop in the 
cord after some time, months or even years, similar to the condition in late cere- 
bral apoplexies (compare the reports of Stadelmann, Langerhans) which must be 
referred to the progress of the original shock which produced the spinal lesion. 
The investigations of Minor also teach that contusion of the spinal cord may 
result in anatomical lesions in the form of small hemorrhages. If shock is 
capable of tearing a blood vessel, the more sensitive nervous tissue will suffer 
much more readily. 

This is also proven by Schmaus's experiments. He placed a hard rubber 
plate, which he struck repeatedly with a hammer, upon the vertebral column 
of rabbits; finally paresis of the hind legs appeared after which the animals 
were killed and their spinal cords examined. There were found, partly dif- 
fuse, partly system-like degenerations, myelitic softening, hydromyelia and 
gliosis. Schmaus, quite correctly, emphasizes that contusion of the cord not 
only produces lesions in a few vessels, nerve fibers, and ganglion cells, but that 
finer changes may be the result which become obvious only later on, as post-trau- 
matic pathologic conditions. Shock of the spinal cord, therefore, according to 



ETIOLOGY OF ACUTE MYELITIS 427 

Schrnaus, may give rise to traumatic necrosis of the axis cylinders and of the 
cells with arrest of their function, either by " decomposition " of the molecule, 
i. e., a change of position of the individual atoms, or, that from a definite 
strength of the irritation of the mechanical nerve stimulation the nerve fibers 
are not only paralyzed, but from the excess of the stimulation they are de- 
stroyed. This necrosis may, according to Schmaus, produce secondarily, from 
destruction and reactive hyperplastic processes, distinct column degeneration, 
softening, cavity formation and gliosis. Therefore, according to experimental 
and clinico-anatomical facts at hand, the possibility of development of myelitis 
after shock of the vertebral column must be recognized. 

Caisson paralysis, observed in tunnel workers, which may be assigned to 
"internal trauma," may be included with traumatic myelitis. In 1879, 
v. Leyden had an opportunity of observing and reporting such paralyses; in 
the dorsal portion of the cord, especially in the posterior columns and posterior 
lateral columns, he found numerous small foci of softening and referred these 
to tears in the medullary substance, which had arisen from the escape of gas 
bubbles, in consequence of rapid decompression. Recently v. Schrotter, Mager 
and Heller, have published a voluminous book upon caisson paralysis and have 
reported quite a number of such air-pressure paralyses; they refer them to 
ischemic necroses, especially of the white medullary substance. With rapid 
decompression, engorgement of nitrogen gas and extravasation into the blood 
vessels occur, because the lungs are incapable of giving off the nitrogen rapidly 
enough. The extravasation of gas into the blood vessels produces gas embolism 
which has been repeatedly demonstrated in the mesentery, the heart, and the 
central nervous system. This gas embolism of the spinal cord arterioles, ac- 
cording to the previously mentioned investigators, produces multiple focal 
necroses sjonptomatically most often indicated by paraplegia. 

In connection with trauma are those forms of myelitis due to inflammatory 
processes which ascend from the periphery along the cellular tissue or the 
nerve trunks. The development of myelitis from ascending neuritis and poly- 
neuritis has been proven repeatedly, both experimentally and clinically, for 
example, after trauma and erosion of the peripheral nerve trunks. 

Exertion is also noted among the etiologic factors of myelitis; as a rule 
exertion is only a secondary cause as with a preexisting syphilis. Fiirstner 
was able, however, by his " rotary movements " to produce degeneration in both 
lateral and posterior columns in dogs. According to Edinger's ingenious 
" function theory " as well as from his experiments on rats, an immoderate 
employment ("over-exertion") of the nerve tracts may produce such damage 
that atrophy of the nerve substance results and the glia tissue is destroyed. 
He produced experimentally by over-exertion in rats, degeneration of the pos- 
terior columns, such as are characteristic of tabes. 

The etiology of those cases of myelitis is less clear in which refrigeration, 
sexual excess, and great mental emotion are mentioned as causes. Myelitis 
after severe exposure to cold (wetting, effects of snow, etc.) has been demon- 
strated clinically and experimentally in rabbits by forced application of cold 
to the vertebral column. Not infrequently the actions of cold are combined 
with those of exertion or infection. 

Finally myelitis has been reported after psychical effects, especially fright. 
Such cases have been described by v. Leyden in his " KUniJc der BuckenmarTcs- 



428 MYELITIS 

krankheiten," and later in the Zeitschrift fur klinische Medicin, Bd. I, 1879. 
In the latter, there are illustrations of the histologic changes in the cord in a 
case of myelitis due to fright; the patient was a servant girl, aet. 21, who was 
frightened by a fire occurring during the night. A few days later a paralysis 
of the legs developed, which gradually increased in severity, and finally after 
several months, terminated fatally. The histologic examination of the cord 
revealed the picture of chronic myelitis in the middle thoracic cord, especially 
involving the posterior and lateral columns, as well as the posterior horns. 
Similar cases have been reported by Kohts and Brieger. 

The pathogenetic connection between psychical shock and myelitis is not 
easy to explain. Great emotion may produce such an acute vascular contrac- 
tion (vasomotor reflex), that nutritive disturbances arise in the delicate struc- 
ture of the cord. It is further possible that psychical shock causes an intense 
increase of blood pressure, which, with a co-existing arterial disease analogous 
to the condition in the brain, causes a spinal cord apoplexy. Quite often in 
addition to " psychical trauma " other etiologic or predisposing factors can be 
discovered. 

It must still be mentioned that many conditions which debilitate the organ- 
ism predispose to myelitis. Among these are the paraplegias in pernicious 
anemia (Minnich), of leukemia, of carcinomatous cachexia, and of the climac- 
terium. Myelitis has been observed several times during pregnancy and in the 
puerperium; possibly infectious processes also are here concerned. 

Myelitis occurs in both sexes to the same extent. In regard to age, acute 
inflammation of the anterior horns is much more common in children, while 
typical, acute myelitis is more usual during middle age. In the aged, para- 
plegia sometimes appears in connection with incontinence of the bladder. 
These are sometimes due to endarteritic processes of the arterioles of the spinal 
cord, occasionally there may be compression, the result of a kyphoscoliosis or 
of a deforming, anchylosed spondylitis. 

DIFFERENTIAL DIAGNOSIS OF MYELITIS 

We must propound 3 questions in paraplegia : 

(1) What is the etiology and the pathologico-anatomical nature of the 
disease, especially is there an extramedullar process present (tumor, lesion 
of the bone) ? This is the etiologic or pathologic diagnosis. 

(2) Which segments are affected (segmental diagnosis) ? 

(3) Which parts of the segment are involved — poliomyelitis, myelitis 
transversa vel disseminata, leukomyelitis — (transverse section diagnosis) ? 

Topographical diagnosis has been described under segmental localization 
and also under the special forms of myelitis; we shall, therefore, devote our- 
selves to the pathological diagnosis. The clinical picture of myelitis may be 
produced by any transverse lesion of the cord, such as hemorrhage, compression, 
tumor or trauma. Later the differential diagnosis from meningitis, neuritic 
and psychical paralyses must be considered. In regard to the differentiation 
of myelitis from spastic spinal paralysis and from multiple sclerosis, we may 
refer to the chapters upon these diseases in this volume by Professor Eedlich. 

Hematomyelia — hemorrhages into the pia, into the arachnoid, apoplexia 
canalis spinalis, rupture of a cerebral hemorrhage into the ventricles or of 



DIFFERENTIAL DIAGNOSIS OF MYELITIS 429 

an aneurysm into the spinal canal — may have an apoplectiform onset in com- 
mon with acute myelitis. Usually prodromes precede the onset of myelitis for 
some time, which are absent in primary spinal cord hemorrhage. In favor 
of the latter affection are a preceding injury, as well as diseases of the blood 
and of the vessels. The result of lumbar puncture may be of decisive impor- 
tance as when the cerebrospinal fluid is admixed with blood; recent hemor- 
rhages, due to lesion of a dural vessel consequent upon injury during puncture, 
must not be confounded with these. In favor of the clinical picture of hemato- 
myelia, accurately described by L. Minor, in a unilateral affection, is the 
Brown-Sequard type of anesthesia; when both halves of the gray substance, 
as in the lower cervical cord, are implicated, atrophy appears in the arms; 
in the trunk, and in the legs ; simple pareses without atrophy, and dissociated 
sensory paralysis are noted. The pathologic picture of hematomyelia may, 
therefore, resemble central gliomatosis (syringomyelia). 

Paraplegia from embolism of the aorta also appears suddenly; but in this 
condition the vascular disturbances are most prominent — cessation of femoral 
pulsation, ischemic tissue necroses, etc. 

In spinal meningitis the severe pain, the stiffness of the vertebral column, 
tonic tension of the muscles of the trunk and extremities, Kernig's symptom, 
and the fever are prominent in the clinical picture, while the signs of paralysis, 
at least at the onset of the disease, are ill defined. The discoverv of an in- 
creased number of lymphocytes and leukocytes and especially of intracellular 
bacteria in the cerebrospinal fluid is decisive. 

A unilateral paralysis (Brown-Sequard) in consequence of partial medul- 
lary disease, can hardly be confounded with cerebral hemiplegia ; in the latter 
the characteristic bladder, rectal, and trophic disturbances of the cord lesion 
are absent, and, on the other hand, the typical, segmentary, sensory disturb- 
ances only occur in Brown- Sequard's paralysis. 

The differential diagnosis between myelitis and compression of the spinal 
cord may be exceedingly difficult, if, externally neither by inspection, palpa- 
tion nor percussion (local pain, stiffness, deformity), a disease of the vertebra 
can be demonstrated. In disease of the vertebra the history is most impor- 
tant. Thus in young persons who present signs of scrophulosis or tubercu- 
losis in other organs, a tuberculous spondylitis is thought of while in a para- 
plegic woman, in whom a brief time previously a carcinomatous breast has 
been removed, a vertebral metastasis should be thought of, especially if intense 
pains are present. Sometimes the vibration of the tuning fork is experienced 
as pain over the diseased vertebra. In caries of the vertebra in children, 
according to Finkler, in about one-half the cases the local pressure pain is 
absent ; the lumbar vertebrae are most often affected by caries. The first symp- 
toms of medullary compression are often pareses and root neuralgias. The 
sensitiveness to pressure of the sacral vertebrae upon palpation through the 
rectum is also in favor of an extramedullary seat of the disease, particularly 
if a mass can be felt (tumor, callus). 

Of great significance is the X-ray finding in regard to differential diag- 
nosis. Leyden and Grunmach x several years ago published a number of radio- 

i " Die Ront^enstrahlen im Dienste der Riiekenmarkskrankheiten " Arch. f. Psy- 
chiatric, Bd. XXXVII, Heft 1 (1903). 



430 MYELITIS 

scopic examinations in disease of the spinal cord. These proved that even 
slight changes in the bones and displacements of the vertebrae, which were 
impossible to demonstrate by means of former methods, are revealed with sur- 
prising clearness by radioscopy. On the other hand primary medullary dis- 
eases could not be recognized by the X-ray with sufficient clearness. But the 
negative X-ray finding is also of value, as it renders it extremely likely that 
the myelitic disease is independent of an affection of the vertebrae. Grunmach 
observed, in affections of the transverse section of the cord, an osteoporosis of 
the vertebrae and of the members downward from the focus, corresponding to 
the distribution of the lesion; he believed, therefore, that it was possible to 
determine, from the distribution of the osteoporosis, the height of the lesion 
in the cord. Radioscopy of the paralyzed limbs is also important in so far 
as the abnormal shadow of the bone indicates osteoporosis, that is, the ana- 
tomical nature of the malady, and indicates also the danger of a spontaneous 
fracture. Osteoporotic bones are often anesthetic (test by means of vibra- 
tion of the tuning fork). 

If there be suspicion of medullary compression, in consequence of tubercu- 
lous spondylitis, when pain upon pressure, stiffness, and deformity of the 
vertebral column are absent (Pott's curvature), the diagnosis may be made 
by the demonstration of tuberculosis in other organs (lungs, lymph glands), 
further by the presence of tubercle bacilli in the cerebrospinal fluid (pachy- 
meningitis tuberculosa) , or by the positive result of the tuberculin reaction, 
provided there is no other tuberculous focus present. 

Difficult and even impossible at times is the differentiation of myelitis 
from an intraspinal tumor. In favor of an extramedullary seat of a tumor 
are the signs of compression of the nerve roots ; these consist of intense neural- 
gic pains (intercostal neuralgia, sciatica), which are commonly unilateral and 
only later, when the tumor presses upon the other half of the cord, also impli- 
cate the other side. At the onset motor, usually spastic paralysis is limited 
to one side of the body. The objective sensory disturbances are not so well 
defined in compression of the cord as in a primary transverse disease. In 
addition there is sometimes sensitiveness of the vertebral column to palpation 
and vibration. The slow progression of the paralysis to the opposite side while 
the localization in height remains the same, except when there are multiple 
tumors, which may then produce the clinical picture of multiple sclerosis, is 
characteristic of an extramedullary tumor. 

An intramedullary seat of the tumor, according to its extent, is favored 
by the symptom-picture of a unilateral lesion or complete transverse paralysis ; 
further its location in the cervical cord where primary myelitis is much rarer. 
With an intramedullary seat of the tumor pain may be entirely absent (com- 
pare the article by Professor Schultze in this volume). 

The presence of particles of the tumor in the cerebrospinal fluid will ren- 
der the diagnosis positive. Also in meningomyelitis of syphilitic origin, as 
well as in pachymeningitis gummosa the results of lumbar puncture may be 
of importance. The presence of numerous lymphocytes in the cerebrospinal 
fluid is greatly in favor of the syphilitic nature of the disease. 

Landry's paralysis may give rise to confusion. This affection may appear 
in the form of an acute polyneuritis or as an acute degeneration of the motor 
cells in the anterior horns and medulla oblongata (bulbar variety). An acute 



DIFFERENTIAL DIAGNOSIS OF MYELITIS 431 

ascending, occasionally also descending paralysis, of all nerve tracts is char- 
acteristic. This commonly develops with fever, beginning with complete, 
tiaccicl paralysis of the legs, which, in a few hours or days, involves the muscles 
of the trunk, the arms, the nape, the face, and finally the muscles of speech, 
of deglutition and of respiration. The paralysis is flaccid often combined 
with DeR; the tendon reflexes are abolished. Pain and sensory disturbances 
may be absent; the functions of the bladder and rectum are normal. Some- 
times the symptoms of a general infection are still present — fever, enlarge- 
ment of the spleen, albuminuria. Death results in a few days or weeks from 
paralysis of respiration (Cheyne- Stokes' type), or the paralysis improves; 
recovery has several times been reported. 

In the differential diagnosis the decision of the question is very important, 
in the given case, as to whether the paraplegia is due to a disease of the cord 
(myelitis) or to a peripheral affection (multiple neuritis, polyneuritis). The 
latter malady was formerly included with spinal paralysis. In my (Leyden) 
various publications (Zeitschr. f. klin. Med., Bd. I, 1880, and also in the 
Deutsche militararztliche Zeitschrift, 1888: Die Entziindung der peripheren 
Xerven: Polyneuritis — Xeuritis multiplex) I described the clinical differences 
of neuritic and spinal paralyses and the clinical picture of polyneuritis. 

In favor of a disease of the peripheral nerve trunk or of the root within 
or without the dura, are marked sensory symptoms with decided irritative phe- 
nomena, paresthesia, pressure points, lightning pains, root neuralgias. Typical 
of a lesion of the posterior roots is analgesia hypcrastlictica, that is, anaesthesia 
dolorosa, with absent motor disturbances. The interruption of the posterior 
roots on the one hand causes an arrest in the conduction from the periphery 
to the center, and on the other hand the irritation of the lesion in the center 
of the roots causes pain, which, in regard to its development, may be com- 
pared to the pain projection in amputated members. In medullary disease, 
however, motion suffers sooner and to a greater client and it may even be 
involved alone, with sensation completely retained. Further, in a medullary 
disease, the paralysis is distributed to the entire area below the point of lesion ; 
thus in an affection of the cervical cord to all four members, the trunk, the 
bladder and the rectum. Paralysis below the lesion is commonly rigid with 
increased tendon reflexes and contractures. 

In contrast to this, a disease of the roots, for example, those passing out 
of the cervical enlargement, gives rise to disturbances exclusively within the 
region of distribution of the affected roots, such as a flaccid paralysis and neu- 
ralgia in the arm, while the trunk and the legs are not involved. In an incom- 
plete medullary disease, only a portion of the member may be attacked. In 
this condition, however, in an anterior horn affection, motion is lost and sen- 
sation retained or in an incomplete affection of the posterior column there is 
dissociated sensory paralysis, as the spinal cord fibers which supply the various 
qualities of sensation run in tracts separated from one another. For this rea- 
son dissociated sensory paralysis, for example, analgesia and thermoanesthesia 
with retained tactile sense, as well as the presence of sensory disturbances in 
definite zones, is characteristic of an incomplete medullary disease. In con- 
trast to this the sensory disturbances in peripheral disease involve all sensory 
qualities and extend to a definite cutaneous area. As is well known, in dis- 
ease of a peripheral nerve, for example, in alcoholic neuritis, degenerative 



432 MYELITIS 

atrophy, motor paralysis, DeK, and the abolished reflexes are limited to the 
distribution of the affected nerve trunk, while myelitic paralysis does not con- 
fine itself to the area of innervation of the peripheral nerve. 

In a complete transverse disease of the cord the reflexes may also be absent ; 
but then the other symptoms of transverse disease are unmistakable, especially 
the bladder and rectal disturbances which are only exceptionally affected in a 
pure neuritic or polyneuritic lesion. The arrangement of unilateral motor, and 
contralateral sensory, paralysis ( Brown- Sequard), further the appearance of 
priapism and the demonstration of extramedullary diseases (spondylitis, 
tumor) also favor the central seat of the paralysis. The differential diagnosis 
between myelitis and disease of the cauda equina has been detailed elsewhere 
(see page 423). We must also refer to the article in this volume by Professor 
Bernhardt upon paralysis of the peripheral nerves. 

Confusion of myelitic, with hysterical, paralysis has occurred several times. 
In favor of the psychic nature of the paralysis is the sudden appearance of the 
paralysis after emotion, the presence of other hysterical stigmata, the typical 
distribution of the hysterical sensory alterations, the absence of incontinence 
of the sphincters as well as of other objective paralytic phenomena (degenera- 
tive atrophy, DeR), the relatively rapid change in the intensity of the paral- 
ysis, the disappearance of the paralysis during sleep and under anesthetics, 
negative Babinshi reflex and the normal composition of the cerebrospinal fluid. 

PROGNOSIS OF MYELITIS 

The function necessary to maintain life (respiration, heart, deglutition) 
are usually not involved in myelitis. Nor is the pain so intense that it exhausts 
the strength of the patient. On the other hand, in the course of the first 
or second week, complications on the part of the bladder and rectum may arise, 
which from their sequels, cystopyelitis and bed-sores, seriously endanger life. 
To this there is often added psychical depression and physical exhaustion 
(insomnia, deficient ingestion of food, cardiac asthenia), which according to 
their intensity increase the anxiety of the physician. The prognosis of myelitis, 
therefore, quoad vitam et sanationem is always serious. 

Prognosis depends particularly upon (1) the etiology, (2) the course, 
(3) the distribution and intensity of the myelitic paralysis, that is, upon the 
size and seat of the focus, (4) upon complications. 

In an etiologic respect compression myelitis in spondylitis tuberculosa, in 
caisson paralysis and in myelitis after syphilis or acute infectious diseases, 
offers a more favorable prognosis than other varieties. 

In regard to the course the prognosis is naturally more serious in a rapid, 
progressive paralysis. The more rapid the progress the slower is the stage 
of regression. The conditions are more favorable as to prognosis when the 
signs of paralysis are incomplete, bladder and rectal symptoms are absent and 
no serious complications (bed-sores, cystitis) are present. 

In the severe cases of myelitis, only when regeneration begins, is there a 
hope of improvement, i. e., only when the paralytic phenomena improve, the 
abolished reflexes return, the flaccid paralysis assumes a spastic character, the 
bladder and rectal symptoms ameliorate, and the bed-sore shows a tendency 
to heal. The muscular regeneration is shown by an improvement in the 



THERAPY OF MYELITIS 433 

electric reaction and only in this stage of regeneration is the danger of life- 
threatening complications less and the hope of a more or less complete recov- 
ery established. 

In the stage of restitution we must remember that in organic disease of 
the cord a restitutio ad integrum does not occur and that improvement is but 
very gradual. In the mild cases it takes many weeks even in the favorable ones, 
and in severe cases many months. It must also be remembered that just as 
in subacute and chronic inflammations of other organs so also in myelitis 
there are temporary stages of arrest and even apparent improvement, until 
spontaneously or from some special cause (refrigeration, exertion, febrile in- 
fection) the apparently dormant inflammatory process flares up again; it 
then destroys the effects of improvement and brings about even a more severe 
paralysis. This relapsing form of the inflammation may produce several acute 
relapses (myelite a rechutes). 

In regard to the localization of the focus, cervical myelitis presents the 
most serious prognosis, especially when the process ascends to the medulla 
(bulbar paralysis). The larger the focus the more serious the prognosis. The 
involvement of the sensory qualities favors a more intense disease of the cord. 
Small foci may heal without producing marked functional disturbance. For 
this reason disseminated myelitis, which usually presents small foci, and uni- 
lateral lesions, offers a more favorable prognosis than transverse myelitis 
which is associated with large foci. Extensive disease of the anterior horns, 
that is, degenerative muscular atrophy with electric DeR due to it, as well 
as complete, flaccid paralysis or serious contractures, are of unfavorable import. 
The prognosis of myelitis is rendered more grave by general complications 
(old age, debilitated constitution, diseases of the heart or lungs, cachexia, 
etc.), further by unfavorable external conditions of life. 

When the symptoms of paralysis have reached a definite stage of arrest 
(stationary stage) then with suitable care the patient may live for many 
years. It may be stated that in general the prognosis of myelitis, thanks to 
the advance in early diagnosis and the recognition of the advantages of physi- 
cal treatment including proper nursing, has improved very much. In the 
course of the last few years we have repeatedly succeeded in putting many 
a myelitic in the position of earning his livelihood. 



THERAPY OF MYELITIS 

(1) PROPHYLAXIS 

The prophylaxis of myelitis consists in the avoidance of the etiologic fac- 
tors. Immunization acts in this sense as in the case of diphtheria, hydro- 
phobia (protective inoculations) and in malaria (the administration of 
quinin). Caisson paralysis may be prevented by prophylaxis and therapeutic 
measures, by sufficient supply of air, and ventilation of the working cham- 
bers, as well as by other means. L T pon the first signs of caisson paralysis the 
patient is put back into the chamber, where, by the introduction of compressed 
air, the pressure of the air is increased to the degree in which the patient 
had labored in the caisson. The introduction of the compressed air, accord- 
ing to v. Schrotter, should be gradual, two minutes for every fifteen pounds 



434 MYELITIS 

of atmospheric pressure; besides, oxygen is to be administered. Inhalation 
of oxygen often relieves the disorder of the pulmonary circulation and of res- 
piration; it indirectly increases the excretion of free nitrogen from the blood 
vessels. The patient remains in the " artificial caisson " until the serious 
symptoms have disappeared, after which the atmospheric pressure is gradually 
decreased. Since the introduction of State regulations (in Germany) in 
caisson working (tunnels, bridge building) and of careful observation of the 
prophylactic measures, caisson paralysis has become exceedingly rare. 

First aid, in injuries to the vertebra, is most important in a prophylactic 
respect, such as expert ambulance service and proper surgical treatment 
(extension). In a similar manner an early rational treatment of spondylitis 
is effective, that is, before pressure symptoms on the part of the cord appear. 

The avoidance of the predisposing causes of myelitis acts in the same sense, 
especially in neuropathically predisposed persons; also in syphilitics or alco- 
holics (avoidance of immoderate indulgence in various sports, wetting, intox- 
ications, such as lead, alcohol, etc.). 

It may, therefore, be observed that the physician can do much in the 
prevention of myelitis. 

(2) TREATMENT 

In attempting the treatment of myelitis it is well to remember and also 
to inform the patient that the disease is a very protracted one, lasting months 
and not infrequently years. 

The anatomical process can rarely be influenced directly, for example, 
by specific treatment in syphilitic myelitis, or by relieving the compression, as 
in pressure myelitis. In most of the cases our aid is limited to improving 
the functional disturbances, in facilitating the efforts of healing by proper 
and careful nursing, which will maintain the strength of the patient and 
prevent serious complications. The last named, the psychical and physical 
exhaustion, bed-sores and cystitis, and not the disease of the cord itself, are 
the cause of the fatal issue in myelitis. For this reason the main object of 
the physician consists in a prophylactic treatment which will prevent these 
serious complications, by a proper control of the mental state of the patient 
and by careful nursing. Leyden has made the apparently paradoxical state- 
ment that the recovery of the patient often depends upon the fact that we 
keep him alive, i. e., so that he does not perish from complications. Among 
these, which may often be avoided by proper treatment, is inanition; this is 
especially true of such a conspicuously chronic disease as myelitis. In a con- 
siderable number of paraplegias, who terminate fatally, hyponutrition is the 
cause of exhaustion and such patients then succumb to complications or inter- 
current febrile affections. 

The food of the patient may be adjusted to his wishes, in so far as this is 
possible, for, except in the case of intestinal crises, atony of the intestine, and 
incontinence of the sphincters (limitation of fluid) there are no special indi- 
cations. The daily amount of food should not be below 2,000 calories, espe- 
cially during convalescence, for during this time treatment by systematic 
exercise demands increased exertion on the part of the patient. It is service- 
able to vary the diet as much as possible and to increase the ingestion of food 
by the addition of substances rich in calories, such as honey, malt extract, 



THERAPY OF MYELITIS 435 

or cream (1 liter = 1,920 calories). Fats which are very rich in calories 
are advantageously given with vegetables (mashed potatoes), milk and rice, 
or bread. Alcohol had better be limited especially during the acute stage. 
Naturally an immoderate gain in weight, which renders the restitution of 
motion difficult, should be prevented. 

A difficult and exceedingly important object in the treatment of myelitis 
is the patient's posture in bed especially in regard to the prevention of bed- 
sores. This develops upon those portions of the body that are exposed to pro- 
longed pressure, which in combination with paralysis of motion, of sensation, 
and of the vessels (vasoplegia) leads to disturbances of the cutaneous circula- 
tion; these may be readily recognized from the bluish-red discoloration. Fur- 
ther there is transudation, formation of vesicles, hemorrhage, and even necro- 
sis. The patient is greatly debilitated by the bed-sore; often enough in 
consequence of complications arising from it (erysipelas, sepsis, embolism, 
gangrene, rapidly progressing phlegmons) it is the cause of death. The preven- 
tion of the bed-sore is, therefore, a vital question to the patient, and in treat- 
ment it is a matter of conscience to the physician; it is true when there is 
incontinence of the bladder and rectum this is one of the most difficult prob- 
lems of nursing. 

Bed-sores are prevented by a suitable position in bed and cleanliness, pro- 
vided that the strength of the body is maintained by suitable nourishment. 
It is all the more necessary to maintain the power of the heart, for cyanosis, 
that is, stasis hyperemia, favors the development of the decubitus. 

The bed of the myelitic, which, often for months and years, forms his place 
of rest, should be placed in a room in which there is sunshine, and at the same 
time is sufficiently large and airy. The patient should rest comfortably in bed 
and all circumscribed, constant pressure must be avoided. As flat a position 
as possible, with a slightly raised position for the head, distributes the pres- 
sure over a large surface and thereby prevents circumscribed pressure points. 
The bed may be either an air, or water bed; this should only be one-half 
filled or at most two-thirds, so as to render it more pliable ; the bed sheets must 
be dry and without folds. Patients with incontinence should be placed upon 
some impermeable material. The pressure of the bed clothes upon the dorsum 
of the foot, and the tendency to the formation of pes equinus is prevented 
by a loop arrangement over the feet upon which the bed clothes rest; a cush- 
ion filled with chaff may be placed at the foot of the bed to support the feet. 
A change of position in bed or even removal to another bed is a pleasant 
change for the patient whose mood is liable to be exceedingly depressed by 
being compelled to assume the dorsal decubitus for a long time. 

When cystitis has appeared it is of advantage to raise the upper end of the 
bed. The inclined position favors the off-flow of urine and diminishes the 
danger of an ascent of the cystitis to the ureters. For this purpose some of 
the special invalid's beds may be used or the head of the bed may be raised 
about 15 cm. by wooden blocks. The patient soon accustoms himself to the 
change in position. 

The endangered areas of the body are placed upon air pillows (the center 

being hollow so that there is no pressure) and dusted with a zinc powder. 

Placing the patient upon elastic felt, in which the pressure points are cut out, 

has been advised. By means of gentle massage and the inunction of alcohol, 

29 



436 MYELITIS 

wine of camphor, or lemon juice, an attempt may be made to improve the 
circulation and the tonus of the skin; painting with ichthyol-collodium (five 
per cent.) prevents infection of the skin. After the voidance of urine and 
feces special cleanliness is necessary. Washing with an antiseptic soap is 
advisable. The bed-sore may be best avoided by systematic sitz- or full baths 
with subsequent sponging of the endangered areas with alcohol. 

Much more difficult than the prevention is the treatment of a bed-sore that 
has already formed. It should not have plasters applied as the secretions are 
retained by this means. Among local applications hydrogen dioxid has proven 
most useful in our hands. The demarcation of the ulcer and the desquama- 
tion of the necrotic eschar proceed rapidly upon the daily use of this agent. 
When the borders of the ulcer are inflamed a moist dressing of aluminum 
acetate or 70 per cent, alcohol should be used, otherwise a dry dressing of 
xeroform or iodoform gauze; when there is much secretion, bismuth gauze 
should be employed. Undermined margins, as well as fistulae, are to be divided 
early to prevent the retention of secretions. Gangrenous bed-sores are best 
treated with warm sitz- or full baths (35° C.) lasting several hours; in the 
severest cases the permanent water-bed, advised by Hebra, may be employed. 
Putrid ulcers are to be deodorized with iodoform and charcoal in equal parts. 
Indolent granulations are treated with hydrogen dioxid or with a 1 per cent, 
caustic salve, but the newly formed margin of epidermis, at the border of the 
ulcer, should be avoided. The clean granulating base of the ulcer should have 
an indifferent salve applied (zinc salve, or boric acid and lanolin). Lately 
sunlight, electricity and the X-ray have been used in the treatment of ulcer. 

No less difficult is the treatment of the cystitis. In spite of the strictest 
antisepsis in catheterization, especially in women, cystitis is a very common 
complication of paralysis of the bladder. Proper evacuation of the bladder, 
catheterization with all antiseptic precautions prevents retention of urine. 
Bladder washings and urinary antiseptics prevent decomposition of the urine; 
a high position of the upper end of the bed stops the ascent of the catarrh 
of the bladder to the renal pelvis. Double tube catheters are employed for 
these washings and warm solutions at 30° C. (2 per cent, boric acid, rose red 
potassium permanganate solution, -J per cent, ichthyol, 1-1,000 silver nitrate) ; 
in putrid cystitis solutions of -|-1 per cent, hydrogen dioxid ; finally permanent 
bladder drainage with the Nelaton catheter. If the urine is alkaline we admin- 
ister benzoic acid by the mouth, or salol, urotropin, helmitol ; further, teas of 
herba herniarise or folia uvse ursi, or mineral waters of various kinds (Wil- 
dungen, Biliner, etc.). On account of the prolonged character of the disease 
we must constantly change these remedies. 

When there is incontinence of the bladder a urinal is placed in front of the 
patient or a permanent catheter is introduced into the bladder. Further, in 
this condition massage of the bladder, by means of suitable appliances with 
the aid of electricity (vibration apparatus), has been employed with success. 
This bladder vibration is to be practised with the bladder empty; the semi- 
globular electrode is pressed above the symphysis or upon the perineum, toward 
the bladder. In women the bladder may be caused to vibrate from the anterior 
vaginal wall. The duration of the vibration treatment, which is practiced 
daily once or twice, should be about 5 minutes. In nearly all cases this method 
produces a favorable influence upon the incontinence. In the same manner 



THERAPY OF MYELITIS 437 

faradization of the bladder, that is, of the sphincter urethras may be attempted 
(with a sound electrode), the patient being encouraged to contract the bladder 
with the entrance of the current. 

The method of nursing, including the patient's position in bed, and the 
diet, are to be maintained during the entire acute course. Only some weeks 
after this stage has run its course may an attempt be made, with great cau- 
tion, to place the patient in an easy chair, provided that this does not exhaust 
him too much. Change of position often immediately alters the mental atti- 
tude of the patient and thereby favorably influences the other functions of the 
body; appetite, ingestion of food, sleep, and digestion. 

It is the duty of the physician to strive in elevating the psychical condi- 
tion of the myelitic, especially in desperate cases (elever le physique et le 
moral). 

If the question of posture is so extremely important in uncomplicated 
myelitis, its solution represents the chief indication in the treatment of com- 
pression of the cord, as for example in caries of the vertebra. The vertebral 
column, on the one hand, must be kept at rest and, on the other hand, must 
be relieved of weight. For this purpose, formerly circular plaster casts were 
employed, which were applied to the patient while in an extended position, 
and, in caries of one of the upper thoracic vertebra, reached from the head to 
the pelvis. This process kept the vertebral column quiet, but it had great 
disadvantages, for often pressure areas and eczema were produced and the sen- 
sation of constriction, particularly in the hot seasons, was almost unbearable. 
Bonnet's wire basket or Eauchfuss's suspensory apparatus, with an accurately 
adjusted posterior piece, is much more suitable. 

We prefer extension and suspension treatment upon an inclined bed, that 
is, in the extension bed. The avoidance of pressure is brought about with 
Glisson's head apparatus and the oblique position of the body aids in exten- 
sion. It is well to begin with mild extension (twice daily for 10 minutes) 
which may be increased gradually to several hours daily. By this means the 
vertebral column may be lengthened 2-4 cm. 

After the severe symptoms of compression have been relieved, we employ 
supportive and extension corsets (of celluloid, leather, some strong material 
or sailcloth with steel braces (Hessing) or of light and flexible felt) which 
may be modelled upon the trunk of the patient. If the focus is situated in 
the cervical vertebrae, an apparatus constructed of pasteboard is sufficient; 
an appliance which extends the neck between the occiput and chin on the one 
hand, and, the clavicle and the shoulder on the other hand, keeping the parts 
at rest. In caries of the thoracic and lumbar vertebras extension is obtained 
by a jury mast and a few steel braces, which reach from the axilla to the crest 
of the ilium. 

We must mention that in tuberculous spondylitis, sapo Jcalinus venalis 
transparens, in inunction, has been in use for a long time. Two or three times 
weekly about 30 grams are rubbed in similar to the inunction treatment in 
syphilis, the patient having a bath one hour later. 

In paralysis due to pressure, operations have been attempted to remove 
the cause — exudates, tumor, fungoid proliferations, caries of the bones. It 
may be stated that in general the operative treatment of pressure myelitis has 
not been satisfactory, particularly as the pathologic focus is usually situated 



438 MYELITIS 

in the body of the vertebra. It is, therefore, hardly possible completely to 
remove the affected parts, without risking a secondary fracture of the verte- 
bras with compression of the cord. Only in disease of the posterior portions 
of the vertebral arches, or of the transverse arches does laminectomy come 
into question, with a possibility of covering the defect by a celluloid plate 
to close the cavity in the vertebral column. " Redressement " of the spondylitic 
deformity, described 10 years ago by Calot, has been generally abandoned, 
particularly in the original manner, for injuries to the cord and even fatalities 
have resulted from this treatment. 

We must now discuss the treatment of the irritative symptoms which prob- 
ably no myelitic escapes; these represent the most distressing features of the 
disease. This is especially true of the sensory irritative symptoms, the pares- 
thesia, the neuralgic or continuous pains in the vertebral column, or in the 
nerve trunks. The anatomical cause of the pain is due to an inflammation 
of the membranes of the cord (intradural exudate, meningitic induration) 
or to an inflammatory irritation of the posterior roots (rhizitis posterior). 
With the exception of gummatous meningomyelitis, it is impossible to influ- 
ence the local lesion ; we must, therefore, aim to diminish the pain by decreas- 
ing irritability with the aid of local or internal remedies. In acute meningo- 
myelitis an ice bag, rubber or metal tubes (Chapmann's tubes) are applied to 
the spinal column; cold water is permitted to circulate through these. They 
may remain in situ until the pain has subsided. Former treatment by means 
of the red hot iron has quite j:>roperly been abandoned. Milder cutaneous 
irritants have been substituted, which often have an anodyne effect : faradiza- 
tion at the point of pain, sinapisms, liniments, blisters, massage, applications 
of hot air, etc. Painting with tincture of iodin or dry cups along the vertebral 
column, as well as the administration of laxatives, often decrease the neuralgic 
pains of the myelitic. 

Among drags the various antineuralgics and sedatives are useful : aspirin, 
phenacetin, the salicylates, quinin, caffein, pyramidon, antipyrin (also 0.1-0.2 
subcutaneously), the bromids and veronal. The sovereign remedy, however, 
is morphin hypodermically ; but caution must be observed so that the patient 
does not become addicted to its use. Injections of cocain, eucain, antipyrin, 
stovain, Schleich's solution, and iodipin may be used in the vicinity of the 
painful nerve trunk. In desperate cases the painful nerve trunks are exposed 
and stretched or anesthetized (touched with osmic- or carbolic acid), even 
neurotomized. 

The motor irritative symptoms — hypertonia, muscular contractions, spasms, 
and contractures — are no less difficult to manage. The best remedy to combat 
spasm is absolute rest and the avoidance of all reflex irritation. To avoid 
pressure on the part of the bed clothes, the spasmodic members have a rest 
placed over them, the soles of the feet are supported by a soft pillow (filled 
with any soft material). Protracted luke-warm baths (33° C.) or luke-warm 
sand baths, or enveloping the patient in hot compresses and sometimes splint- 
ing the extremity are of service. 

Stabile anodal galvanization has a sedative effect ascribed to it. In this 
treatment a large anodal plate is placed over the vertebral column or better 
upon the irritative points of the muscles and nerves, and only weak currents 
(1-2 Ma. are used; these are gradually increased and decreased. This ano- 



THERAPY OF MYELITIS 439 

dyne effect, which also decreases irritation, is not to be obtained by Volta's 
alternatives or any other method ; it may be continued for one hour. For this 
purpose the electrodes are attached to the part-. 

With greater certainty and more rapidly than galvanization with the anode, 
is the action of drug sedatives, which in general decrease reflex irritability and 
irritation transmitted from the cerebral cortex. Among these are chiefly the 
bromids, chloral hydrate, and atropin; the last named also being valuable in 
intestinal colic and spasm of the bladder; also curare which paralyzes the 
terminal nerve filaments. It is best administered subcutaneously for the pain- 
ful clonic contractions (1 c.c.) ; nitroglycerin ("2-3 tablets gr. y-J-g- daily) which 
is of use in angiospastic conditions, is useful for the muscle spasms. 

In desperate cases we have employed lumbar infusions of cocain, eucain, 
or stovain-adrenalin (Bier), and have obtained analgesia and flaccidity of the 
muscles lasting several hours. In general we must advise against repeated 
infusions into the cerebrospinal canal for the}' sometimes give rise to very 
disagreeable reactive phenomena (fever, chills, severe headache), and espe- 
cially as the relief of pain lasts but a few hours. Besides the introduction 
of drugs into the spinal canal, when the cord is already in the state of irrita- 
tive inflammation, appears to us to lie a more serious measure than when the 
parts are normal. 

In general the drug treatment of myelitis does not play a great role apart 
from the specific treatment in syphilitic disease of the cord. Under these 
circumstances we prefer the hypodermic employment of iodin and mercury, 
the former given in the form of iodopin (25 per cent.) 5-25 c.c. every other 
day. Mercury we administer as corrosive sublimate injected into the muscles 
or as hydrarg. succinimid.. eucain, aa .<>1 for an injection (this is painless and 
may be given every second day). In this manner 20-30 injections should be 
given. Occasionally, however, even energetic antisvphilitic treatment is with- 
out effect in luetic spinal disease; even dural injections of sodium iodid we 
have found to be ineffective. 

Etiologic treatment, serum therapy, has up to this time shown no direct 
results, even in the case of post diphtheritic myelitis. In post infectious 
myelitis, Oppenheim advises diaphoretic treatment combined with the admin- 
istration of the salicylates. 

The use of silver nitrate or strychnin has rarely been followed by good 
results. Strychnin is employed in paralysis of the sphincters, in anesthesia, 
and in flaccid paralysis. By stimulation of the reflex centers it increases the 
excitability of the motor nerves in the paralyzed muscles and in combination 
with electric and gymnastic measures it favors the restitution of the muscles. 
Strychnin is best employed in solution 1-1,000, best subcutaneously 1-5 
mgms. daily. 

Other indications, in the course of myelitis, sometimes require the employ- 
ment of drugs, such as analeptics, tonics, stomachics, laxatives, etc. ; these are 
prescribed according to well established therapeutic principles. In general 
the value of drugs, even in the most favorable case, is purely symptomatic; 
physical curative methods are quite correctly substituted for them. 

Tlie physico-mechanical treatment of myelitic paraplegia must fulfill the 
following objects: 

Prevention or relief of muscle atrophy and contractures; the most thor- 



440 MYELITIS 

ough restitution of motion by the compensatory education of retained muscle 
functions (exercise, etc.) or by apparatus, that is orthopedic-surgical measures. 

Before proceeding to employ these methods one question must be answered 
which is of fundamental importance in the physical therapy of myelitis: In 
how far is there an anatomical possibility of restitution in myelitis? If the 
inflammation has destroyed the entire transverse section of the cord naturally 
there can be no question of a restitution of motion. On the other hand in 
partial, insular foci a certain functional rehabilitation may take place. The 
unaffected half of the cord may, to a certain degree, function vicariously for 
the diseased portion. Turner showed experimentally (Brain, 1891), that 
even with a unilateral section of the cord almost complete functional activity 
may occur. Kocher has reported almost complete recovery in patients suffer- 
ing from unilateral lesions of the cord. In our case of Brown- Sequard's paral- 
ysis, following a stab in the 2d dorsal segment, sensation was abolished upon 
the contralateral side, but motion returned to the extent that the patient was 
enabled to walk without aid, almost like a normal person. 

Regeneration does not take place at the point of lesion, on the contrary, 
ascending and descending degeneration here occurs, but in the surrounding 
of the pathologic focus the compensator}^ function of the retained tracts of 
conduction substitute for those that have been destroyed. If the main tract of 
motion is destroyed, the pyramidal lateral column tract, upon one side, the 
contralateral pyramidal tract and also the extra-pyramidal motor tracts 
(v. Monakow's rubrospinal tract, the corpora quadrigemina anterior column 
tract) may act alone, and by suitable exercise may become substitution tracts 
(compare also P. Lazarus, Die Bahnungstherapie der Hemiplegie, Zeitschr. 
f. Min. Med., 45. Band, 1902). 

Less clear is the special localization of the sensory spinal cord tracts ; some 
qualities of sensation, such as deep sensation, are innervated from both halves 
of the cord, so that the normal side of the cord may act for the diseased por- 
tion to a certain extent. The bladder and rectal tracts are also to be regarded 
as bilateral, so that incontinence of the sphincters indicates, as a rule, a bilat- 
eral disease of the spinal cord. In a unilateral affection, as in Brown- Sequard's 
paralysis, sphincter disturbance, as a rule, is lacking. Further the symptoms 
at the onset of myelitis point to an extensive lesion, for in the surrounding 
of the pathologic focus, similar to the condition in inflammation of the brain 
or apoplexy, reactive disturbances appear (alterations in the circulation, 
edema, small extravasations of blood). Only with retrogression or absorption 
of these secondary phenomena is the loss of function limited to its anatomical 
minimum. The compressed ganglion cells and nerve fibers then have an 
opportunity to recuperate. This is especially true of pressure paralysis and 
of multiple sclerosis, in which the medullary sheath is preeminently involved, 
while the axis cylinder is retained for a long time. The problem of physical 
therapy consists in educating the nerve tracts which remain to perform com- 
pensatory functions by means of methodic exercise. 

The first object of prrysical therapy in myelitic paralysis is a prophylactic 
one and consists in the prevention of muscular atrophy and of loose joints; 
of no less importance is the avoidance of spastic and paralytic contractures. 
It is very necessary to meet this indication because contracture deprives a 
paretic muscle of all possibility of motion, while if the tonus be at all normal 



THERAPY OF MYELITIS 441 

it may still be capable of function. A number of measures serve this purpose 
which should be utilized as early as possible. 

'We begin with a passive change in position of the paralyzed members. 
According to a definite formula of motion, the paralyzed extremities are placed 
in different positions several times daily, no joint, no muscle group being 
neglected. Primarily those positions are preferred which antagonize the ten- 
dency to contracture. Thus the ankle joint is flexed dorsally and pronated; 
the foot, in consequence of its weight and on account of pressure of the bed 
clothes, shows a tendency to plantar flexion and in consequence to pes equinus ; 
this is prevented by using a pillow which will maintain the position of dorsal 
flexion and by a loop appliance to prevent pressure of the bed clothes. 

These passive movements are gently and gradually extended to the normal 
limit. Rough handling only increases the contracture or produces it. Passive 
exercises are practised in one joint or in several at the same time, for example, 
flexion in the hip, knee, and ankle joints or abduction of both limbs. The 
joints may be held in position temporarily by sand bags, bandages or braces. 
We endeavor to prevent the very frequent adduction contracture of the legs 
by placing a pillow, containing some soft material or air, between the knees, 
or placing the patient for from 1-2 hours upon " straddle-board " (Mikulicz, 
Hoffa). 

Relaxation exercises may also be employed to prevent contractures. For 
this purpose the patient is encouraged to let the hypertonic members hang 
flaccidly and as limp as possible. By this means many patients succeed in 
relieving the tendency to contracture at least temporarily. (Muscular relaxa- 
tion, according to experiments in apes performed by E. Hering and Sherring- 
ton, is due to a feeble stimulation of the cortex.) These atomic gymnastic 
exercises are even more effective in a warm, prolonged bath (general or local). 
Other forms of application of heat, packing the rigid limbs in hot cloths, sur- 
rounding them with rubber tubes in which hot water is caused to circulate, 
the application of hot bottles or thermophores, compresses, and finally hot air 
baths. The use of heat also improves the cutaneous circulation; the cool 
cyanotic extremities becoming warm and red. Warm sand baths (caution 
must be exercised so as not to produce burns) have an influence in producing 
relaxation. 

In addition to the " antagonistic change of position/' the " exercises in 
relaxation" and the application of heat, massage, and electricity may be 
employed; those muscle groups should be especially treated which act against 
the tendency to contracture. Vibratory and stroking massage is used for the 
muscles and joints and for the nerve trunks, in the form of friction and vibra- 
tion. Percussion massage of the contracted tendons, movement of the joints 
which are in positions due to contracture, have a transitory effect in relaxing 
the muscles and improving motion. 

These methods of treatment are also opposed to the development of inac- 
tivity atrophy of the muscles. The muscle fibers whose trophic anterior horn 
centers are completely destroyed cannot be protected from atrophy. In this 
case and also in paraplegia after complete transverse myelitis, the baths, 
massage, change of posture and the passive movements have the purpose of 
stimulating the circulation in the paralyzed members, of preventing venous 
and lymph stasis, of opposing the development of inactivity atrophy and of 



442 MYELITIS 

vasomotor and trophic disturbances and thus improve the entire metabolism 
of the organism. In this sense we employ massage and gymnastic exercises 
in our paraplegics twice daily (morning and evening). 

The treatment of contracture, that has already developed, is much more 
difficult. We endeavor to combat it by extension and redressment, particu- 
larly with the aid of hot air or the warm water bath, or by fixation by means 
of steel braces in a position of over-correction. For this purpose we employ, 
in contracture of the knee joint, screw extension or our own redressment appa- 
ratus, in which the principle of extension is combined with direct redressment 
of the joint (compare P. Lazarus, Charite-Annalen, 27. Jahrg., 5. Jahres- 
bericht, Ueber die Anwendung der physikalischen Heilmethoden in der I. 
medic. Klink und Poliklinik). Passive pendular or swinging exercises, by 
manual aid or special apparatus (pendular apparatus of Krukenberg, Zander) 
are of value. In advanced contracture, where the joints in consequence of 
atrophy of the contracted tendons and fascia, are very difficult to move, surgi- 
cal measures or apparatus come into consideration in which elastic opposition 
maintains the equilbrium against the contracted muscles. Old, rigid, espe- 
cially painful, contractures can be relieved only by cutting the tendons. 

In connection with the treatment of contracture and atrophy is " exercise " 
therapy. This consists in the use of passive, autopassive and active methodic 
gymnastics. 

Passive motion, either by the hand of the physician, or by the electric 
current, or by any apparatus, conducts sensation from the altered tension of 
the nerve terminations in the muscles, tendons, fascia and joints, from the 
parts set in motion to their cortical centers. The central convolutions, accord- 
ing to the celebrated experiments of Hitzig and Fritsch, as well as those of 
H. Munk, are not only the motor, but also the sensory centers for the ex- 
tremities, so that a sensory stimulation of the muscle sense produces a simul- 
taneous stimulation of the motor tract. Passive motion, therefore, stimulates 
motor innervation, for an irritation of the muscle and joint sense stimulates 
the center of the affected extremity from which then the motor impulse may 
descend to the periphery. 

In an interruption of the pyramidal tract, either in the internal capsule, 
or in the spinal cord, the motor impulses cease above the lesion. In spite 
of all endeavor on the part of the patient there is no motion or at most the 
merest trace; only after repeated attempts, according to the law of summa- 
tion of stimulation, is there a distinct, although incoordinate movement. 
This fact, that the paralyzed patient is enabled to exert his will, even though 
in an incoordinate manner, proves, that a new communication has taken place 
between the sensory and motor tracts at a point outside the lesion. 

Our object now consists in cultivating this tract by systematic exercise. 
The repetition of the impulse of the will prepares the way for movement at 
the command of the will. By this we are to understand those movements 
of the paralyzed members which the patient is able to carry out himself with 
the aid of the normal members. The active muscles guide the paralyzed ones ; 
thus the legs, with the aid of the arms directly or by means of apparatus, are 
set into motion; in the same manner the leg that has more movement may 
be fixed to the paralyzed one, so that the latter is caused to partake of the 
same action. By these simple " auto-exercises " the path is prepared for the 



THERAPY OF MYELITIS 443 

motor impulse from the cortex of the brain to the muscle; the patient, whose 
voluntary motion has suffered from the prolonged inactivity, once more gains 
the sensation and conception of motion ; he learns to reinnervate, until, finally, 
he is enabled to perforin simple, active movements. 

In this stage the patient must practice walking movements, first in the 
recumbent posture; similar to the method that we employ in the physical 
treatment of hemiplegic contracture (see P. Lazarus, Zeitschr. f. didt. u. 
phys. Ther., 1901-1902, B. V, Heft 7). The patient at the word of command 
is ordered to raise and lower the leg, or to extend it in 4 quarter time ; at the 
knee joint to raise it, bend it, extend it and again lower the leg. The ankle 
joint is also exercised actively in stepping movements. These should first 
be practiced with the heel upon a base of support, such as sliding movements 
followed by exercises with the leg raised. In this manner the movements 
of walking are practiced in bed. In case the patient is able to sit up he is 
put either at the edge of the bed or upon a high chair and encouraged to 
perform extension and flexion of the knee and ankle joints with the legs hang- 
ing free. These exercises must always be of brief duration, but may be re- 
peated frequently during the day. If contraction or fatigue are noted, a pause 
should be made. It is serviceable to note in writing the nature and manner 
of the different movements, especially in regard to the active movement of the 
individual joint. Patient and physician gain a better insight by this means 
of the technic and progress of the treatment. 

We advance gradually with our exercises and permit the patient to attempt 
methodic standing and walking exercises in the erect posture. For this pur- 
pose we employ crutches or walking frames. Our latest model of a walking 
frame consists of a horse-shoe shaped iron frame; this is propelled upon 3 
wheels. The closed portion of the loop behind has a convenient chair at- 
tached. The front, free ends of the frame, have movable arm and axillary 
supports to which a scale is attached. This construction has the following 
advantages : the patient is able to walk unencumbered, without constriction 
about the chest or abdomen, his movements become more independent, espe- 
cially as he need not depend upon a second person to control the chair as 
in most apparatus of this kind. The paraplegic may also practice walking 
in this apparatus while in the sitting posture; the greater exertion of the 
erect posture is lacking, the legs are relieved of the weight of the body and the 
movements of walking are readily instituted. 

For walking we have had an exercise-shoe constructed which in shape re- 
sembles a skee. At the anterior and posterior end of a thick oak wood block, 
80 cm. long 4 cm. broad and about 0.8 cm. thick, there are sandals in which 
the feet are strapped. Those in front are used by the patient, who is manually 
supported or placed in the walking frame, those behind are occupied by the 
physician. The latter is enabled, by the movements of his own leg, to pro- 
duce the corresponding motion in the paralyzed lower extremity of the patient 
and thus to regulate the step-movement or to improve it. Finally the physi- 
cian may institute resistance gymnastics, permitting the patient to use active 
motion with the leg. while the physician opposes it. 

We gradually pass on to free exercises; the attendant steps behind the 
patient, holding him either by a girdle or by the clothing or passing his arms 
under the axillae of the paraplegic. Both step out simultaneously the physi- 
30 



444 MYELITIS 

cian with the tip of his toes directing the movements of the patient or having 
some one else place the patient's feet in the correct position; or the patient 
is encouraged to walk in foot marks that are drawn upon the floor. In this 
stage walking exercises in the parallel bars or along banisters, passive and 
active exercise in swinging apparatus and the like are also useful. Later 
by locking arms with another person or holding hands ; or walking with 2 sticks 
then with one stick, and finally entirely alone locomotion is reestablished. 
A result of this kind will naturally only be obtained in favorable cases; in 
the severe ones we cannot attain our purpose without orthopedic or surgical 
measures. The mechanical treatment of paralysis is greatly facilitated by 
wave-baths and electrotherapij. 

In the warm bath muscular rigidity and even recent contractures are re- 
laxed. The first traces of returning motion are often then observed. Leyden 
and Goldscheider have called attention to the great value of " kinetothera- 
peutic baths " in the treatment of paralysis. In the bath the weight of the 
member that is to be moved is compensated for by the buoyancy of the water. 
The specific gravity of the water may be increased by the addition of salt 
or brine. Maintaining the paralyzed members in equilibrium, combined with 
the relaxation of the hypertonia, prepares the road for active motion. This 
is particularly of importance in those patients who must learn to overcome 
the actual weight of their paralyzed parts in the water-bath. The impulse 
of motion, in these patients, receives a mighty stimulus by the returning 
power of motion obtained in the bath. The apparently dormant motor impulses 
awaken in the bath and after a long period of inactivity again reach the mus- 
cles. If the tract has once been established, between the impulse to motion 
and the muscles, our further object consists in cultivating this tract by methodic 
exercises until the patient is capable of sending definite impulses of motion 
precisely into those muscle groups to which they belong. 

For this purpose all muscles and joints of the paralyzed members are moved 
actively and passively in the kinetotherapeutic bath. Standing and walking 
exercises, in high bath tubs, are especially useful (Goldscheider's standing 
bath). The same author praises, quite properly, the favorable psychical influ- 
ence (plaisir de mouvement) which these movements in the bath exert upon 
the motor impulses (see Goldscheider und P. Jacob, Handbuch der physika- 
lischen Therapie, 1902). 

We must now turn to the electric treatment in myelitic paralysis. The 
curative effect of the electric current was formerly greatly exaggerated. There 
was an idea that the electric current, employed therapeutically, was able to 
penetrate to the spinal cord substance and there unfold its curative action. 
On the other hand we may assume that electric irritation, in consequence of 
the artificially stimulating muscular contractions, excites the dormant sen- 
sation of motion in the paralytic; to this may be added the passive motion 
which is produced by the electricity; this stimulates regeneration of the para- 
lyzed muscles and nerves in causing them to assume activity. The sensory 
stimulation produced by the electric current in the periphery has an inhibitive 
action upon the cortical motor impulse. This has been experimentally proven 
by Exner, Bubnow and Haidenhein, who showed that it requires a weaker 
electric irritation of the cerebral cortex, when the member, which is supplied 
by it, has previously been treated by electricity at the periphery. 



THERAPY OF MYELITIS 445 

Spastic paralyses should have sedative treatment ; active electrical contrac- 
tions should be avoided. For this purpose we apply a weak constant current 
which is permitted to act upon the motor nerves, motor points, and muscles. 
The strength of the current is gradually increased to from 2-5 Ma. and this 
is again gradually decreased. The anode is placed either stabile upon the 
motor point or is run slowly 20-30 times over the muscle, longitudinally and 
transversely. When marked reflex irritability is present and also in the acute 
stage, electric treatment is not employed. 

Flaccid paralysis and atrophy are treated by stimulation either with the 
galvanic, faradic, or the combined, currents. When there is DeR we only 
employ galvanism; the anode, as the stimulating electrode, in case, as is usual, 
it is the more active. Strong contractions may be produced by Yolta's alterna- 
tives. To increase the stimulation, especially in the deeper muscular areas, 
the combined galvano-faradic current is applied. The combination of both 
currents, according to de Watteville, is said to unite the stimulating influence 
of the intermittent faradic current with the " interstitial electrolysis " of the 
uniform galvanic current. 

The faradic battery is most often employed in flaccid paralysis, in mus- 
cular atrophy, in paresthesia, and in anesthesia. It is serviceable to have the 
muscular contraction produced by faradism occur simultaneously with the 
intent of the patient ; in this manner the motor impulse to the muscle is rein- 
forced. In the same way, by increasing or decreasing the strength of the 
current, we are enabled to aid the motor impulse more or less. By gradually 
decreasing the electric current we may burden the will more and more, until, 
finally, the patient is able to produce the movement without our aid. 

Cutaneous anesthesia is treated with the faradic brush or with franklin- 
ization (by drawing sparks). Stabile or labile cathodal galvanization (2-6 Ma. 
for 5 minutes) is said to be effective in anesthesia and paresthesia. Some other 
mechanical and thermal cutaneous stimulants (massage, carbonic acid or oxy- 
gen baths) further, contrast irritation (alternating applications of heat and 
cold) restore sensibility to the skin. 

In the management of spinal irritation and the girdle pains, the current 
may be sent through the thorax (cathode upon the vertebral column and anode 
upon the sternum) or in a longitudinal direction (anode above the supposed 
focus, cathode below or labile upon the peripheral nerves ) . 

For some years we have combined electricity with hath treatment. In the 
warm bath (full or local) the motor impulse is aided by the decrease of mus- 
cular rigidity especially if this be combined with electric stimulation in the 
manner described above. 

Recently paralysis and anesthesia have been treated with the electric 4-cell 
bath according to Dr. C. Schnee. By means of the 4-cell bath electricity may 
be employed in many different forms — galvanism, faradism or combined, as 
a pulsating direct current or as a sinusoidal alternating current; finally also 
for cataphoresis. By means of apparatus the current may. be sent through 
the body in any desirable strength or direction. 

To attain deep action, as in incontinence of the bladder, we employ the 
combination of faradization with electric vibration-massage. For this pur- 
pose the negative pole of the opening induction current is united with the wave 
of the vibration apparatus, which has a metal electrode at its end covered with 



446 MYELITIS 

flannel. This forms the common stimulation electrode of the faradic and 
vibration apparatus. By the wave-like propagated vibration the faradic cur- 
rent penetrates deeply and this increases the stimulating action of even weak 
faradic currents. 1 

If these methods of treatment bring about the coarse power of motion then 
a further object arises: the improvement of the coordination. For this pur- 
pose complicated movements are divided into their essential components and 
these are practised just as untiringly as the tactile exercises of the blind, until 
they are performed satisfactorily. In these coordination exercises, which are 
often very difficult, the delicacy of the sensation of motion is most important ; 
often the paraplegic loses his motor experiences, his memory of motion, from 
prolonged inactivity. By methodic exercise, passive or active, with closed 
eyes, the movements being carefully adjusted, just as in ataxia, we occasionally 
succeed in improving the deeper disturbances of the muscle and joint senses. 

The same principles are also operative in paralysis of the upper extrem- 
ities; but here the hand requires especial consideration. All muscles of the 
forearm, also the interossei and lumbricales, as well as all of the muscles of 
the thumb, should be subjected to careful electric and mechanicar treatment. 
The treatment of the nerve is particularly important in those cases in which 
the same nerve supplies muscles that act synergistically. 

Spa treatment can only be considered after the acute stage has passed, the 
patient no longer has fever and there is no danger in transportation. In gen- 
eral it is not good practice to send a myelitic away for mineral spring treat- 
ment sooner than 6 months after the inflammatory symptoms have passed 
away. Extreme temperatures, hot as well as cold, and particularly steam baths 
are to be avoided. 

On the other hand, moderate thermal baths, as in all other hydrotherapeu- 
tic procedures, at a temperature of about 37° C, are useful. They have a 
sedative effect upon the nerves of the skin and therefore reflexly upon the 
nerve centers; they decrease muscular rigidity and increase metabolism as 
well as the cutaneous and general circulation. The duration of the bath, at 
the onset, should be about 30 minutes, but in spastic paralysis, in hyperesthesia 
and hyperkinesia the time may be extended to several hours. This prolonged 
bath often has a very quieting effect. 

Among the indifferent thermal baths are Bagaz-Pfaffers in Switzerland 
(35° C), Badenweiler (26° C), Bomerbad (37^° C), Schlangenbad (18-32J° 
C), Wildbad (26i°-32° C), etc. 

The thermal, salt and chalybeate springs rich in gas are of service, such 
as Wiesbaden, Baden-Baden, Oeynhausen-Eehme, Nauheim and Minister. 
The carbonated thermal baths have a sedative action in diseases of the spinal 
cord and at the same time stimulate motility and sensibility. The favorable 
action of carbonic acid baths has gained for them many adherents among the 
laity, a proof of this being the many apparatus and preparations for the arti- 
ficial development of carbonic acid baths. In our Clinic we employ the 
v. Orth system. With this system the compressed carbonic acid gas (or 

1 In regard to the diagnostic and therapeutic indications of immediate electrization 
of the spinal cord and the cauda, see P. Lazarus, " Ueber die spinale Localisation der 
motorischen Functionen." Zeitschr. f. klin. Med., Bd. LVII, p. 99 (1905). 



THERAPY OF MYELITIS 447 

oxygen) is led from the steel cylinder into porous tubes which enter the 
batli tub and from which the gas emerges in small bubbles. The amount of 
gas may be accurately controlled by a manometer and stopped at any time. 
This system is characterized by simplicity, accuracy, lack of destruction of the 
bath tub, and by the low cost; it being possible to prepare a carbonic acid 
bath at a cost of about 2% cents. The duration of the bath should be 10 min- 
utes, at most 20 minutes, the temperature 36° C.-31 C. The mechanical effect 
may be increased by undulations or shower baths. In paralysis combined with 
anemia, iron-peat baths, such as Franzensbad, Marienbad, Elster, Cudova, are 
valuable. Peat baths stimulate the skin and have a great absorbent effect. 
The hot sulphur springs are very useful in syphilitic myelitis (Aix la Cha- 
pelle, Baden near Vienna. Pistyan). When arthropathies are present, peat, 
mud and fango packs may be employed. 

The physico-mechanical methods of treatment (massage, gymnastic exer- 
cises, electricity, hydrotherapy and balneotherapy) which have been described 
are Dot sufficient when rigid contractures or grave, flaccid, atrophic paralyses 
have appeared. Under these circumstances apparatus and surgical measures 
are necessary. 

The treatment by apparatus has for its purpose the relief of the contracture, 
the substitution of function for the paralyzed muscles, and the strengthening 
of the flaccid joints. The contractures are managed by redression apparatus. 
best adjusted after a previous tenotomy, or by a plastic operation of the con- 
tracted tendon. We attempt to supply the function of the paralyzed muscles 
by elastic bandages, which, in their action, imitate the physiologic mechanism 
of movement and by their tension maintain in equilibrium the power of the 
antagonists. Thus in paralysis of the quadriceps or of the peronei muscles 
an apparatus ma} r be applied which has a hinge for the knee or ankle joints, 
and elastic hands corresponding to the insufficient muscle groups; this appa- 
ratus, to a certain extent, converting the displacement of the muscle power 
to the outside of the leg. 

Apparatus may also be applied to support loose or deformed joints. By 
this means we have often succeeded in again putting a paraplegic "upon his 
legs." We shall report a case of myelomeningitis lumbalis in a man aged 31. 

This patient came to us with atrophy and flaccid paralysis of the muscles of both 
legs; those of the hip joint being- markedly paretic, of the knee and ankle joints being 
immotile to active impulses. The left leg. nerve and muscles, could not be stimulated 
by galvanism or faradism; the right leg hardly reacted to faradism and only responded 
to maximal galvanic currents and then only the quadriceps, crural, and peroneal mus- 
cles. The muscles of the right calf did not react to the strongest currents. Upon the 
posterior surface of both legs as well as upon the dorsum of the feet there was anes- 
thesia and analgesia ; the scrotum was also anesthetic. Cutaneous and tendon reflexes 
were abolished in both legs. It was, therefore, a case of transverse disease reaching to 
about the second lumbar segment. 

The treatment consisted in hydrotherapy and electro-gymnastics, sand and water 
baths, bloodless nerve stretching, this having an especially favorable effect upon the 
severe sciatica: further, injections of iodopin and corrosive sublimate. The result con- 
sisted in increased motion of both hip joints and of the right knee joint. The galvanic 
contractility of the muscles of the right thigh returned. The patient, however, was 
unable to walk on account of the residual, complete, flaccid-atrophic paralysis of both 
ankle and knee joints. Only by means of the apparatus here illustrated and with the 
aid of 2 canes could the patient walk with some degree of certainty; he was able to 
resume his occupation, and can now descend from a carriage and ascend stairs. 



448 



MYELITIS 



If the paresis involves the hip joints, the patient may be aided by an appa- 
ratus consisting of a corset to which 2 splints are attached for the legs. Such 

an apparatus is shown by Fig. 172. 
This illustration was taken from a 
patient aged 50, suffering from, 
high grade ataxia of the arms, legs, 
and trunk, combined with pareses, 
muscular atrophy and arthropathies 
in the ankle, knee and shoulder 
joints. Even with support the pa- 
tient could neither stand nor walk, 
and upon attempts to assume the 
erect posture she collapsed at the 
hip and knee joints. In consequence 
of ataxia of the arms she could not 
even hold a cane. The supportive 
apparatus consisted of a corset with 
steel braces, with supports to the 
hips and axillas; this was united 
by means of rubber to a brace for 
the legs, induction and flexion of 
the hip joints was somewhat hin- 
dered but not enough to allow for- 
ward movements. The illustrations, 
Fig. 172, a, b, c, show in detail the 
structure of the apparatus; this 
permitted the patient to stand un- 
supported and even to walk with 
slight assistance. 

In complete paralysis, when the 
antagonists and the agonists are 
both involved, another principle 
may be applied, namely, the trans- 
mission of the normal muscle 
power to those involved. Thus the 
shoulder or the arm may be con- 
nected with the paralyzed leg by 
inextensible bands, so that the leg 
is moved by the arm. 

But even the best apparatus is 
undesirable company, especially if 
it must be worn day and night; 
it gives rise to circulatory disturbances and to atrophy; these must be com- 
bated by exercise, baths and electricity. In those cases in which the mechani- 
cal and gymnastic methods, described above, give but little hope of success 
on account of great muscle and arthritic changes, surgical measures come 
into question. Here we must mention the elongation of contracted tendons, 
as in club-foot, the Z-shaped splitting of the Achilles tendon and uniting 
the ends which have been displaced; shortening of tendons with hyper- 







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m^M 






m 




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,* ,, ifflBi rtMW ,— ,A 



Fig. 171. 




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w 

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O 

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PS 
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04 
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449 



450 MYELITIS 

distension of the flaccid muscle, as the peronei or the dorsal flexors of the 
foot in paralytic pes equinus; further in loose joints, arthrodesis either by 
uniting the tendon to the capsule of the joint or by freshening and suture of 
the arthritic surfaces. Arthrodesis should be performed only when all other 
methods of treatment have failed and when there is paralysis of all peri-articu- 
lar muscles. 

In regard to the operative treatment of the spondylitic gibbus, see Hoffa 
" Die moderne Behandlung der Spondylitis, 1900." 

Transplantation of tendons, proposed and performed by Nicoladoni in 1881, 
has assumed great importance; this is most successful in dissociated muscular 
paralyses. In this condition only one muscle group is affected, while the 
antagonists are preserved and are usually strongly contracted. In these 
paralyses only surgical and orthopedic measures present hopes of success, and 
either redression apparatus or operative transplantation of the functionating 
muscles to the insufficient ones. The latter is accomplished by tendon trans- 
plantation, by which the normal muscle is implanted upon the affected one; 
thus in paralysis of the dorsal flexors of the foot and of the peronei, the 
Achilles tendon may be divided in 3 parts and 2 of these may be attached 
to the muscles that do not function. Prior to the operation we must deter- 
mine by careful and accurate functional and electrical tests that the muscle 
to be used is actually normal. During the operation we may form an opinion 
of the condition of the muscle by electric stimulation and by examination; 
normal muscles have a fresh, red appearance, those presenting fatty degen- 
eration are of a yellowish color and are flaccid. 

With the transplantation of the normal tendon into the affected one, com- 
pensation is as yet not brought about; in the cortex of the brain a change 
must also take place. When, as was done successfully by F. Krause in a case 
of infantile paralysis, the quadriceps femoris is replaced by the normal flexors 
of the knee, this alone does not transmit the function. The patient must first 
learn to extend the knee with the center for the flexors of the knee, he must 
learn to innervate in a diametrically opposed manner, and this is one of the 
objects of our treatment. This function is usually attained rapidly and the 
patient, especially if he be young, soon learns to contract the transplanted 
muscle portion. According to these principles, in the last few years, numer- 
ous operations of this kind have been performed with marked excellent func- 
tional success. 

Many patients owe their power of earning their living and their freedom 
from the rolling chair, to these physical-orthopedic methods. The common 
labors of the internist and the surgeon have had a beneficent effect in the 
" border land " of myelitic paralysis and many paraplegics have been saved 
from the almshouse and home for cripples. The prognosis of myelitis has 
improved vastly owing to the progress of physico-orthopedic therapy. 

Even the apparently hopeless cases of myelitic paralysis should be treated 
by the physician, free from nihilistic scepticism and just as free from uncrit- 
ical polypragmaty. Full of hope, he should prepare a plan of treatment which 
will meet all of the indications in a harmonious manner and this should con- 
tain all the well tried pharmacologic and physical methods of cure sanctioned 
by our science and our experience. 



EXPLANATION OF THE COLORED PLATE 



Fig. 1. — Influenza myelitis. Hemorrhagic focus of softening (4) ; extensive hemorrhagic 
infiltrations (2, 3, 5, 6), ascending degeneration of Goll's columns (1). 

Fig. 2. — Acute myelitis in the puerperium. Spinal cord hemorrhage with secondary 
myelitis (ascending and descending degeneration). 

Fig. 3. — Hematomyelia in the puerperium. Description in the text. 



PLATE I 



Fig. 1. — Influenza Myelitis. 
1 4 



Fig. 2. — Acute Myelitis in the Puerperium. 
1 5 








W:%* 




Fig. 3. — Hematomyelia in the Puerperium. 
a 






Granular cells Hematoidin Reddish brown Myelin g J) 
crystals axis cylinders drops 




TABES DORSALIS 

(GRAY DEGENERATION OF THE POSTERIOR COLUMNS OF THE SPINAL 
CORD; ATAXIE LOCOMOTRICE PROGRESSIVE; LOCOMOTOR ATAXIA) 

By WILHELM ERB, Heidelberg 

I. INTRODUCTION AND HISTORY 

In the points of frequency and importance, tabes 1 is unequalled by any- 
other chronic disease of the spinal cord. It is the most widely distributed, the 
most thoroughly studied, the richest in symptoms, and in the number of scien- 
tific problems it presents, and is unexcelled by any in the number of opinions 
to which it constantly gives rise. 

Clinically as well as anatomically it is sharply characterized, and is one 
of the forms of spinal disease which have a most sure foundation. Clinically 
— in spite of the unending variety of symptoms which it produces — it is 
marked by phenomena which recur with the greatest regularity, and which 
form the true foundation of the pathologic picture, around which at times 
many other symptoms crystallize. In the first stage of the disease we chiefly 
note disturbances of the sensory sphere (pain, paresthesia, hyperesthesia, anes- 
thesia of typical form and localization, and sensations of fatigue) combined 
with disturbances of certain cranial nerves (the optic, the nerves of the mus- 
cles of the eye) and of the bladder and sexual apparatus : in the second stage, 
by the addition of a new and very characteristic motor disturbance, tabetic 
ataxia and disturbance of coordination are produced, and dominate the clinical 
picture. Anatomically, it is mainly marked by a gray degeneration of the 
spinal posterior columns and the posterior roots, as well as by other invariable 
or accidental lesions. 

A comprehensive knowledge of this remarkable and many-sided disease 
which affects so severely the life of the individual and society in general is 
one of the achievements of the nineteenth century. In the writings of the 
ancients from the time of Hippocrates all search for a description of tabes 
had been fruitless; it is hardly conceivable that the typical peculiarities of the 
disease could have escaped the attention of the great investigators in all former 
ages, or that its description in their works should not be recognizable. Tabes 
does not appear to have existed in antiquity, nor for many centuries after. 
Since we know to-day that the most essential, the almost exclusive, requisite 

1 We agree with Moebius that the term " tabes " now everywhere accepted is a 
suitable designation for the affection under consideration. 

451 



452 TABES DORSALIS 

for the development of tabes is syphilis, this appears reasonable, for the most 
recent investigations indicate with increasing positiveness that syphilis was 
transported to Europe only after the discovery of America and toward the end 
of the fifteenth century. 

It is not our purpose here to enter upon a discussion of the history of 
tabes. It is sufficient to state that these views concerning it date from about 
the middle of the preceding century; there were reports of this affection by 
Horn (1827), Stanley (1840), Steinthal (1844), and others, but it was the 
comprehensive descriptions of Todd (1847), of Romberg (1851), and of Du- 
chenne (1858-9) which acquainted the professional world With the disease; 
the classical description of Romberg and the masterly clinical presentation of 
Duchenne, the latter of whom first characterized it as " ataxie locomotrice 
progressive," have established the clinical picture and have inspired number- 
less investigations and much labor concerning the apparently " new " disease. 
The period from 1860 to 1870 witnessed a flood of important and compre- 
hensive clinical and pathologico-anatomical investigations, especially in France 
(Trousseau, Charcot, Vulpian, Dujardin-Beaumetz, Mar. Carre, Topinard, 
Jaccoud, and many others) and less so in England ; in Germany, however, in 
the year 1863 three reports appeared by Eisenmann, Ley den, and Friedreich, 
and these formed a landmark in the study of tabes, even in a pathologico- 
anatomic respect. Many other reports by German physicians followed, clinical 
and anatomical investigations increased in number, until it appeared from the 
explicit presentations of the subject published about 1865 by Leyden and Erb 
as if our knowledge were complete. 

But not so ! The subject proved to be inexhaustible, and in all civilized 
countries the investigation was continued until a true high water mark of 
reports upon tabes seemed to be reached, thousands being poured out, in an 
endless, overwhelming stream ! 1 

This led to a comprehensive consideration and specialization of the symp- 
tomatology: The recognition of reflex pupillary rigidity, the absence of the 
tendon reflexes, the hypotonia of the muscles, the special form, localization, 
and topography of the sensory disturbances connected with the old Romberg's 
symptom, the determination of an enormous number of so-called visceral symp- 
toms (gastric and intestinal crises, laryngeal, renal, bladder, genital, and 
cardiac crises, etc.), the very important trophic disturbances (tabetic arthrop- 
athy, loss of teeth, atrophy of bones, etc.), the description of nuclear and 
bulbar cranial nerve disturbances, of peripheral, neuritic, muscular atrophies, 
of paralysis of the recurrent laryngeal nerve, and many other conditions filled 
literature, and revealed a most unexpected richness of individual symptoms. 

The improved methods of our time have given us comprehensive patho- 
logico-anatomical reports which furnished new and varying conceptions, and 
led to most surprising conclusions; unfortunately, much in this realm still 
remains obscure, and contradictory views have not yet been reconciled. 

In the study of the etiology of tabes the conditions have been different. 
A debate which has continued to the present time was started about 1870 
when Fournier and Vulpian maintained that the great majority of tabetics 
had a syphilitic history, that tabes, therefore, was to be regarded as a " para- 

i See the report by Mobius, 1-14. 



INTRODUCTION AND HISTORY 453 

syphilitic," or according to other authors, a meta- syphilitic affection due to 
syphilis. I investigated these reports, at once confirmed them, and took my 
stand on the side of the French authors; Gowers, Striimpell, Mobius, and 
others followed, but there was opposition on the part of nearly all the other 
German authors. Thus a lively partisanship arose. Full statistics and casuis- 
try were compiled; reasons and opposing reasons were advanced, pathological 
anatomy and treatment were invoked to decide the issue. The result of the 
dispute is as follows : The assumption of a connection between tabes and pre- 
ceding syphilis is sustained, although the exact relations and nature of this 
connection are still in doubt. 

Nearly all of the prominent neurologists and internists, almost all of the 
recent text-books, recognize this connection, not only in Germany but else- 
where — in France, England, America, Russia, and Italy. Only a few eminent 
authorities (v. Leyden, Goldscheider, Moczutkowski, and others) are opposed 
to this and deny the relation. It is to be hoped that the overwhelming mass 
of statistical, clinical, and anatomical facts in j^roof thereof will overcome this 
opposition. 

Much time has been consumed in discussing general pathologic questions, 
questions of the physiology and pathology of the spinal cord which naturally 
arise in the consideration of tabes : The position of the sensory tracts, the con- 
duction of various sensory impressions in the spinal cord, the various reflexes, 
the location of their tracts, the innervation of the pupils, above all, the typical 
disturbance of coordination ; briefly, the ataxia and much besides without com- 
ing to any conclusion. 

The actual nature and origin of the pcUhologico-anatomical changes, their 
intimate relation to syphilis, the pathogenesis of the individual disturbances, 
their exact cause and peculiar localization, etc., have, of course, been much 
debated. 

Finally, in the last few years we have undertaken the finer investigation 
of the various forms of the affection, we have learned to recognize the " formes 
frustes," to discriminate between the varieties of incomplete tabes, their com- 
binations, and their widely different types ; we have elaborated a much more 
comprehensive picture of this many-sided disease than would have been thought 
possible from the earlier, pure, and typical cases ; but this is usually the case 
in studying the pathology of the nervous system. At first typical forms only 
are observed, and from these the clinical picture is constructed ; by and by 
we recognize that this extends beyond the limits drawn, that other varieties, 
transitional forms, and related processes exist which not infrequently are ex- 
ceedingly perplexing to the practitioner. 

All these factors, however, have made the diagnosis of tabes more exact and 
more minute; we now fully recognize the gravity of the disease, even in its 
early stages, and the dawn of this century has just furnished us an important 
and most valuable aid to diagnosis in lumbar puncture and cytodiagnosis of 
the lumbar fluid. 

That the prognosis of the disease is to-day vastly different from what it 
was fifty years ago when Romberg gave expression to his celebrated and hope- 
less view, will later be evident. 

As a matter of course, from our modern etiolooric knowledge new indica- 
tions and broader methods of treatment have arisen; these are still being 



454 TABES DORSALIS 

developed and amplified, but so far have yielded no decided results ; new meth- 
ods of treatment, such as nerve stretching, suspension, and orthopedic treat- 
ment, have been introduced into practice, and have in great part again fallen 
into disuse ; much more important, however, is the " compensatory practical 
treatment" (by movements, the " reeducation des mouvements" of the French) 
introduced by Frenkel, and especially advocated and practised by v. Leyden 
and his adherents ; this, naturally, is only adapted to a late symptom of tabes, 
the ataxia, for which it is often beneficial. 

Briefly reviewing the advance of the last thirty years in the teaching of 
tabes, and with what possessions it enters into the new century, we may say 
that in all probability the etiology of the disease has undergone the greatest 
change, that it has been worked out conclusively by demonstrating the inti- 
mate relation to syphilis; yet, in spite of this, much remains obscure — the 
auxiliary causes, the minute pathogenesis, etc. 

On the other hand, the symptomatology of tabes, the recognition of differ- 
ent forms, of transitional stages, their diagnosis and prognosis, have all been 
greatly amplified. 

In spite of this close investigation, the pathological anatomy and the 
pathogenesis of the disease are still doubtful; much which relates to the gen- 
eral pathology is still theoretical ; the present treatment of tabes is still imper- 
fect and unsatisfactory. 

Therefore, .notwithstanding our great advance, the enormous amount of 
labor expended, and the voluminous literature which has appeared, the twen- 
tieth century has before it a vast field in elucidating the teaching of tabes. 

Let us hope that we shall get to the very depths of this problem ! 

II. ETIOLOGY. (THE CAUSES OF TABES) 

It is obvious that with the introduction of tabes into pathology as a typical 
form of disease, the question of its cause immediately arose. Little care and 
acuity were shown in the answer. Authors were content to designate two 
etiologic factors, sexual excesses and exposure to cold, and even these were sub- 
ject to doubt; in addition, over-exertion, profound emotion, nervous predis- 
position, rheumatic processes, and syphilis were mentioned, the latter of which 
even Duchenne did not greatly emphasize. These etiologic views convinced 
no one. 

When Fournier reported (1871) the frequency of syphilis in the history 
of tabes, and this was soon after confirmed by Vulpian, Grasset, Gowers, and 
myself, a new factor appeared which formed the basis for numerous and thor- 
ough investigations. The number of those who adhered to Fournier's teaching 
steadily increased, for by compiling full statistics, by emphasizing the value 
of individual clinical observations and general considerations, they attempted 
to make the connection between syphilis and tabes more clear and more posi- 
tive; the number of opponents who at first combated this view with other 
statistics and much reasoning, who attempted to make other etiologic factors 
prominent, and to emphasize their importance and frequency, constantly de- 
creased; many an energetic opponent was convinced of the etiologic relation 
of syphilis, and only a few still cling tenaciously to the opposite view. 

In the following presentation I propose to state facts which embody the 



ETIOLOGY 455 

results of the investigations of the last 2-5 years; the reader will, however, be 
referred to special treatises for all details. A comprehensive and critical review 
is beyond the scope of this article. 

We shall first consider the general circumstances under which tabes appears, 
and shall then discuss the special factors which are directly causative. 

Tabes, upon the whole, is not a rare affection, and among the chronic dis- 
eases of the spinal cord it may even be regarded as common; whether it has 
become more frequent in our time, as some assume, is not certainly known, 
although it does not seem unlikely, especially when we consider its close rela- 
tionship to a disease of the brain (progressive paralysis), in which this condi- 
tion has been almost positively proven. 

It is a disease of adult and middle life, and in the majority of cases appears 
between the ages of 25 and 50; it is rare in the aged, but cases have been 
observed which began after 60, and even later than 70; it is almost as rare 
in children, but recently cases of tabes in children as well as during puberty 
(infantile and juvenile tabes) have increased. 

In regard to sex, tabes predominantly affects males; in my own large 
experience, which has been principally among the better classes, there were 
1,258 tabetic men and only 63 women (therefore a ratio of 20 to 1). Other 
observers have found a much larger percentage of women, so that proportions 
of 10 to 1, 7 to 1, 5 to 1, and even 2.5 to 1, have been given. A very decided 
disproportion has been noted between the higher and lower classes, as well as 
1 >et ween the different localities in which the affection occurred. Among women 
of the higher classes tabes is exceedingly rare; on the other hand, it is rela- 
tively more frequent in men of the higher than of the lower classes, and is 
much more common in women of the lower than in women of the higher classes, 
so that the number of tabetic women among the lower classes corresponds to 
that of tabetic men in the same class of society. This proportion is noted, 
as the rule, in large cities. 

In fact tabes is much more common in the city than in the country; the 
larger the city the more numerous the cases of tabes, especially in women of 
the lower classes. 

The disease prevails more extensively in civilized countries than in non- 
civilized; "civilization with its many deleterious factors appears to wield a 
predominant influence." 

The social position seems to be of considerable importance in the etiology ; 
the educated classes are much more frequently attacked than the uneducated, 
particularly merchants, travelling salesmen, and soldiers; among theologians 
it is rare; those who live much in the open air and are inured to cold and 
privation are not attacked to any special extent. 

These peculiarities of the affection point to peculiar etiologic factors, which 
cannot be explained as the ordinary effects of cold, sexual excesses, trauma, etc. 

We shall now consider what has been learned in the past 25 years concern- 
ing the causes of tabes. 

Among these, according to my experience, syphilis plays by far the most 
prominent role. Statistics compiled from only the most carefully selected 
material, accurate histories, and exact clinical data prove its importance. 

In illustration of this I shall first quote my own statistics which — begun 
with the view that tabes and syphilis had nothing in common — had been col- 



456 TABES DORSALIS 

lected in the past twenty-five years. I can guarantee their thoroughness and 
correctness, and the results which have never varied from the first hour (1879) 
to the present time seem to favor their reliability. 

I shall not discuss the individual difficulties and the sources of error in 
these investigations; they have often been described, and with some practice 
and care in detail may in great part be overcome. 

The best subjects for statistics are male tabetics of the better classes; 
from them alone are accurate and reliable reports to be obtained, far more 
so than from males of the lower classes, or even from women in whom, as is 
well known, the detection of preceding syphilis is often very difficult, and 
can be arrived at only by a roundabout process (through the husband, the 
physician, or the family, the prior social history, reports from hospitals, minute 
investigation for the sequelse of syphilis, abortion, etc. ) . Nonne has published 
an especially valuable report on this matter. 

My compilation, recently published, 1 of 1,100 cases of tabes among the 
better classes, shows that of this number 89.45 per cent, had previously been 
infected by venereal disease; among these 62.9 per cent, gave positive evidence 
of secondary syphilis, and 26.54 per cent, reported merely a chancre; doubt- 
less many of the latter should also be regarded as positively syphilitic (because 
of their designating the chancre as a hard sore, of their having been treated 
with mercury and iodin, of leukoplakia in the mouth, of abortion in the 
wife, etc.). 

Of the remaining 10.55 per cent, who denied infection, there were many 
cases which were more or less suspicious (because of repeated attacks of gon- 
orrhea, of buboes, of questionable ulcers, of profuse leukoplakia of the mouth, 
of abortions and premature births in the wives, etc.) and which could not be 
regarded as positively free from infection. In this respect minute compila- 
tions of the last 900 cases showed that in at least 3 per cent, (more accurately 
2.8 per cent.) of the cases the most carefully elicited history and closest 
investigation failed to reveal preceding infection. Any one familiar with the 
history of occult syphilis, " syphilis ignoree," syphilis insontium, will appre- 
ciate the importance of this fact. 

Hence it appears from these voluminous statistics that among 100 cases 
of tabes in men of the better classes about 90 had formerly suffered from 
syphilis or chancre, and only 10 were apparently free from infection. 

A striking and almost overwhelming demonstration ! And yet these figures 
in themselves would not be conclusive (for with like statistics we might 
demonstrate that 80, 90, or a still greater percentage of tabetics had formerly 
suffered from measles, coryza, angina tonsillaris, influenza, and the like) 
provided it had not been simultaneously proven that in non-tabetics of the 
same class of society quite different relations exist. Therefore a control test 
must be made, i. e., we must determine what percentage of infected and non- 
infected are found in the same category, that is, among non-tabetic men of 
the higher classes over 25 years of age. I made this control test and, that 
it might not be partial, included even the paralytics and other patients with 
cerebral syphilis, etc. ; this test now includes records of more than 10,000 
men of the higher classes, and shows that of these only 21.5 per cent, had 

i Berliner Jclin. Wochenschr., 1904, Nr. 1-4. 



ETIOLOGY 457 

been previously infected and 78.5 per cent, were uninfected ; 9.8 per cent, 
of the former apparently had merely a chancre; and 11.7 per cent, had sec- 
ondary syphilis. 

The result is conclusive : Of those uninfected 10.5 per cent, had tabes and 
78.5 per cent, were non-tabetic; of those infected 89.5 per cent, had tabes, 
and 21.5 per cent, were non-tabetics. Therefore, among the tabetics there were 
almost J+ and -J times as many infections as among the non-tabetic men in the 
same class of society ! 1 

I believe these statistics to be most valuable and decisive, because they 
were based on very reliable data ; but my statistics of men in the lower classes, 
and of women with tabes give almost the same results. I shall briefly quote 
these. 

Among 158 tabetics in the lower classes 77.2 per cent, were infected, and 
22.8 per cent, were uninfected with syphilis; therefore a smaller ratio than 
in the higher classes, yet, considering the difficulties in eliciting the history, 
a very high percentage. But the control test made in patients in my Clinic 
showed that among 1,300 cases (males over 25 years of age) only 6.54 per 
cent, were infected, and 93.46 per cent, were non-infected. This surprisingly 
low number of infections is probably accounted for by the circumstance that 
my clinical material was largely composed of country people. But other inves- 
tigators also (Kuhn, Sarbo, Collins, and others) failed to find essentially 
higher figures in the lower classes. 

Finally, we come to tabes in women. To simplify matters, I shall con- 
sider the higher and lower classes together, but I must remark that in the 
former infection could be much more positively determined (86.7 per cent, 
in a series of 16 cases) than in the latter. Combined, therefore, in a total 
of 63 cases, 44.44 per cent, showed positive, 36.5 per cent., probable infection, 
and in 19.05 per cent, no infection could be demonstrated. This result suffi- 
ciently agrees with the results in men. 

So much for my own statistics ! Far be it from me to regard these alone as 
decisive; but a brief review of the numerous cases in literature — and it is 
impossible here to enter into all the details — at once shows that many, and 
in fact most, other observers found similar or even higher percentages; I 
shall quote a few of these, and will state in this connection that earlier statis- 
tics — from about 1880 — are not all reliable, as is shown by the fact that at 
first some physicians found only a small percentage, but, with more experience, 
this constantly increased. (See table on page 458.) 

The statistics of tabes in women vary much more, and this is readily 
explained. In 13 cases Moebius found syphilitic infection in 80 per cent., 
and in 5 cases in 93 per cent. ; Minor (8 cases) in 100 per cent.; Redlich (30 
cases among the lower classes), positive in 23.4 per cent.; Kuhn of Berlin 
(78 cases), positive or suspicious in 64.1 per cent.; Kron (Berlin, 41 cases), 
positive in 44 per cent., most likely in 12 per cent. = 56 per cent. ; Nonne 
(22 cases) in 77.3 per cent,; Silex (8 cases) in 87 per cent.; Fehre (Ger- 
hardt's Clinic, 41 cases) in 66 per cent.; Collins (16 cases) in 70 per cent.; 

1 In pathologic anatomy it has finally been decided to adopt the control test. Fritz 
Lesser reports that in performing autopsies on 96 tabetics he demonstrated syphilis in 
28 per cent., but in all other cases over 35 years of age which came to autopsy he found 
it in only 9.5 per cent. ; therefore, it was thrice as common among tabetics ! 



458 TABES DORSALIS 

From France : l Infected 

Fournier (last compilation of 1,000 cases) 93 per cent. 

Labbe 93 « « 

Martineau a c 95 « « 

Dejerine 97 " « 

Ferras „ „ 91 « « 

Belugou and Faure (1,903-1,960 cases) 77 " « 

From Germany and Austria: 

Voigt, first (1881), 67 per cent. ; later (1885) 82 " " 

Rumpf (1883), 66 per cent. ; later (1887) 85 " " 

Gg. Fischer, positive, 72 per cent. ; probable , 90 " " 

Seeligmann (G-. Fischer) 86 " " 

Eisenlohr 60 " " 

Bernhardt, first, 40 per cent., 60 per cent., 58 per cent. ; finally 83 " " 

Remak (1884) 63.5" " 

Strumpell 90 " " 

Hirt (319 cases) 92 " " 

Gerlach (Hitzig) About 78 " " 

Fr. Schultze 66-71 " " 

Kuhn (Jolly's clinic, lower classes, 240 cases) 60.3-81.6 " " 

Silex (Berlin) 81.5 " " 

Dinkier (37 cases) 93 " " 

Schittenhelm (Kast's clinic, lower classes) 67.8 " " 

Sarbo (Budapest), higher classes 86 . 6 " " 

" " lower " 74.6" " 

Redlich (lower classes) 65 . 2 " " 

v. Halban (v. Krafft-Ebing's clinic, 236 cases) 13 .5 + 73 " " 

Coester (Wiesbaden, 93 cases) 82.8 " " 

Determann (132 cases), positive syphilis (7.2 + ) 72 " " 

From England: 

Gowers (1889), 170 cases 75-80 " « 

Althaus (1881), 90.6 per cent. ; later (1884) 86.5 " " 

Byrora Brain well 76 " " 

Mott(1903) Positive, over 70 " " 

From America: 

Seguin 72 " " 

Spitzka.. 80 " " 

Bonar (286 cases) 69 " " 

Dana (50 cases) 68 " " 

Collins (140 cases) 80 " " 

From Italy: 

Negro (232 males, 1901) 89 " " 

From Russia: 

Minor 60-70 " " 

Gajkiewiez 90 " " 

Anfimow (322 cases) 83 " " 

Tumpowski (Warsaw, 257 cases) 64 " " 

Homen (Helsingfors) 83 " " 

Moczutkowski, positive, 36 per cent. ; probable 72.2 " " 

1 Very few statistics from France have come to my knowledge in the last few years ; as I 
know from verbal communications, the opinion is general in that country that tabes is in 
almost all cases a syphilogenous disease (compare Dejerine and Thomas, " Malad, d. la moelle 
epiniere," Paris, 1902). 



ETIOLOGY 459 

Kojewnikoif in 63 per cent.; Friederiehsen in 39.3 per cent.; Negro (25 cases) 
in 68 per cent. ; Schittenhelm in 54 per cent., etc. 

The total number of cases in these statistics of tabes certainly amounts to 
many thousands, and they all reveal the same conditions as my own. 

But I cannot deny that there are compilations of cases which give a decid- 
edly lower percentage; for example, that of A. Eulenburg, showing 36.8 per 
cent., of Hofmann 3? per cent., of Petrone 48 per cent., and others. Simi- 
lar ones have been published, even up to most recent times, and usually based 
on a small number of cases, so that they may be added to the above statistics 
without decidedly modifying the result. Two of these, because emanating 
from very prominent authors, demand notice. They were published by Stor- 
beck and Gutmann of v. Ley den's Clinic. These compilations are based on 
statistics of both men and women of the higher and lower classes of society. 
In his 108 cases Storbeck calculates 20.4 per cent., or, at most, 30.6 per cent, 
of positive syphilis (in the 75 men comprising his cases I calculated 60 per 
cent, as being previously infected although not positive syphilitics !), and in 
111 cases Gutmann found only 35 per cent, with preceding syphilis; even on 
adding 20 cases from the records of a life insurance company, he found only 
28.6 per cent. ! 

In the light of my own experience and that of other observers, I must 
admit that these low figures, which agree with a few other statistics, are abso- 
lutely incomprehensible to me, the more so because simultaneously, from other 
sources, in Berlin (Jolly's and Gerhardt's Clinics. Silex, Kron and others), 
figures much higher, even twice as high, have been obtained. It is not worth 
while to discuss the probable cause of these differences; an accidental distri- 
bution of the cases may play a role in these relatively low figures, or they 
may be due to a lack of thoroughness in the investigation, to an admixture 
of unreliable material, as, for example, of women from the lower classes 
(Storbeck reported 111 cases, among them 33 women, only 2 [ ! !] of whom 
were said to have been positively syphilitic), or to placing too great confidence 
in unreliable reports such as those obtained from soldiers who have become 
tabetic, or from life insurance companies whose statistics concerning preced- 
ing syphilis are unquestionably most unreliable — these may be the causes of 
the low figures, and time will reveal the true state of affairs. In any case 
I believe I may state that these statistics which give relatively low figures, 
when contrasted with the overwhelming majority of cases with high percentage, 
are not very important, and that even when added to the other figures they 
do not essentially change the result. 

And this result is that from 70 to 90 per cent, of all the cases of tabes are 
preceded by a syphilitic infection. Experienced syphilographers who are famil- 
iar with syphilis occulta and syphilis ignoree are of the opinion that it is wise 
to recognize syphilis in 100 per cent, of the cases ! 

The question by what length of time syphilitic infection precedes the 
appearance of tabes is answered by the fact that in the overwhelming major- 
ity of cases (70 to 80 per cent.) tabes appears from 5 to 20 years after infec- 
tion, quite frequently even in the first five years (certainly after the third 
year), more rarely from the twentieth to the thirtieth year; but there are 
reports according to which tabes appeared 34 and even 38 years after infec- 
tion (the last case was confirmed by the positive cytodiagnosis of the cerebro- 



460 TABES DORSALIS 

spinal fluid ! ) . Tabes therefore generally follows a preceding syphilitic 
infection after the same interval of time as do the other forms of tertiary, vis- 
ceral, cutaneous, and mucous syphilis, and is therefore absolutely identical 
with, so-called " tertiarism " in point of time. 

The results of these statistics may be briefly summed up in the statement, 
previously made, that nearly all tabetics have previously been syphilitic, hence 
no one is likely to become tabetic unless previously infected by syphilis. 1 

Obviously the next conclusion is that this preceding syphilitic infection is 
the most invariable, essential, and, we may even say, absolute prerequisite for 
the development of tabes. 

Another deduction is that in the overwhelming majority of cases (although 
not in all) tabes is the direct sequel of syphilis, is a post- syphilitic or a posi- 
tively syphilogenous disease, also that it may represent a late form of syphilis 
of the nervous system. 

This opinion is held by most authors, by some only in a modified form, 
by a few it is directly denied; these latter will admit no connection between 
tabes and syphilis. 

It must be conceded that — in spite of striking facts — statistics alone do 
not justify this conclusion, neither do they positively prove the relation be- 
tween tabes and preceding syphilis. 

Searching for other facts and experiences to confirm this conclusion, we 
find many clinical observations of the last few decades which positively sustain 
the opinion that tabes is actually a syphilogenous disease, even though it be 
a peculiar manifestation of syphilis. I shall quote some of these. 

1. In regular, periodic sequence tabes follows syphilis in from 3 to 10, 
or, at most, 20 years, in rare cases somewhat later. This is based on the fact 
that if syphilis is acquired very late tabes will appear late; there is a series 
of observations which show that after a recent syphilitic infection in the 48th, 
54th, 55th, 57th and 68th years, tabes appeared in the 58th, 59th, 66th, 68th 
and 70th years respectively; the mere fact of the sequence of two conditions, 
in themselves rare, warrants the conclusion of an intimate connection between 
them. 

In the same sense the unusually early appearance of tabes after early ac- 
quired syphilis may be mentioned (infection at 19 years — tabes at 22 !) ; espe- 
cially noteworthy is the frequent occurrence of tabes in children {infantile 
and juvenile tabes) which may almost invariably be attributed to hereditary 
syphilis or that acquired during infancy. The observations of Rumpf, Four- 
nier, Dydinski, Brasch, Kron, Gumpertz, Babinski, Souques, myself, Linser, 
Brookshank-James, v. Bad, Nonne, and others permit not the slightest doubt 
of this. The fact is interesting that infantile tabes appears in both sexes 
with almost equal frequency, as does hereditary syphilis. 

Tabes occurring in syphilis which is still florid, as well as in the presence 
of various tertiary symptoms in the skin, mucous membranes, bones, etc., in 
tabes of prolonged existence, favors the intimate connection of both affections; 
no less so does the appearance of other phenomena of nervous syphilis, such 

1 This expression " syphilitically infected " is used here in its broadest sense, that 
the mere existence of a chancre is presumptive evidence of an actual syphilitic infec- 
tion. (Compare Erb, Aetiol. der Tabes. Volkmann'sche Samml. klin. Vortr., N. F., 
Nr. 53, p. 521, 1892.) 



ETIOLOGY 461 

as hemiplegia, paraplegia, headache with nocturnal exacerbations, etc.; all 
these things are occasionally, and by no means rarely, observed. (The patho- 
logico-anatomical facts which may be utilized in the same sense will be dis- 
cussed later.) 

The parallel frequency of case% of syphilis and tabes in men and women of 
the higher and lower classes is important; both are much more common in 
men of the higher classes than in women, while in the lower classes the propor- 
tion of women to men is very much greater; Minor has shown that syphilis 
and tabes are much rarer among the Jews in Eussia than among the Russians ; 
the same is also true of the different classes of society. Those classes which 
are most often exposed to syphilitic infection, such as merchants, commercial 
travellers, officers, etc., also furnish the largest contingent of tabetics; among 
clergymen, syphilis and tabes are hardly ever observed. The few tabetic 
clergymen that I had an opportunity of observing had all had syphilis. 

The appearance of tabes in man and wife is almost decisive; not seldom 
the}^ are affected by tabes one after the other (in a similar way tabes may 
be combined with paresis) ; in almost all such case- syphilis was demonstrated 
(Raecke found it in 90 per cent., Hudovernig in 96.3 per cent., and Pourrey- 
ron even in 98 per cent, of the cases!). It is clear that this cannot be due 
merely to living together, to deleterious effects, to excesses, etc., which cause 
tabes, otherwise among the hundreds of thousands of married couples in which 
this occurs many would be affected who are not syphilitic; but the factor of 
syphilis alone is common to all. and is etiologically of the utmost significance. 

This significance is even increased when tabes or paralysis (infantile or 
juvenile) or cerebral syphilis (so-called family tabes) is found in the children 
of such tabetic parent-. 

The extreme rarity of tabes in virgins need hardly be mentioned; but 
as a matter of fact, tabes has been observed among "virgines." Syphilis 
was almost invariably demonstrated in these cases, but this had been 
acquired either hereditarily, in childhood, or subsequently in an extragenital 
manner. 

I must devote a few words to the remarkable statement which, however, 
is not yet sufficiently corroborated, that tabetics appear to be immune from 
syphilis. In the early stages of tabes many patients are exposed to infec- 
tion, but I have never heard of recently acquired syphilis in a patient with 
severe tabes; even if such a case should be observed, the strong probability 
of its being a reinfection would decidedly weaken this proof in a negative 
sense. 1 

Among the few symptoms of the pathologic picture of tabes which indi- 
cate a syphilitic origin we must first mention paralysis of the muscles of the 
eye, which is so commonly observed, especially in the early stages of tabes, 
and is a forerunner of this affection; to every experienced eye specialist — 
provided no other deleterious factors, such as intoxication, cerebral affection, 
etc., can be detected — these suggest syphilis, and to this they are almost exclu- 
sively due. But another most invariable and significant symptom of tabes is 

1 It might be possible to solve this question by the " experimental " test reported by 
v. Krafft-Ebing at the Moscow Congress as having been made in the case of paresis. 
But in the present state of affairs this is hardly practicable. 



462 TABES DORSALIS 

of greater importance: Spinal myosis with reflex rigidity of the pupil (Argyll- 
Eobertson). Babinski and some French authors succeeded in proving that 
this condition is invariably due to syphilis, and they regard it as almost pathog- 
nomonic of syphilitic infection ; it is interesting to know that this view has been 
confirmed by cytodiagnosis of the cerebrospinal fluid. 

The lymphocytosis of this fluid, which is almost invariably found in tabes, 
and even now may be regarded as a valuable clinical symptom, will be dis- 
cussed later ; here it need only be stated that it decidedly points to the syphilitic 
nature of tabes. The condition, however, has been so recently observed that 
no conclusive opinion is as yet justified. 

Every unprejudiced observer who considers these facts, and keeps statistics 
in mind, must admit that they decidedly bear out the statement expressed 
above that " tabes is positively a syphilogenous disease " ; each of these facts, 
while not in itself absolutely decisive, strengthens the value of the others, 
and their sum makes a convincing " indicational proof " of the relation of 
tabes and syphilis. 

Nevertheless even to-day this connection is not generally recognized, and 
some prominent opponents absolutely deny it for the following reasons : 

Statistics were rejected in this investigation. The data upon which they 
were based were declared to be incorrect, and their importance was minimized ; 
to-day there can be no serious difference of opinion on this point; statistics 
must undoubtedly be recognized as a valuable aid in investigation, and in 
regard to number and quality their results are so uniform and convincing 
that no objection can now be made to them. 

It was hoped that pathological anatomists would decide these questions, 
it was believed that the tabetic changes in the spinal cord were not specific, 
and did not conform to the recognized type of syphilitic alterations (gum- 
matous or cellular infiltration, inflammation, proliferation, etc.), therefore 
could not be of syphilitic nature. It was left to these pathological anatomists 
to decide what processes were or were not syphilitic, but quite incorrectly! 
In the present state of pathologic anatomy, this will by no means prove which 
of the lesions are syphilitic, and still less so which are non-syphilitic. If, not- 
withstanding this, it has become customary — and quite properly so ! — to regard 
certain changes like small-cell infiltration, the " gummatous " products in the 
vessels, meninges, and central nervous system, as undoubtedly syphilitic, this 
view is sanctioned by custom, but pathologic anatomy can by no means furnish 
convincing proofs. It has chiefly been justified by clinical observation, and 
even to-day it is clinical observation that usually decides these questions, and 
if we clinicians say "Here are spinal forms of disease (for example, tabes) 
in which in 90 per cent, of the cases a preceding syphilis may be demon- 
strated," pathological anatomy, whether it desires to do so or not, must admit 
the alterations found to be syphilitic or of syphilogenous origin. If doubts 
arise, it is impossible to prove that these conditions, especially the gray degen- 
erations, are not of syphilitic origin. So far as I can see, pathologic anato- 
mists with increasing unanimity admit that these gray degenerations, the 
primary atrophy of the nerve tracts and nerve-cells, as well as " specific " cellu- 
lar infiltrations, may be produced by syphilis although they do not have its 
characteristic appearance. 

I have investigated this question repeatedly, first in the year 1892, and 



ETIOLOGY 463 

again in 1902/ and from weighty reasons I have demonstrated that these 
gray parenchymatous degenerations and atrophies may be considered and des- 
ignated as syphilogenous with as much justice as the so-called " specific," 
gummatous, late products of syphilis. I must refer the reader to these inves- 
tigations, and will not further discuss the subject at this point. 

But in tabes, besides the typical gray degeneration of the posterior horns, 
other conspicuous and " specific " changes are demonstrated with increasing 
frequency in the meninges, in the vessels, etc. I have compiled these cases in 
the article last referred to. Prominent French observers (Xageotte, for ex- 
ample) have lately stated that " specific " changes, such as small cell infiltra- 
tion, had invariably been found in the meninges, especially in the small veins 
of this membrane, and have declared them to be typical of syphilis. 

These authors (Pierre Marie, Xageotte, and others) with increasing cer- 
tainty ascribe the pathologico-anatomical origin of tabes to a " syphilosis " 
of the meninges, and look upon the lymphocytosis of the cerebrospinal fluid 
which is almost invariably found in tabes a- confirming this opinion. There- 
fore, this pathologico-anatomical finding decidedly points to the syphilogenous 
nature of the tabetic changes. 

Some of these facts still require confirmation and call for further research, 
but, when all is considered, the objections raised to the views of pathological 
anatomists which have been regarded as decisive cannot in my opinion be 
sustained. 

This is also true of the criticism frequently made and regarded as impor- 
tant, that tabes can have nothing in common with syphilis because specific 
antisyphilitic treatment is fruitless. Aside from the fact that this demurrer 
is objectively incorrect, since there are undoubtedly many cases in which 
specific treatment has been shown to be beneficial, bringing about an almost 
complete recovery, the fact that it cannot be cured by mercury and iodin is 
no proof of the non-syphilitic nature of the affection. Who in the world dare 
maintain that in persons previously syphilitic all forms of disease which cannot 
be cured by mercury and iodin are therefore of non-syphilitic nature? Why 
do we find upon the autopsy table so many typical cases of " specific " menin- 
gitis, of meningomyelitis and encephalitis, with gummata, with specific endo- 
carditis, with hepatitis and aortitis, even after energetic treatment with mer- 
cury and quantities of potassium iodid ? This argument had better be dropped ! 

At the onset of this discussion it was remarked that specific treatment 
had only the slightest effect upon the tabetic changes in primary or secondary 
degeneration of the nerve elements, as well as upon the insidious development, 
the long duration, and the progressive character of the malady. If specific 
treatment often fails to remove specific products and cellular infiltrations, how 
much less can we expect it to act upon the severe tissue disturbances which 
affect the nervous elements themselves ! Therefore this objection, too, is not 
sustained by the facts. 

Another criticism has been made with some degree of justice, namely, that 
compared with syphilis tabes is relatively rare ! It has been deemed expedient 

i Erb. "Die Aetioloofie der Tabes." Volkmann's Sammlung Jclinischer Yortriige, 
Xeue Folge, 1892. Xr. 53. p. 529. — Erb, " Bemerkungen zur patbologiscben Anatornie 
des Centralnervensystems." Dcutsclie Zeitschr. f. Xervenheill-iuidc. 1902. XXII. 



464 TABES DORSALIS 

to determine statistically how many syphilitics become tabetic, and how many 
tabetics were previously syphilitic. And as it is notorious of the syphilitics 
that but few become tabetic, it was triumphantly declared that, ergo, tabes 
could not be the result of syphilis. Every one familiar with the pathology 
of syphilis will at once admit that this reasoning is erroneous, and that the 
form in which the question has been put is entirely wrong. Syphilis is a 
disease with numberless early and late symptoms; a few of these are common 
and almost invariable, many are rare, a few exceedingly rare, but all are signs 
of syphilis ! Shall hepatic syphilis, syphilitic aortitis, cardiac gummata, syph- 
ilitic meningitis and meningomyelitis, syphilitic spinal paralysis, gummata 
of the brain and spinal cord, etc., be cast out from the pathology of syphilis 
and be declared to be non-syphilitic merely because they are rare sequelae of 
syphilis? Certainly not! I believe that no one will demand this. Now all 
of these conditions are more rare than tabes, some of them decidedly so. 
Therefore tabes may still be considered as belonging among post-syphilitic dis- 
eases. And this is much more likely than in the previously mentioned forms 
of disease. It has never been stated that most syphilitics become tabetic, nor 
that " where there is much syphilis there is also much tabes/' But we main- 
tain that nearly all tabetics have previously been syphilitic, and this is the 
important point, for among 100 tabetics at least 80 to 90 (perhaps even more) 
were previously infected, and this is the main question, not, however, how 
many of 100 syphilitics will become tabetic, whether one, or 2, or 5, or 10. 
It is very interesting to determine this ratio in order to appreciate correctly 
the dangers of syphilis, but not to determine the relation of this affection to 
tabes. 1 

Such a question concerning other conditions would lead to remarkable eti- 
ologic views : There are thousands of alcoholics, yet how few are attacked by 
polyneuritis or hepatic cirrhosis ! Innumerable workers in lead show the signs 
of lead intoxication, yet how few suffer from lead paralysis or contracted kid- 
ney ! Incalculable cases of diphtheria, yet how rare is post-diphtheritic paral- 
ysis ! There are thousands of tuberculous patients, yet how few develop Addi- 
son's disease ! Etiology is not thus investigated ! 

Another erroneous statement frequently made and regarded as a forcible 
objection is this — that, in certain countries in which syphilis (as well as 
paresis) is exceedingly prevalent, tabes is unknown or exceedingly rare. These 
are countries with both a wholly uncivilized and also a very highly civilized 
population, such as Bosnia, Herzegovina, and Abyssinia ; it is also true of the 
Kirghiz, the negroes of East Africa and America, and also of the Japanese. 
At first this appears to be quite remarkable; but, even though the main facts 
are correct, they must chiefly be considered from the same standpoint as the 
frequency of tabes in syphilitics in general. Such persons need not become 
tabetic, and there may be factors in the mode of life, in the greater or less 
development of these countries, to prevent it; perhaps there is a peculiar, 
modified, and attenuated form of syphilis such as is so frequently observed in 
countries where syphilis is endemic. 

i The assumption that tabes is comparatively rare among prostitutes was found upon 
minute investigation to amount to nothing: Among 36 over 25 years of age Kron found 
5 cases of tabes (but none among 148 younger ones), and Jadassohn declared in 1896 
that tabes was quite common in prostitutes. 



ETIOLOGY 465 

But these assertions are not even true, as is proven by many recent inves- 
tigations ; 1 have lately discussed this question minutely in two different arti- 
cles/ and have found that the actual conditions are quite different from what 
has been stated: In Japan there are many tabetics (Xose) giving a high 
percentage of syphilitics; tabes is not rare in Abyssinia (not more so than are 
nervous affections in Vienna, v. Halban) ; among the negroes of America it 
is always associated with syphilis, and it has been observed in Bosnia and 
Herzegovina. The Transactions of the Eighth German Dermatological Con- 
gress at Sarajevo (September, 1903) furnished an exceedingly full report on 
this subject, but it can be critically discussed only after the full report of the 
Congress, which it is hoped will bring to light some very interesting facts and 
considerations. 

1 shall not here enter into details, but, leaving this exotic tabes to the 
critics, I shall limit myself to the consideration of tabes as it exists in Europe 
and America, particularly among men in the better walks of life; what has 
been shown to be true and invariable among these will probably also prove true 
of the lower classes and the opposite sex. 

The compilation of the foregoing leads me to the conclusion that for the 
previously mentioned portion of the population tabes is undoubtedly a syphilog- 
enous affection. 

But this does not clear up the whole question; the etiology of tabes is not 
yet exhausted. Are there not other causes or may these be excluded? These 
matters must be investigated. 

It is no doubt true that but relatively few syphilitics later become tabetic ; 
how many we do not as yet know; perhaps one or two, possibly three to five 
per cent, or even more; this proportion varies according to the different regions 
and the different classes of society. 

Wliy do so few become tabetic? 

This brings us to the question of the etiological factors and the auxiliary 
causes of tabes in syphilitics. Only a few new ones can be added to the causes 
which have been known since 18S0, and the importance of which will now be 
inquired into. In 1S92 I collected reports of nearly 300 cases of tabes which I 
attempted accurately to classify, and made an investigation 2 to which I here 
refer. Eecently Schittenhelm 3 published a similar investigation with corre- 
sponding results. I may, therefore, be brief. 

(1) Direct heredity plays but a slight role in tabes. This is not remark- 
able, since tabes now appears to be an " exogenous " disease ; otherwise, con- 
sidering the great number of tabetics who for a long time are capable of rear- 
ing children, tabes would be more frequentty transmitted from father to son 
or from mother to children. In the quite isolated cases in which this has 
been observed, syphilis was demonstrated in both generations, therefore noth- 
ing was proven as to the heredity of tabes. It also leads us to form an opin- 
ion regarding so-called " family tabes " ; that is. the occurrence of tabes among 
children of the same family, or in parents and children, etc. In the not infre- 

i Erb. " Syphilis und Tabes." Offener Brief an Prof. v. Krafft-Ebing, Jahrbnch f. 
Psych, n. Xeurologie, 1902. — Erb, " Syphilis und Tabes." Berliner klin. Wochenschr., 
1904. Xr. 1-4. 

2 L. c. Yolkmann'sche Sammlvng, 1892. 

3 Schittenhelm, Deutsche Zeitschr. f. Xcrvenhlc, 1903, XXIV. 

31 



466 TABES DORSALIS 

quent cases of tabes in brothers, syphilis was almost invariably demonstrated 
(I published a number of such cases) ; in cases in which both parents and 
children suffered from tabes (such cases are described in my last report — 1904), 
syphilis of the parents and hereditary syphilis of the children was always 
demonstrated. But although this transmission may perhaps be explained by 
the peculiarities of the syphilitic poison, nevertheless we cannot deny the fact 
that in some families there is a certain predisposition to tabes when syphilis 
already exists. 

(2) Such cases should be classified as due to neurotic predisposition, the 
importance of which in the development of tabes was greatly exaggerated by 
Charcot and his pupils. If every nervous affection, even in distant relatives, 
is to be regarded as the sign of a neuropathic predisposition, in the case of 
tabes these will be found to be very common — just as in most other diseases. 
I found this condition in scarcely one-third of the cases (28 per cent.), while 
Fournier found it in only 25 per cent., Mobius, Oppenheim, Voigt, and Eehlen 
only in 9 to 14 per cent. This indicates no noteworthy influence. I observed 
a nervous predisposition in the patient in 42 per cent, of the cases, but even 
this proportion is unreliable, and is only slightly in excess of - what every 
nervous specialist nowadays finds among his male patients over 25 years of 
age. No doubt, however, the growing nervous predisposition and nervous- 
ness of our times may have some effect by diminishing the resistance of the 
nervous system, and thereby increasing its susceptibility to the syphilitic 
poison. 

It is certainly not permissible for us to confine a congenital endogenous 
predisposition to tabes alone, which view culminates in Benedikt's statement, 
" Tabicus non fit, sed nascitur." We need merely to consider the effect of this 
statement upon the etiology and pathology of tabes, as we know them to-day, 
to appreciate its rashness. Are persons perhaps born with a predisposition 
to syphilis? 

(3) Chilling of the body, by some authors considered the chief deleterious 
agent, is unquestionably a cause of tabes. Even when we consider how inci- 
dentally cold is often mentioned by the patients as a cause, and is so accepted 
by physicians, when we consider how many patients for years regard their 
pains to be " rheumatic," and are of the firm opinion that they must have 
taken cold, even though they cannot accurately state when — nevertheless a 
certain number remain in whom exposure to cold immediately preceded the 
first symptoms of tabes, and the conjoint and prolonged effect of a damp and 
cold work-room, living-room, or sleeping-room, of laboring in a swamp, or 
in water, or snow, of cold journeys during the winter, and the like, may be 
properly regarded as predisposing to the affection; but all this is observed 
almost exclusively in those previously syphilitic. Moreover, in my statistics 
a short interval is mentioned only in 34.5 per cent, of the cases, and for rea- 
sons above stated this figure is probably too high. In any case we cannot 
maintain that taking cold is the most important or the sole cause of tabes. 

(4) The consensus of opinion among recent authors is that sexual excesses, 
which were at first regarded as almost exclusively the cause of tabes, and which 
have again been brought forward prominently by Moczutkowski, apparently 
play but a slight role. I found this cause mentioned only in 15.8 per cent., 
but this proportion is perhaps somewhat too low because the facts are so diffi- 



ETIOLOGY 467 

cult to determine. Undoubtedly this cause is operative in individuals who 
commit sexual excesses, as in young married people who return from their 
honeymoon with the first tabetic symptoms, and especially, as I have repeatedly 
noticed, in elderly men who have married young wives. 

(5) Similar conditions are noted after bodily over-exertion and long 
marches, for example, in the army, while hunting, upon fatiguing journeys, 
during military maneuvers, in Alpine climbing, bicycling, etc. Also from 
excessive exercise among so-called leg-workers, those who must stand or walk 
the entire day; for example, waiters, policemen, postmen, etc. I found this 
was supposed to be the cause in 27 per cent, of my cases; this, however, was 
not wholly correct, as is borne out by the investigations of Kron in sewing 
machine workers. But the view is quite justified that all of these occupations 
debilitate the nervous system, and lessen its power to resist the syphilitic poison. 

Here we will briefly consider Edinger's substitution hypothesis, according 
to which the increased combustion of material by work, function, and exertion, 
etc., is not fully compensated and thereby leads to defective nutrition and to 
degeneration of the functioning elements. Under some circumstances, espe- 
cially when syphilis, anemia, impoverished nutrition, or constitutional affec- 
tions, etc., coexist, this lack of restitution produces characteristic tabetic degen- 
erations in the central nervous system. This was Edinger's ingenious and 
convincing hypothesis for a number of functions in tabes as well as for their 
disturbance. But in my opinion a much better basis of support is necessary; 
for thousands and thousands of persons daily experience these effects, yet 
do not become tabetic, unless they are simultaneously syphilitic. And innu- 
merable persons are debilitated by anemia, tuberculosis, digestive diseases, 
anxiety, and sorrow, yet do not become tabetic from these same deleterious 
influences unless they are also syphilitic. Edinger, as I know personally, is 
in entire accord with the view that syphilis is the most essential and perhaps 
the sole cause of tabes, but he thinks his theory explains the fact that definite 
areas of the spinal cord and nerve tracts become susceptible to the attacks 
of the syphilitic poison, and from this cause the typical picture of tabes, 
sometimes in isolated cases also the atypical picture, appears. Of course, 
this needs to be definitely proven: at present, it seems to depend much more 
upon the specific action of the syphilitic virus than upon the nature and 
localization of the functions which are overtaxed. 

(6) Mental exertion, over-exertion in any occupation, excitement, and 
psychical emotions, which are often mentioned by patients as the causal fac- 
tors, appear to act in the same way ; they have a depressing effect, damaging 
the entire nervous system, and thus reducing its power of resistance to 
syphilitic intoxication. I can give no accurate figures concerning this point. 

(7) Traumata of various kinds, a fall from a horse or from a great height, 
severe shock to the body, the fracture of a leg, continuous shock for years 
in certain occupations, accidents upon the railway, or in some occupation, 
etc., are frequently mentioned, and the recent laws concerning accidents have 
an added importance thereto. Several authors (E. Schulze, Spillmann and 
Parisot, Trommer, Klemperer, and others, and finally v. Leyden) favor this 
view. Others (Hitzig, Mendel, Collins. Windscheid, and Schittenhelm) have 
sharply criticized it. Even the circumstance that trauma precedes less than 
5 per cent, of all cases of tabes assigns to this factor a subordinate role in its 



468 TABES DORSALIS 

etiology. Cases in which syphilis has certainly not preceded, and in which 
before the trauma tabetic symptoms were positively absent — in which, there- 
fore, the genetic connection could not be proven — are exceedingly rare; the 
majority will not withstand strict investigation. Taking into consideration 
the cases of chronic progressive diseases of the central nervous system, such 
as progressive muscular atrophy, amyotropic lateral sclerosis, chronic an- 
terior poliomyelitis, multiple sclerosis, syringomyelia, and the like, which 
occur in connection with trauma, we must admit at least the possibility of 
a pure traumatic tabes; but it is certainly extremely rare. It appears, how- 
ever, to be proven that when syphilis exists such a trauma may occasionally 
lead to the development of tabes, that the first tabetic symptoms are apt to 
follow it, and that very often, if tabes is beginning to develop, such a trauma 
decidedly aggravates and accelerates its course. 

(8) The effect of certain poisons must also be considered. In addition to 
ergotin which, as Tuczek ascertained by thorough investigation, produces a 
disease of the spinal cord somewhat resembling tabes but not identical with it, 
and which may be designated as " ergotin tabes," I must also mention " alco- 
holic tabes," which is merely a peripheral multiple neuritis, some of the symp- 
toms of which resemble tabes. 

Several authors have maintained that mercury is a "tabes poison," and 
that syphilitics who have long been treated with large doses of it are apt 
to develop locomotor ataxia. Fournier and others have disproven this ridicu- 
lous assumption, and Neisser and P. Cohn have also lately shown it to be 
absurd; to say nothing of the fact that tabes never appears as a sequel of 
chronic mercurial poisoning (in mirror workers, etc.), it has been demonstrated 
that by far the overwhelming majority of tabetics have never been treated 
by Hg, or very incidentally and for a short time. This should be sufficient 
to stamp this constantly expressed opinion with its true value. 

But the question is quite permissible whether poisons such as alcohol and 
tobacco taken immoderately have any influence upon the development of tabes. 
I find them mentioned in only about 18 per cent, of the cases, a figure which 
appears to be too low. That either of these poisons alone is capable of pro- 
ducing tabes I believe to be exceedingly unlikely, and, considering that we 
have recognized another specific poison as the chief cause of tabes, I think 
this may be absolutely excluded; on the other hand, the fact that these 
poisons may possibly prepare the soil for the syphilitic noxa, or in combina- 
tion with it may produce tabes, seems very likely. Further researches must 
be undertaken to solve this question. 

(9) Little is known of other diseases, such as infections, etc., which may 
cause tabes. Tabes may occasionally appear after such affections provided 
they have decreased the resistance of the nervous system to the syphilitic virus. 
The experience of the last 15 years has taught others as well as myself that 
influenza, which was at first regarded as a very mild malady, has this effect ; 
it is certain that if influenza be added to tabes (as is also the case with many 
other diseases) it decidedly aggravates the affection and accelerates its course, 
and there is no doubt that the first tabetic symptoms occasionally appear after 
an attack of influenza; but whether this alone without preceding syphilis 
will produce tabes is doubtful, and remains to be proven. At present I am 
ignorant of such an occurrence. 



ETIOLOGY 469 

(10) Gonorrhea, which I recently 1 brought into this discussion, is still to 
be mentioned. It is true statistics show only 50 cases of tabes, in 90 per 
cent, of which there was a history of gonorrhea (of course 88 per cent, of 
syphilis also). In the male population of the higher classes (600 cases), 
only 50 per cent, were found to have gonorrhea. Close research, however, 
shows that since almost all the tabetics previously had syphilis only those 
cases could be used for comparison in which there had also been syphilis or 
chancre, and among these there were 75 per cent.; therefore there is not a 
sufficient difference to indicate the predominating influence of gonorrhea in 
the origin of tabes. This is also shown by another investigation which reveals 
that of 45 tabetics with gonorrhea 90 per cent, were simultaneously syphilitic, 
and of 265 cases of gonorrhea which were not tabetic, only 34.7 per cent. 

My present opinion is that it is most unlikely that gonorrhea has any 
influence upon the development of tabes. Nevertheless the relation of these 
diseases should be further investigated; the combination and cumulation of 
"poisons," of "toxins," is possibly of importance. Here I may refer to the 
opinion Hitzig expressed in 1892 that tabes is perhaps not due to the actual 
poison of syphilis but also to some other specific poison, and that these are 
simultaneously transmitted, occasionally also with the toxin of soft chancre 
(perhaps with that of gonorrhea?). In this connection who does not think 
of the remarkable theory of " syphilis a virus nerveux " ? 

Nothing is now known of other injurious factors as causes of tabes, but 
it is self-evident that those which have been enumerated may occasionally exist 
in great numbers. There are many persons who expose themselves to all 
the injurious effects of over-exertion and cold, also drink and smoke to excess, 
and pass their nights in gambling or with women, etc., and who readily 
succumb to tabes provided they have been previously infected. A number of 
tabetics have admitted this to me. 

In considering the numerous conditions which may be the " cause " of 
tabes, the presumption is obvious that tabes may frequently result from the 
combined action of several, and it is well to decide which are the most impor- 
tant; statistics show a certain degree of uniformity. They indicate that in 
nine-tenths of all the cases of tabes there is a syphilitic infection, in about one- 
third tabes is preceded by exposure to cold, in about one-fourth by over- 
exertion, in about one-sixth by sexual excesses, in about one-twentieth by trau- 
mata, and that more than one-third of all tabetics belong to neurotic families. 

This shows the powerful influence of syphilis, and it becomes still more 
evident when we endeavor to ascertain whether, and in what proportion, these 
causes alone are operative. In 1892 I published a Table which shows that 
in 281 cases of tabes the disease was preceded by : 

Syphilis alone in 27.0 per cent, of the eases 

Neuropathic predisposition alone 

Refrigeration alone. 

Exertion alone 

Sexual excesses alone 

Trauma alone 

i Erb, Berliner klin. YYocTienschr., 1904, Nr. 1-4. 



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1.4 


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470 TABES DORSALIS 

This fully demonstrates the predominating influence of syphilis. It also 
shows that the other damaging factors alone are unimportant, and that 
almost without exception they are active only when combined with syphilis 
(which is also shown by the Table published at the same time). 

These facts and considerations lead to the conviction that syphilis is the 
most common and chief factor in the development of tabes. In the over- 
whelming majority of cases it is the main cause, beside which all others, 
widely divergent in themselves, are merely auxiliary factors and predisposing 
causes. 

Curiously enough, tabes frequently follows mild, rapidly healing forms of 
syphilis which have been almost without symptoms, and have had little or no 
treatment. This should be investigated. Certainly experience has proven that 
even a thorough and long-continued specific treatment will not absolutely 
prevent a subsequent outbreak of tabes, a fact which is true also of some of 
the other forms of the tertiary stage. 

The recognition of syphilis as the principal cause of tabes sheds light upon 
the peculiarities and the frequent occurrence of the disease; this was clearly 
set forth by Mobius in his article on "Tabes" (1897) as follows: "This 
accounts for the appearance of tabes in middle life, rarely in the aged and in 
children; its overwhelming frequency in men, its extreme rarity in women 
of the higher, its greater frequency in those of the lower classes; its uniform 
occurrence in children of both sexes ; its prevalence in large cities, the liability 
of certain occupations, and the exemption of others (that of theologians!). 
I advise the reader to investigate these features for himself. They are highly 
convincing." 

This makes it clear that civilization with all of its nerve-damaging conse- 
quences, with its increased facilities for debauchery and luxury, with its wide 
distribution of syphilis, its tendency to over-exertion and irritation of the 
nervous centers, etc., has an essential influence upon the development of tabes, 
and that for this affection, as v. Krafft-Ebing tersely remarked in Moscow 
of progressive paralysis, " syphilization and civilization " are the causative 
factors. This explains the great rarity of tabes among uncivilized people, even 
when they are thoroughly infected with syphilis. 

But, after all, it cannot be denied that there are cases of tabes in which 
the most careful research will disclose no trace, and will even refute the possi- 
bility, of syphilitic infection. Upon close investigation the 10 per cent, of 
non-infected in my chief Table is decidedly reduced, for in many cases there 
is a possibility, or likelihood, of syphilitic infection. Three per cent, at least 
are beyond suspicion, and I have seen not a few cases (about 5 per cent.) in 
which the most minute questioning revealed absolutely no cause for the affec- 
tion, neither syphilis nor any other factor. What is to be done with these 
cases ? 

As is well known, Mobius energetically maintains, and for excellent reasons, 
that under all circumstances tabes is a meta-syphilitic (syphilogenous) disease, 
a primary atrophy of the nervous elements of which syphilis is the conditio 
sine qua non. 

All the facts lead to the conclusion that only a poison with chronic action 
can be the cause of tabes. This applies to the poison of syphilis, which in 
itself is just as specific and peculiar as is tabes as a disease. There is little 



SYMPTOMATOLOGY 471 

likelihood that in pathology some other poison produces exactly the same dis- 
ease; certainly there is no example of it. Consequently, syphilis with its poi- 
sonous effects must be regarded as the exclusive cause of tabes. 

I must admit that Mobius's reasoning is very forcible. The small propor- 
tion of tabetics in whom no syphilis can be demonstrated does not prevent us 
from regarding tabes as exclusively due to syphilis; our knowledge of syphilis 
occulta and the fact that in from 30 to 65 per cent, of the cases of undoubted 
late syphilis (gummata, etc.) there is no evidence of preceding infection prove 
this. But the number of " doubtful " cases is so great that we cannot at once 
ignore them on merely logical grounds. It is still possible that tabes may be 
produced by unknown infections, by bacterial or other poisons, or a combina- 
tion of various baneful factors, and although such poisons may be unknown 
to-da}' they may be discovered to-morrow. We have not yet reached the end, 
but only the beginning, of knowledge regarding such action of poisons. 

Although I incline very strongly to Mobius's theory, and believe it more 
than likely to be true, yet I adhere to my reiterated opinion that there is as 
yet no absolute proof that tabes is invariably a syphilogenous disease. 

So far as I can see, the result of these discussions concerning the cause 
of tabes, and the conclusion which is to-day shared by most experts, are as 
follows: " That, among all the causes of tabes, syphilis is by far the chief and 
most common, that tabes in the overwhelming majority of cases — perhapsf 
in all — is due to syphilis, that it is, however, a relatively rare sequel or mani- 
festation of syphilis, and that other deleterious factors are auxiliary and pre- 
disposing causes for its development." 

How syphilis acts, what is the nature of the tabetic disease, what its patho- 
genesis, and what part the so-called auxiliary causes play, will be explained 
after we have discussed the pathological anatomy of tabes. 

We will first consider the symptomatologic picture of the affection. 



III. SYMPTOMATOLOGY 

Since my comprehensive description of the pathologic picture of tabes 
appeared in Ziemssen's Handbook (1876) many additions, although perhaps 
not of vital importance, have been made to it, and in many ways the symp- 
tomatology has been minutely elaborated and made more accurate. 

To avoid being prolix, I will delineate as succinctly as possible the individ- 
ual features which form the nucleus of the pathologic picture, and will empha- 
size their fundamental and most important points; special consideration will 
be given to new view points, those which aid in the diagnosis, and the symp- 
toms which are most important to the practitioner ; the numerous and variable 
svmptoms which are not typical will form a framework surrounding the many- 
sided, interesting, and inexhaustible symptom-complex. 

As a rule, we first note in tabes an introductory, prodromal, neuralgic, or 
pre-ataxic stage, the duration of which may vary from a few months, which 
is rare, to many months or several years, occasionally even decades, 1 the dis- 

1 Within the last few years I have seen two eases in which typical lancinating pains 
with objective tabetic symptoms had persisted for fully 22 to 24 years without a trace 
of ataxia. 



472 TABES DORSALIS 

ease terminating on the appearance of the characteristic and peculiar motor 
disturbance known as ataxia. 

This pre-ataxic stage is characterized by a series of subjective disturbances, 
localized in various regions, which usually introduce the affection; the most 
prominent are peculiar disturbances of sensation, lancinating pains, pares- 
thesia of the lower extremity and of the trunk, a feeling of exhaustion or of 
uncertainty in the leg, weakness of the bladder and sexual organs, finally the 
disturbance of certain cranial nerves (the nerves of the muscles of the eye — 
diplopia, strabismus; the optic nerve — amblyopia, feebleness of vision) ; on the 
other hand, there are a number of significant objective symptoms which, even 
from the beginning, permit the recognition of the severe affection: Reflex 
rigidity of the pupils, absence of the patella tendon reflex, swaying upon clos- 
ure of the eyes, and certain typical sensory disturbances in the legs and in the 
trunk (definitely localized hyperesthesia of the feet and of the trunk, hyper- 
algesia, analgesia, and the slowing of pain conduction, regional hyperesthesia 
to cold, etc.) ; finally, strabismus, double vision, and amblyopia from atrophy 
of the optic nerve. 

The disease may set in with any of these symptoms. Sometimes one or 
another subjective or objective symptom may precede for a varying time, and 
then other symptoms are added. Certain symptoms, however, almost invariably 
introduce the affection, while others are less constantly noted. 

To the former belong extremely typical and lancinating neuralgic (or 
lightning-like) pains: Acute, stabbing, boring, darting, paroxysmal pains, 
localized sometimes in one region, sometimes in another, often persisting in 
the affected area for hours, sometimes for days. They are of varying inten- 
sity, often appearing as sudden, flashing, but very mild sensations of contrac- 
tion; often the attacks are of almost unbearable severity, and by their fre- 
quency and duration they keep the patient in misery (tabes dolorosa) ; mild 
stroking, the touch of the clothes, etc., will usually, to a greater or less degree, 
increase the hyperesthesia, while firm pressure over the painful spots will often 
give relief. They are sometimes localized in the skin, frequently in the deeper 
soft parts, in the bones, and in certain nerve regions (the sciatic nerve) ; almost 
invariably they first attack the lower extremities — the calves, the shins, the 
dorsum of the foot, and the thigh; they then extend to the trunk where they 
appear as most severe girdle pains, and to the thorax; rarely, at least at the 
onset of the affection, they attack the upper half of the body (the arms, in 
the ulnar nerve), the neck, or even the face (tabes superior). They always 
appear to be influenced by changes in the weather (by rain, fog, wind, the 
first snow, a low barometer, storm, or humidity), therefore are most frequent 
in the spring and autumn ; for this reason they are usually regarded as " rheu- 
matic," and many patients never refer to them as "pain" but as "rheuma- 
tism ! » 

Over-exertion, shock, strong emotion, sexual intercourse, exposure to cold, 
etc., are especially liable to produce pain. 

Every physician of experience will easily recognize these tabetic lancinat- 
ing pains from their localization and the above described severity, and will 
at once differentiate them from rheumatic or true neuralgic pain — and this 
is very important. They form an almost invariable symptom of the early 
stage, are present in about 90 per cent, of all the cases, and in the majority 



SYMPTOMATOLOGY 473 

(about 70 per cent.) form the first symptom; sometimes these pains exist for 
years and even decades without any other symptoms. 

Paresthesia, which is rarely absent, follows the pain in varying forms and 
localization. There is formication, numbness, or a furry feeling in the soles 
of the feet and the legs which makes the patient feel as if he were walking 
upon carpet, wool, inflated rubber, or the like ; also hot or cold sensations, and 
a feeling of tension around the joints of the foot and knee as if these were 
encased in apparatus, etc. Sometimes their localization is very peculiar : They 
appear in the region of the peroneus nerve, upon the anterior surface of the 
thigh (the lateral cutaneous femoral nerve), are especially typical and dis- 
tressing around the anus, in the perineum, in the scrotum, or in the vulva, 
and are here referable to the lower segments of the spinal cord; the path of 
the ulnar nerve in the forearm and hand is especially liable to these sensa- 
tions, and this has led to the term " ulnar nerve sensation " ; they have also 
been occasionally observed in the face along the course of the fifth nerve (a 
feeling of numbness, etc.). 

Perhaps those paresthesias are most characteristic which occur upon the 
trunk as the familiar girdle sensation: That peculiar paresthesia which, like 
a broad zone or girdle, encircles the trunk at various heights (the thorax, 
epigastrium, umbilical region, hypogastrium), and manifests itself as formi- 
cation, or, more frequently, as a disagreeable sense of tension or constriction 
as though the body were in armor, which in some patients becomes almost 
unbearable. As stated, it is occasionally felt around the joints of the knee 
and the foot, and also in the calves. 

Some patients have reported an increased sensitiveness to cold water and 
the like, that they suffer from hyperesthesia of the shin, usually upon the 
trunk, and particularly on changes of temperature; that in the dark and espe- 
cially when ascending stairs their gait is uncertain, and that early in the 
morning when washing the face they readily lose their balance and fall, etc. — 
the subjective appearance of Komberg's symptom. 

Disturbance of the ocular muscles, which becomes apparent to the patient 
as indistinct vision, double vision, or ocular vertigo, is an early accompaniment 
of the disease, frequently appears long before Eomberg's symptom as a single 
premonitory sign, and probably indicates a preceding localized syphilis. These 
are pareses and paralyses of the muscles of the eye which usually implicate the 
oculomotor and abducens nerves, more rarely the trochlear ; they are frequently 
transitory and fleeting, continuing in varying intensity for a few days, or weeks, 
or even months, and then disappearing, or they may be permanent. 

Some patients report that one or the other pupil is dilated, that reading 
is prevented by disturbances of accommodation, and the like; but these condi- 
tions must be accurately determined by an objective examination. 

In a certain proportion of the cases (10 to 15 per cent.) there is a slow 
or rapid but progressive decrease in the acuity of vision-, a dimness and nar- 
rowing of the field of vision, and a diminished color sense; usually this in- 
creases rapidly and continuously, and leads to permanent blindness; occasion- 
ally, however, there are long periods of arrest, even of improvement in the 
condition, or it is limited to one eye, and may not attack the other for years. 
This symptom frequently precedes the appearance of the other tabetic phenom- 
ena, perhaps for 10 years. 
32 



474 TABES DORSALIS 

Following this are subjective signs in the legs — fatigue, loss of activity 
in walking and decreased endurance in standing — a slight feeling of uncer- 
tainty, especially in the dark, which at first is unnoticed; usually these symp- 
toms are referable to the sensory sphere, perhaps to paresthesia of the sensory 
nerves of the muscle; but unquestionably there is often a decrease in motor 
activity, until at last ataxia distinctly but gradually develops. 

Among the most common phenomena of the first stage (appearing in about 
80 per cent, of the cases) are disturbances of the urinary and sexual func- 
tions, usually paretic conditions, rarely irritative phenomena. 

In the bladder these are manifested chiefly as dysuria, as hyperesthesia 
at the neck of the organ, sometimes also as neuralgic pain, more frequently 
as paresis. At first there is a tendency to retention, to a slow discharge, or 
to dribbling, very rarely to rapid micturition — or there may be a moderate 
degree of incontinence, the frequent necessity of immediate relief, sometimes 
in the clothes, or in the bed. This disturbance is usually slight, rarely so 
marked that the patient is obliged to use the catheter or to carry a urinal, 
but this occasionally happens, or is persistently the case, and cystitis soon 
develops. 

Analogous functional disorders of the rectum are more rare; the most fre- 
quent complaint of the patients is that, on account of hypesthesia of the anal 
region, they are unaware of an approaching evacuation, and therefore, espe- 
cially if there is diarrhea or purgatives have been taken, the discharges are 
apt to be involuntary. 

There are almost invariably disturbances of the sexual functions even in 
the first stage of tabes. Irritative phenomena do not often appear, but the 
chief symptom is sexual weakness which shows itself by a decrease of sexual 
power, incomplete erection, premature ejaculation, a feeling of fatigue and 
weakness as well as pain post coitum; finally there may be pollutions and 
spermatorrhea without erection, etc. Libido may be undiminished. The con- 
dition terminates in complete impotence, but this may occur very late; I know 
tabetics during the course of whose disease the sexual functions were more or 
less unimpaired for years, yet — often to their detriment — they practised inter- 
course more frequently than was advisable, and raised a number of children. 

In rare cases there is increased sexual excitability, even satyriasis, at the 
onset of the affection; this is seldom accompanied by increased potency, but 
usually with all the signs of beginning sexual debility which is likely to become 
more marked. 

We rarely hear of these conditions in tabetic women; as a rule there is a 
decrease of sexual desire and sensations of lust during coitus; but a tabetic 
prostitute once admitted to me that during the first stage of her disease there 
was increased libido sexualis. Menstruation, pregnancy, and the puerperal 
period are not affected by tabes dorsalis. 

In contrast with the principal subjective symptoms of the first stage are 
certain objective symptoms which are usually noted early, and permit the cer- 
tain diagnosis of tabes; the first four to be mentioned are the most invariable 
and important. 

First we observe reflex rigidity of the pupil (Erb) with or without myosis 
(the so-called Argyll-Robertson symptom). This is one of the earliest and 
most common symptoms of tabes, and has been the subject of innumerable 



SYMPTOMATOLOGY 475 

investigations. It occurs in at least 80 to 90 per cent, of all cases, appears 
very early, sometimes in advance of any other symptoms, and, as a rule, per- 
sists after it has once appeared. 

The pupil, while retaining its power of sight, contracts neither directly 
nor indirectly to light, therefore is rigid to light, while in convergence and 
accommodation for near objects it distinctly contracts. Occasionally there is 
no contraction, and we then speak of total pupillary rigidity, which is not so 
important as the pure reflex form. The tabetic pupil shows no reaction to 
light either upon direct or indirect stimulation. The recognition of the con- 
dition is not difficult, but it presupposes accurate investigation and some prac- 
tice. Accommodation must be excluded, and the pupil must be examined with 
a sufficiently powerful light (daylight or lamplight, with or without a re- 
flector) ; the corneal reflex, the extreme narrowness [pin-point] of the pupils, 
and a dark color of the iris, may cause some difficulty, but this may be over- 
come. Sometimes reflex rigidity is unilateral ; frequently it reveals itself only 
by sluggishness and a very slight reaction, which, however, in youthful per- 
sons is of great significance. (The very rare paradoxical reaction of the pupil 
— i. e, apparent dilatation of the pupil to light — is of no practical importance, 
and probably must be referred to processes of accommodation.) 

Keflex rigidity of the pupil is almost always combined with an abnormal 
size, often with more or less extreme contraction, of the pupil — myosis. The 
pupils may then be reduced to J or one mm. in diameter, and the so-called 
" stabbing glance " is produced. To this smallness of the pupils the term 
" spinal myosis " has been given. It is the most common pupillary anomaly, 
and in spite of a narrowness of the pupil which is often astonishing it con- 
tracts further upon accommodation. Myosis and reflex rigidity, however, do 
not necessarily accompany each other; they are substantive symptoms which 
appear at different times, and each may exist alone; usually reflex rigidity is 
first noted, more rarely it is combined with myosis. Occasionally, however, 
there is unilateral or bilateral mydriasis, particularly when total pupillary 
rigidity is present. The difference of the pupils in tabes is of much less sig- 
nificance than in paralysis. Occasionally the tabetic pupil is changed in form, 
it may be distorted, elliptical, or the like, but this is unimportant. As I was 
the first to maintain, in the pupil which is rigid to light there is a permanent 
absence of the sensory pupillary reflex, i. e., of dilatation of the pupil to sen- 
sory cutaneous irritation; therefore the pupil is reflexly rigid in every sense. 
We shall refer later to the exceedingly abstruse question of the accurate local- 
ization and pathogenesis of these pupillary phenomena. 

The second objective symptom of paramount importance is the absence 
of the tendon reflexes, particularly of the patella tendon reflex (WestphaPs 
symptom), that is, the utter impossibility of producing it (even by means of 
the Jendrassik method), as may readily be done in normal persons provided, 
of course, there is no disturbance of motility, no atrophy of the quadriceps, 
no peripheral nerve disturbance, etc. This also is one of the earliest and 
most common symptoms, appearing in 90-95 per cent, of the cases, and is of 
the greatest diagnostic import ; it may be unilateral or bilateral, usually 
appearing merely as a unilateral decrease and then totally disappearing; or it 
may occur late, in well developed tabes may be absent for a long time, even for 
years, and in rare cases it may be retained. I need scarcely describe the tests 



476 TABES DORSALIS 

for this condition, but they are best made with the patient in either the recum- 
bent or sitting posture so that the knee is flexed almost at a right angle, 
with a complete relaxation of the quadriceps; a practised observer will rarely 
encounter any difficulty. But this test is frequently perplexing in non-tabetics, 
in whom it is sometimes impossible at once to evoke the patella tendon reflex 
(from inability voluntarily to relax the quadriceps, from too close attention, 
etc.) ; experience, however, enables us properly to appreciate even these cases. 
Recently our investigations have ceased to be limited to the patella tendon re- 
flex, but now embrace all of the tendon reflexes, and valuable data concerning 
them have been obtained. 

Above all in importance are the tendon Achilles reflexes. These are apt 
to disappear on one or both sides synchronously with the patella tendon reflex, 
sometimes even earlier, or they may remain unimpaired longer than this. The 
act that the tendon Achilles reflex may be evoked in almost all normal persons 
(Sarbo) is of special value in the early diagnosis of tabes. The test is best 
made with the patient in the lateral position, with the knee flexed, the ankle- 
joint flaccid, and the sole lightly supported by one hand; we then tap with 
the percussion hammer at various points upon the Achilles tendon from the 
calcaneus upward (usually but one point is active) ; finally, we try Jendras- 
sik's method. The reflex is also well developed while the patient kneels upon 
a chair, supporting himself with his hands. According to our present knowl- 
edge, the absence of the Achilles tendon reflex (especially its unilateral ab- 
sence) is just as important a symptom as that of the patella tendon reflex. 

In the upper extremities also the tendon reflexes may be decreased or 
absent in tabes, but the tendon and periosteal reflexes in the wrist- joint and 
the triceps tendon reflex are of little importance. Frenkel maintains that the 
absence of this reflex is just as important, perhaps occurs earlier, and is even 
more invariable than the absence of the patella tendon reflex, but this is 
certainly incorrect. The reflex in normal persons is by no means constant, and 
is absent in only a few tabetics during the first stage. The triceps reflex, 
which originates in the upper thoracic and cervical segments, is absent only 
in so-called tabes superior, and its unilateral absence is, under some circum- 
stances, of great significance and exceedingly valuable. 

The third and most familiar objective symptom is swaying upon standing 
with closed eyes, the so-called Romberg's symptom; it is exceedingly com- 
mon, occurring in over 90 per cent, of the cases, is significant, and easily 
evoked and recognized. The patient should stand still, best with his feet in 
juxtaposition (for the final test the patient may stand upon one foot), and is 
then told to close his eyes, whereupon we observe a more or less distinct 
swaying, a sort of pendulous movement about the axis of the body, which 
increases and, if extreme, makes the patient feel in danger of falling, or he 
actually falls. It is undoubtedly to be attributed to a disturbance of the 
so-called muscular sense, that complicated centripetal sensation originating in 
the skin, the joints, the tendons, and especially the muscles, which is essential 
for maintaining position in space, for preserving the equilibrium of the body, 
and for the erect position of the trunk, and depends upon the uncertainty pro- 
duced by complicated muscular action while standing quiet; in tabes it usu- 
ally appears early, long before actual ataxia. It is the first expression of those 
disturbances of the muscular sense which implicate the sensation of posture 



SYMPTOMATOLOGY 477 

of the members, sensations as to bulk, and the direction of movements pro- 
duced by them, as well as of the passive movements arising therefrom, condi- 
tions to which I will revert in discussing disturbances of sensibility. 

The disturbances of sensation in the skin and in the muscles — form a fourth 
important group of symptoms in the first stage, embracing many individual 
phenomena which, from their nature, peculiarity, and localization, are charac- 
teristic and of great value. In the last 25 years our knowledge of these has been 
greatly amplified. 

Beginning with the lower extremity, sensation may be tested by the sim- 
plest . methods, but it should be carefully done and frequently repeated ; it 
usually requires much time, and is exceedingly tiresome to both the investi- 
gator and the patient. (Tactile sensation may be sufficiently tested by the 
brush or by slight contact with the finger, with any smooth or rough sub- 
stance, or we may use the head or the point of a pin; for the sensation of 
pain the prick of a pin, sharp pinching of folds of the skin, or the faradic 
current with Erb's electrode may be used; the temperature sense may be 
tested by contact with test-tubes filled with hot or cold water, or with glass 
or stone paper-weights, etc. ; for sensations of space any well known method 
may be employed.) 

It will usually be apparent that, in the first stage, the tactile sense has 
suffered but little or not at all; sensation is slightly decreased in the sole 
of the foot and in the toes, at the sides of the foot, on the anterior surface 
of the leg, and occasionally up to the knee and thigh. Sometimes in the late 
phases of the disease we observe a decided diminution or complete loss of the 
tactile sense which extends to the trunk, perhaps to the upper extremity and 
the head. 

Disturbance of the pain sense is almost invariable and of more importance; 
in the stage of onset we frequently note hypalgesia which sometimes amounts 
to complete analgesia-, a fold of the skin may be pierced by a coarse needle, yet 
the patient be quite unaware of it. Folds of skin upon the dorsum of the foot or 
in other areas may be pinched hard (which is exceedingly painful to normal 
persons), yet the patient is conscious of nothing more than contact; at all 
events there is no pain. Not rarely this hypalgesia is confined solely to areas 
of the lower extremities and the trunk. Combined with this, or existing 
independently of it, there is a delay in the perception of pain, which amounts 
to \ or to J of a second, or to 2 seconds or more, and is followed by an acute 
sensation of pain, sometimes by an actively increased after-sensation. In the 
cases in which pain sense is decidedly retarded but not actually decreased, upon 
painful stimulation of these parts there is a so-called double sensation : First, 
that of contact, then of pain. 

The conditions in regard to far ado- cutaneous sensibility are somewhat 
different ; this is usually analogous to the pain sense ; i. e., it is decreased 
below the minimum for sensation, as well as for pain ; even powerful currents 
no longer cause pain (see Erb " Electro-Therapeutics ") ; feeble currents anal- 
ogous to tactile sensation are only rarely perceptible, and strong currents pro- 
duce no pain. This method may, therefore, be utilized for accurate tests, and 
for comparing the results of other tests. 

The temperature sense upon the whole corresponds to the sensation of pain ; 
not rarely it varies somewhat for heat and cold. 



478 TABES DORSALIS 

Except for lancinating pain in the leg hyperesthesia is rare; occasionally, 
however, there is a moderate hyperesthesia to cold, similar to that observed in 
the trunk. Here and there a so-called polyesthesia is observed; i. e., a variety 
of sensations upon mere contact, also an allochiria in which the sensation is 
referred to the wrong side; these are of no practical significance. 

Quite analogous conditions are found in the upper extremity in later stages 
or in tabes superior: Mild or extreme hypesthesia, hypalgesia and analgesia, 
thermal hypesthesia, etc., these are usually limited to the ulnar nerve in the 
forearm and the inner surface of the upper arm (the median cutaneous 
branchial, etc.), and thus indicate the involvement of the upper dorsal seg- 
ments and root area (eighth cervical and first dorsal roots). 

Actual astereognosis is rarely noted in tabes ; in the later stages it appears 
in the hands, especially if muscular sensation is greatly disturbed, often to an 
extreme degree and in striking contrast to the tactile sense, which is still fairly 
well retained. On the other hand the frequently demonstrable analgesia of the 
ulnar nerve — absence of sensation upon firm pressure on this nerve above the 
the elbow-joint, this in normal persons usually producing acute pain — is 
worthy of mention. A similar condition is observed in the peroneal nerve 
behind the head of the fibula. 

Analgesia of the testicle, which has lately been emphasized, is quite analo- 
gous. In contrast to the normal conditions, firm pressure upon the testicle 
produces no pain. In women there is mammary analgesia. 

Especially worthy of note, and of equal practical and theoretic impor- 
tance, is the degree of sensibility in the trunk. Hitzig, subsequently Laehr, 
Marinesco, Dejerine, and others demonstrated that, especially in the earlier 
stages of tabes, there are girdle-like zones of hyperesthesia and anesthesia 
upon the trunk, and from their localization these conform to the " root type " 
— that is, the area of distribution of the various higher dorsal posterior roots 
corresponding to the segments of the spinal cord to which they belong — and 
therefore permit the recognition of a decided "root predisposition." These 
zones, as broad as the hand or broader, encircle the thorax like a girdle either 
completely or incompletely, beginning at various isolated areas — at the scapula, 
the axilla, the cardiac or gastric region — and gradually forming a girdle which 
is often unequally developed upon the two sides of the body, being higher on 
one side than on the other ; or two or three such girdle zones may be situated 
one above the other, and be separated by cutaneous areas in which sensation 
is good. They may readily be recognized by tactile and pain tests because in 
this form they rarely occur in any other disease, and they demonstrate most 
convincingly the root and segmental origin of these tabetic anesthesias. Since 
these have been minutely studied, we know that the same conditions are found 
in the extremities. In this region, however, they are not always so distinct, 
because the various root innervation areas are situated one above the other, and 
are frequently not equally implicated in the disturbance. To this category 
belong the paresthesias, above mentioned, which are localized in the buttocks, 
at the anus, in the perineum, and in the genitalia, these being the root areas of 
the conus terminalis. 

Another valuable and common sign which appears upon the trunk of tabet- 
ics, and to which I have lately attached decided significance, is a conspicuous 
hyperesthesia to cold which is noted in the trunk, the abdomen, and the back, 



SYMPTOMATOLOGY 479 

and may reach, an astonishing degree. The patients recoil on coming into 
contact with any cold substance, often crying out aloud, and making strong 
resistance, declaring that the sensation of cold is extremely unpleasant and 
even painful to them. They notice that they no longer bear cold water, are 
no longer able to take a cold bath, etc.; it is true there are some healthy 
persons to whom cold applied to the abdomen and the sides is more or less 
unpleasant, but it never reaches the extreme degree observed daily in tabetics. 
Hyperesthesia for heat is more rare and much less conspicuous, but, even when 
present, it is not so marked as hyperesthesia for cold. 

These are the disturbances of cutaneous sensibility which are apt to be 
found in the first stage of tabes, — more or less well developed, with variations 
and degrees in nature, intensity, and distribution. With the progress of the 
affection all these signs increase, and a high-graded anesthesia or analgesia 
may be distributed over the entire body, — even up to the face and the top of 
the skull. 

Passing to the disturbances of deeper sensibility (inaccurately designated 
muscular sensibility) we find the Romberg sign which has already been dis- 
cussed. It is usually most distinct in the earlier stages, and other symptoms 
are gradually added which, as a rule, can only be determined by minute and 
painstaking investigation. We note primarily that the patients have more or 
less completely lost the sensation of posture and position of their limbs, 
especially of the legs; with closed eyes or in the dark, they do not know 
where their legs are; if in the recumbent posture, whether they are hanging 
out of bed or not; if one leg is passively placed in a certain position, they 
are unable to bring the other leg into exactly the same position, which normal 
persons can do with great precision; furthermore, they lose all control of the 
measure and direction of movements which they arc asked to perform, pro- 
vided their eyes are closed or it is dark. The movements then are aimless, 
excessive, shooting far beyond the mark, apparently because the patients have 
no clear idea of the direction of the movement to be made. 

The sensation for passive movements of the limbs is also more or less com- 
pletely lost in such patients. Even energetic passive movements of the vari- 
ous joints are either recognized imperfectly or not at all, and when their eyes 
are closed these patients are absolutely ignorant of the position in which their 
limbs have been placed. This test should always be made in the lower ex- 
tremities, but is even applicable to the upper extremity which may show similar 
changes. In the small joints of the finger it has led to very interesting theo- 
retical conclusions which, however, are practically of little value. 

Finally, we may mention the absence of the sensation of fatigue which 
tabetics report (for example, the patients can hold their arms for a compara- 
tively long time in one position without feeling tired). The latest observation 
in this realm is the following: The arrest of sensation in the bones, determined 
by testing with a tuning-fork the sensation of vibration which appears in the 
bones (Egger). Besides these invariable and important objective symptoms of 
the first stage, investigation frequently reveals other conditions, some of which 
will be briefly mentioned. 

Chief of these are the cutaneous reflexes; they deviate but little from 
the norm, and only when there is marked disturbance of the tactile sense are 
they at all parallel with it. The plantar reflexes are sometimes retained and 



480 TABES DORSALIS 

as active as under normal circumstances, or they are diminished, or are absent ; 
the less the tactile sensation in the sole of the foot, the more the plantar reflex 
is decreased; with retarded pain conduction a prick in the sole of the foot is 
sometimes followed by a delayed reflex. The cremaster reflex is evidently 
longer retained than the reflex of the sole of the foot. On the other hand, the 
abdominal reflexes (as is well known there are at least three of these at various 
heights of the abdomen) are usually present and conspicuously active, no mat- 
ter what the cutaneous sensibility of the abdomen; with a very thick belly, a 
profuse layer of fat, or with very flaccid and atonic abdominal walls, they are 
of course absent, as in normal persons. 

In tabetic paralysis of the muscles of the eye the findings are more signifi- 
cant, and are frequently referable to the oculomotorius or some of its branches 
or to the abducens, much more rarely to the trochlear nerve; they manifest 
themselves by the familiar disturbances of binocular sight, double vision, ver- 
tigo, paralytic strabismus, etc., and are readily determined by ophthalmological 
tests. The manner of their appearance varies; sometimes there is a paresis, 
at other times a profound paralysis of one muscle, or several, or all of them, 
unilaterally or bilaterally, with or without ptosis. They are usually transi- 
tory, lasting for a few days or weeks, or at most months. They generally 
appear very early, as the first symptom, or in the first stage of the disease. 
Subsequently relapses may be frequent and the condition may become perma- 
nent. They usually indicate nuclear changes, while primary lesions in the 
peripheral nerves of the muscles of the eye are rarer. The associated paralyses 
of the muscles of the eye, which appear in the later stages as more or less com- 
plete external ophthalmoplegias, are of serious import and indicate nuclear 
atrophy. When they occur in persons of middle age with a history of preced- 
ing syphilis but without accompanying symptoms, they awaken our suspicions 
of beginning tabes ; this is true of the much rarer, usually unilateral, ophthal- 
moplegia interna accompanied by mydriasis, general pupillary rigidity, and 
paralysis of accommodation. 

Of most serious import for the patient is progressive tabetic atrophy of the 
optic nerve which fortunately occurs only in a small proportion of the cases — - 
about 10 to 15 per cent. In two-thirds of all cases this severe complication 
appears in the pre-ataxic stage. When patients complain of feebleness of 
vision, or veiling of the field of vision, etc., the physician finds a distinct, and, 
as a rule, rapidly progressive decrease in the acuity of vision, soon combined 
with a loss of color sense (first red-green, then yellow-blue) up to complete 
color blindness [Daltonism] ; simultaneously there is a limitation of the field 
of vision beginning peripherally, and often progressing in a typical manner. 
More or less rapidly beginning amblyopia changes to complete amaurosis, first 
in one eye, sooner or later but invariably also in the other eye. It is charac- 
teristic that the pupils are frequently narrowed and rigid, instead of being 
widely distended as in other cases of amaurosis. 

The ophthalmoscope shows the cause of these disturbances to be the so- 
called white atrophy of the optic nerve. At first we notice a grayish, subse- 
quently a white, glistening appearance of the sharply defined papilla, with 
contracted arteries and veins, yet no change in the retina, a picture which is 
readily differentiated from the choked disc of simple neuritis and of optic 
neuro-retinitis, and from the temporal pallor of the papilla in multiple sclerosis. 



SYMPTOMATOLOGY 481 

It is noteworthy that patients with optic atrophy which appears early 
often present a mild form of tabes, and the former seems to have an inhibi- 
tive effect upon the development of the latter. Such patients have compara- 
tively few symptoms — little pain, and but slight bladder disturbance ; if ataxia 
develops it is late. This remarkable and inexplicable circumstance has been 
noticed by man}' observers as well as by myself. 

Motion still remains to be investigated; in the first stage, provided there 
are no rare symptoms (neuritis, atrophy, arthropathies, etc.), it is, as a rule, 
but slightly disturbed; coarse power is apparently well retained, and may be 
considerable; the exactness and certainty of movements, such as marching, 
leave nothing to be desired. On the other hand, we note in the motor activity 
of the patients an early but distinct general loss of the power of sustained 
movements. They soon tire, can no longer walk much, cannot stand so long 
as formerly while at work, and, in a fair proportion of these cases, in the first 
stage a certain, distinct, muscular weakness gradually develops. The famil- 
iar description of an abrupt " giving way of the legs " must be regarded either 
as a sudden symptom of exhaustion or as the effect of a fleeting, momentary, 
lancinating pain. The rare pareses and paralyses which are limited to individ- 
ual nerve and muscle regions will be described later. 

In this stage there are no quantitative or qualitative changes in the electric 
contractu 'it y of the motor nerves and muscles which can be pratically utilized. 

But, even in this stage of tabes, if the muscles are powerfully developed, 
the easy movability and flaccidity of the members is very conspicuous. Muscu- 
lar tension is never seen in genuine tabes. Passive movements of unusual 
extent may be performed with the greatest ease, which shows that the tonus 
of the muscles has decidedly decreased ; this usually becomes noticeable in the 
second stage from the fact that the legs of such patients can readily be moved 
in any direction. The extended leg may without difficulty be flexed so far 
at the hip-joint that the thigh almost touches the abdomen, the foot reaches 
the head, etc. This phenomenon, which has long been observed, which was 
mentioned by Leyden, myself, and others, and which Frenkel thoroughly stud- 
ied, has lately been appropriately designated by him as " hypotonia of the 
muscles." 

It consists in a decrease, or the entire absence, of inhibition for certain 
active and passive movements, and is due to abnormal flaccidity of the mus- 
cles, without any change in the joints, ligaments, or tendons. These patients 
can extend the leg at an angle of 90° or even more from the hip- joint, whereas 
the normal person can scarcely reach more than 50° to 60° ; with extended knee 
pressed upon the underlying surface of a couch the heel can be raised some 
distance from this surface (hyper-extension at the knee-joint) ; in standing 
genu recurvatum is conspicuous, and the same abnormal movability is apparent 
in almost all of the joints and muscle groups (the leg and foot, the adductors 
and rotators of the thigh, the vertebral column, the muscles of the trunk, the 
joints of the hand and fingers, etc.), as was so clearly shown by Frenkel. 
Therefore, tabetics are able readily to execute movements which are impossible 
for normal persons unless they are professional dancers, clowns, or " contor- 
tionists." 

When the muscles are powerfully developed and firm, this hypotonia is 
usually invariable in the first stage, and sometimes appear very early; it 



482 TABES DORSALIS 

by no means runs parallel with sensory disturbances nor those of the reflexes ; 
it exists for a long time without ataxia; the latter is certainly not the conse- 
quence of hypotonia, but the hypotonia greatly influences the posture of the 
body (genu recurvation, forward flexion, and laxity of the trunk, etc.), there- 
fore also the ataxia. Frenkel includes it among the pathognomonic and initial 
symptoms of tabes. It is probably due to a decrease of the reflex muscle tonus 
conveyed by special reflex tracts. 

In the first stage of tabes, therefore, the constitutional condition of the 
patients appears to be entirely undisturbed, their vegetative functions are nor- 
mal, the vertebral column shows no change, the brain functions normally and 
intellection is unimpaired, provided no complications are present. 

Numerous symptoms, however, which appear in almost all cases in addi- 
tion to these constant and typical ones, we shall not at present touch upon, 
but shall first consider the most important motor symptom of tabes — the 
ataxia. This introduces the second stage of the disease. 

In the later course of the first stage, usually after the passage of years 
or decades (up to 25 years or more), but sometimes after only a short dura- 
tion of the affection — 3 to 6 months — new motor disturbances appear: There 
is a want of certainty and precision in the movements of the limbs, the patients 
become awkward, or combined and complicated movements are performed with 
increasing difficulty and are less exact; in a word, tabetic disturbance of co- 
ordination — ataxia — appears, and the patient enters the " ataxic stage " of the 
affection. Sometimes, however, after a more or less prolonged prodromal stage, 
ataxia may develop within a few days or it may suddenly appear during the 
night. 

It is characteristic of tabes and one of its main features that the pre- 
cision of combined and finer movements is decidedly decreased, while the 
motor power and the strength and certainty of individual movements have 
suffered little if at all. Therefore, it is not a paralysis, but a disturbance of 
the coordination of movement. Even if we occasionally demonstrate a decrease 
of motor power, or a distinct paresis, the disturbance of movement due to the 
ataxia is in no proportion to it. 

Almost invariably ataxia begins in the lower extremities, and here it is 
typical and uniform. In its mildest degree there is some uncertainty and 
irregularity in walking, as shown by unequal steps and an uncertain position 
of the feet ; in more difficult movements, such as rapid turning, there is slight 
swaying; running and jumping, the ascent or descent of stairs, stepping upon 
a foot-stool, walking on a smooth floor or on a line, become more difficult. 
This is most apparent in the dark or on closure of the eyes. On standing 
with the feet in juxtaposition, we note swaying, and contractions in the anterior 
muscles of the leg; standing upon one foot is uncertain, or even impossible. 
In the recumbent posture there may be no evidence of motor changes. 

These mild grades are readily diagnosticated by minute investigation; the 
patient should be directed to sit down slowly and then to rise, to flex the knee, 
to stand up quickly upon command and begin to walk, to halt suddenly while 
walking, to walk in a straight line, to stand upon a chair, to turn rapidly, to 
stand with the feet close together or upon one foot, to ascend or descend stairs 
without the aid of the banister, finally, to walk backward ; in all of these move- 
ments there is considerable uncertainty and swaying, especially if the patient 



SYMPTOMATOLOGY 483 

performs them with the eyes closed, and thus has no assistance from the sense 
of sight. 

Gradually the disturbance becomes more marked, the tip of the foot is pro- 
jected forward and outward, the heel stamps upon the floor, the knees are 
stiff and hyperextended, the body is bent forward, the eyes are riveted on the 
feet and the floor; the steps become unequal and jerky, the route in walking 
more and more irregular, finally, the gait becomes uncertain, aimless, and 
waddling. Thus is developed the characteristic ataxic gait which enables us 
to recognize the tabetic at some distance on the street, and which differs so 
markedly from the spastic and paralytic gaits. In standing the disturbance 
becomes more pronounced, especially when the feet are together, the patients 
oscillating to and fro. Spasmodic movements are noted in the legs and trunk, 
and the patients are unable to remain quietly in one position — a condition 
which has been designated static ataxia, in contrast to the " locomotor form/' 
and which also apppcars when the patient is sitting still, or when he quietly 
extends the legs or the arms. - 

An examination with the patient in the recumbent posture also reveals 
ataxia: Slow and uniform movements are impossible, or they are performed 
paroxysmally or interruptedly ; even the mere raising of the leg is done in an 
irregular, zigzag way, the raised leg falls at a spot more or less remote from 
the place intended; a given object can be touched with the great toe only 
after many irregular, secondary movements; instead of describing a circle 
with the tip of the foot the figure becomes angular, and often irregular ; touch- 
ing the knee with the heel of the opposite foot — " the knee-heel test " — is only 
possible by means of awkward contractions which often cause the foot to shoot 
far beyond the mark; the heel can be slowly moved along the tibia of the other 
leg only with the utmost difficulty. Here it is evident that the ataxia, espe- 
cially the irregularity of movement, becomes more distinct when these maneu- 
vers are performed with the eyes closed, but only when noteworthy disturb- 
ances of the so-called muscular sense are simultaneously present; if these are 
absent, the closure of the eyes has but little effect upon the ataxia, and it is 
also evident that, if well marked, the ataxia is not prevented by the careful 
use of the eyes but at most is merely decreased. 

In the most extreme grades of ataxia, walking and standing finally become 
impossible; the legs lose their hold, are projected in all directions, if left to 
himself the patient falls, and even if supported upon both sides or carried the 
legs wabble about wildly. Spasmodic and paroxysmal movements invariably 
appear, and the patient can no longer assume the position necessary for stand- 
ing or walking. Yet coarse power may be quite well retained, although the 
patients may have become entirely helpless, and are confined to their beds or 
to a rolling chair. 1 

Ataxia usually appears much later in the upper extremities, only the cases 
of tabes superior being exceptions to this rule. Ataxia, of course, interferes 
with all of the finer movements for which the hands are constantly used, such 
as buttoning the coat, tying a cravat, embroidering, cutting, writing, draw- 

i For other details see the works of Frenkel ("Die Behandhmg der tabischen 
Ataxie," Leipzig, 1000) and Otfried Forster ( " Psysiologie und Pathologie der Coordi- 
nation," Jena, 1902) ; these authors have made careful and exhaustive investigations of 
this subject. 



484 TABES DORSALIS 

ing, piano-playing, etc. The movements become uncertain, spasmodic, awk- 
ward, zigzag-like, etc., particularly if they are performed without the aid of 
the eyes (buttoning the suspenders at the back!). Ataxia becomes distinctly 
evident when the patient tries to touch with his finger the tip of his nose or 
an object held in front of him, or if the fingers of both hands, being some 
distance apart, are gradually approximated at their. tips (" index finger test ") ; 
when they attempt to grasp an object held in front of them, the fingers are 
drawn apart and awkward grabbing is noted, as well as when they try to 
describe figures in the air with the finger, such as circles, numerals, etc., and 
in writing and piano-playing — all movements are irregular, aimless, jerky, and 
angular ! 

Finally, the hands become useless for the purposes of daily life; the pa- 
tients can no longer eat or dress without assistance, can hold nothing in the 
hands, and, in fact, are quite helpless. 

Ataxia may also appear in the muscles of the trunk: The harmonious 
action of the muscles necessary to maintain the equilibrium in standing, 
sitting, and in many movements, becomes disturbed, and the bodily move- 
ments are irregular or wabbly. The same is also true of the muscles of 
the neck and the nape. 

In rare cases even the muscles of the face, of the lips, and of the tongue 
are not exempt : This is shown by certain grimaces, especially in laughing and 
speaking, in the impairment of the powers of mimicry, of speech, and of mas- 
tication. Ataxic nystagmus, however, appears to be a manifestation of Fried- 
reich's ataxia only. 

Ataxic movements have frequently been observed in the muscles of the vocal 
cords. 

It is of the utmost importance that these ataxic disturbances of movement 
be differentiated from all related conditions. This, as a rule, is easy, difficul- 
ties arising only in rare cases when similar disturbances of movement appear 
in other diseases. But the mere differentiation of tabes from the other symp- 
toms, usually no arduous task, will preserve us from error. 

Above all, it must be borne in mind that ataxia is a disturbance of volun- 
tary movements, and does not appear during rest; this alone distinguishes it 
from chorea minor, from paralysis agitans, and the other forms of tremor; 
the only disturbance of movement with which it may occasionally be con- 
founded is the intention tremor of multiple sclerosis, which also appears on 
voluntary movement. In this condition, however, there are regular oscilla- 
tions around the axis of movement; in ataxia there are irregular movements, 
zigzags and angles in walking, which by no means resemble tremor. More- 
over, the accompanying symptoms of these affections are absolutely decisive. 
But it should not be forgotten that true ataxia may occasionally occur in mul- 
tiple sclerosis, and even be combined with intention tremor ! 

The ataxia of Friedreich's disease (" hereditary ataxia ") is almost identical 
with that of tabes. Naturally, the experienced observer will detect some 
differences, but these are not marked (possibly more waddling and un- 
certainty in walking, a marked and early implication of the arms, distinct 
disturbance of speech, ataxic nystagmus, marked "static" ataxia, etc.). 
The disease should be clearly recognized from its other symptoms: Its ap- 
pearance in childhood, in several children of the same family, the absence 



SYMPTOMATOLOGY 485 

of sensory disturbances and pain, normal state of the pupils, the absence of 
syphilis, etc. 

The differentiation of cerebellar ataxia from spinal tabetic ataxia is much 
more simple; the gait in the former is unlike that of the latter, being of a 
more waddling character, resembling that of a person under the influence 
of alcohol, and the individual movements are quite different in character, being 
coarser, more complete, and less zigzag in appearance. The arms rarely show 
any sign of this, and, instead of the typical symptoms of tabes, we note those 
of disease of the cerebellum. 

This exhausts the description of the second stage of tabes, ataxia being 
its only differential and essential symptom. 

Investigators have busied themselves with the pathogenesis of this symp- 
tom, and in trying to explain its occurrence have referred it to pathologico- 
anatomical changes intimately connected with the preceding and accompany- 
ing sensory disturbances; but, unfortunately, from the earliest times to the 
present with most unsatisfactory results. We shall revert to this when describ- 
ing the anatomical changes in tabes. 

Xot much more can be stated in regard to the second stage. The symp- 
toms of the first stage continue, and some of them become more distinct and 
intensified, as, for example, the sensory and bladder disturbances and the pain ; 
not infrequently this stage is complemented by the addition of other symptoms. 

We now come to the discussion of some rare and unusual symptoms of 
tabes, no less interesting and remarkable, which complete the typical picture of 
the two preceding stages, rendering it extraordinarily rich in types and varieties. 

These phenomena are almost innumerable, but they all belong to tabes; 
volumes might be filled with their enumeration, exact description, and analysis. 
We shall attempt a brief outline, arranging them according to their frequency 
and importance. They may appear in any stage of tabes — early or late — and, 
for the sake of simplicity, these stages will be discussed together. 

Visceral Crises. — I must first mention the peculiar phenomenon to which 
the term visceral crises has been attached. Those longest known and most 
familiar are the so-called gastric crises which were mentioned by Gull (1858) 
and Delamare (1866), and introduced into the pathology of tabes by Charcot's 
classical description (1868). 

These consist of sudden attacks of severe " gastric pain" occurring at vari- 
ous intervals, radiating into the epigastrium, the abdomen, the lateral aspect 
of the body, and the back; the pain is intense, it is girdle-like, boring, screw- 
ing, or contracting in character, and is accompanied by moans and cries, gen- 
eral prostration, and a feeling of extreme illness which makes the patient 
utterly wretched. Uncontrollable vomiting soon follows, first of food, then of 
mucus and gastric juice, rarely of blood. The patient is unable to take the 
slightest amount of food because it is at once vomited; there is unquenchable 
thirst — briefly the picture of intense suffering. This may last many hours, 
for several days, sometimes even for two or three weeks, new attacks con- 
stantly appearing, and only gradually disappearing. Vomiting is not invaria- 
ble. It may be slight or absent, and the attack may be limited to the pains, 
the general prostration, and the disturbed ingestion of food. Owing to the 
pain, the vomiting, inability to retain food, and the insomnia, the patients lose 
flesh and become greatly debilitated. 



486 TABES DORSALIS 

The attack ceases more or less suddenly, the patient feels relieved, is able 
to eat and drink and to retain food, and he recuperates in the shortest time 
and rapidly increases in weight. 

During the intervals between the attacks the stomach, as a rule, is abso- 
lutely normal, but there may be slight dyspeptic symptoms — a mild gastric 
catarrh — such as the patient has had before the attack. 

The examination of the vomitus and the gastric juice during the attack 
shows varying conditions; in some cases marked hyperchlorhydria and hyper- 
secretion, in other hypochlorhydria, even complete anacidity. These are appar- 
ently of little importance, and the remaining investigation reveals nothing 
essential. The epigastrium and abdomen are markedly contracted and more 
or less sensitive to pressure; there is no splashing sound, no tumor, nothing 
to indicate an ordinary gastric affection. 

The intervals are of varying length, weeks, months, or years, but in the 
individual case the attacks usually show a certain periodicity, and increasing 
frequency ; mild attacks may alternate with severe ones ; they are easily recog- 
nized, and with some attention may readily be differentiated from true gastric 
affections. Formerly these attacks were always regarded (as they are some- 
times to-day) as " gastric spasm," ulcer, or gall-stone colic, and were so treated 
until they reached the neurologists. Now they are treated in sanatoria for 
gastric diseases at Carlsbad, Kissingen, Homburg, and other spas, and, as a 
rule, correctly ; the examination of the pupils, of the tendon reflexes, etc., for 
tabes renders the diagnosis positive. 

These " gastric crises " appear in many cases of tabes, and form one of the 
most severe symptoms of the affection. They may occur at any stage of the 
disease, sometimes at the onset of the affection. Their explanation is still 
uncertain, their point of origin unknown, and the view that they are analo- 
gous to severe attacks of lancinating pain is probably correct ; but this analogy 
extends no further. As slight, rudimentary forms we may note pyrosis, sali- 
vation, burning in the esophagus, singultus, irritation of the throat, causeless 
nausea, and the like. 

Similar to gastric crises are intestinal crises. These consist of attacks of 
severe colicky pains with copious and stubborn diarrhea; either symptom ma), 
however, occur alone, and neither in itself is characteristic; in the intervals, 
the intestinal functions are absolutely normal. Intestinal crises are, upon the 
whole, much more rare than gastric crises. 

Analogous to these are so-called rectal crises, which come on with severe 
pain, a burning sensation as of a hot plug in the rectum, combined with tor- 
menting tenesmus, and with muco-purulent discharges. This may be asso- 
ciated with similar symptoms on the part of the bladder — vesical crises — a 
condition which has frequently been described as neuralgia anovesicalis, an 
exceedingly painful strangury and tenesmus. We also frequently note urethral 
crises in which the pains are localized chiefly in the urethra, and are combined 
with tenesmus, severe and burning pain upon micturition, the pain radiating 
to the perineum, to the thighs, the hypogastrium, and the anus. 

Attacks have been described as renal crises which may simulate renal colic ; 
there is severe pain which is difficult to differentiate from colic clue to calculi 
if there are no objective nor subjective phenomena of tabes. 

Furthermore, we observe testicular crises, paroxysmal and severe attacks of 



SYMPTOMATOLOGY , 487 

pain in one or the other testicle. In women so-called clitorial crises appear, 
either in the form of attacks of pain localized wholly in the vulva, the region 
of the clitoris, the urethra, or the bladder, and in characteristic sexual orgasms 
with painful sensations of lust, a feeling as of coitus with subsequent discharge, 
and simultaneous or subsequent prostration and exhaustion. These attacks 
may also occur at night in the form of " feminine pollutions " with sensations 
of pain. 

In the organs of the throat and chest " crises " are quite common. The 
most common and important are the so-called laryngeal crises, first described 
by Fereol in 1868, which may be more or less severe. The onset is sudden, 
there is a feeling of tickling, a spasmodic contraction of the neck with severe 
dry cough, suffocation accompanied by stertorous respiration, unconsciousness, 
pallor, and cyanosis ; sometimes an epileptic attack occurs, and may be danger- 
ous to life. Usually, however, the spasm of the glottis ceases in a few minutes, 
consciousness returns, and the attack is relieved. 

In mild cases there is a spasmodic cough which resembles whooping cough; 
the respiration is noisy, the cough is paroxysmal, and a tough foamy mucus 
is expectorated. This may recur with great frequency. 

Especially severe attacks of tickling in the throat, arrest of respiration, 
unconsciousness, and epileptic attacks, have been described as ictus laryngeus. 
These laryngeal attacks are sometimes combined with a paralysis of the mus- 
cles of the larynx which will later be described. 

Oppenheim has designated as pharyngeal crises painful sensations in the 
pharynx frequently combined with movements as in deglutition. 

Tracheo-bronch ial crises have also been mentioned. They are analogous 
to the laryngeal attacks, and are accompanied by spasmodic cough, and diffi- 
culty in respiration and deglutition. 

Finally, cardiac crises — more or less severe attacks of angina pectoris which 
may be referred to a disturbance in the tract of the pneumogastric nerve — 
have been observed in tabes. Since the possibility of another cause, such as 
sclerosis of the coronary arteries, cannot be excluded in these patients, this view 
is not incontestable. 

We now turn to another series of phenomena referable to the motor appa- 
ratus, and chief among these are the " atrophies, paralyses, and atrophic paral- 
yses " in the various nerve and muscle regions of the body. 

Dejerine lately called attention to a form of muscular atrophy which, 
although previously described, was never sufficiently appreciated. He believes 
that this forms an integral factor in about 20 per cent, of the cases of tabes; 
it is symmetrical, usually develops early and insidiously in the extremities, 
more markedly in the lower, but occasionally also in the trunk, and may 
finally reach an extreme degree. As a rule it appears in the late course of the 
disease, and is not rarely observed in the pre-ataxic stage. In the lower extrem- 
ity it begins in the small muscles of the foot, where it produces weakness and 
paralysis, a claw-like position of the toes, a sort of " tabetic equinovarus," then 
it passes to the leg, especially to the peroneal region, then to the calf and to the 
thigh, finally some of the muscles become contracted, and a deformity of the 
feet becomes permanent. 

In the arms this atrophy begins in the small muscles of the hand, produces 
the characteristic ape-hand or claw-hand, etc., and slowly involves the forearm 



488 TABES DORSALIS 

and upper arm. Similar conditions are occasionally noted in the trunk and 
in the muscles of the shoulder-blade. 

The paralysis of the muscles is in exact proportion to the atrophy; the 
paralysis therefore does not precede. Fibrillary contractions are absent. Elec- 
tric contractility is either decreased or absent; as a rule, there are no DeR. 
Finally we have the common picture of ataxics — people who are greatly ema- 
ciated, with wabbling legs and sometimes wabbling arms. 

The nature and pathogenesis of these diffuse atrophies is still uncertain; 
they resemble poliomyelitic and other spinal amyotrophias less than they do 
mere wasting of the muscles, or the terminal stages of chronic, neuritic, mus- 
cular atrophies. The latter possibility is favored by the autopsy findings, 
which, in addition to degeneration of the muscles, chiefly show degenerative 
changes in the peripheral nerves, the anterior roots not being implicated. 

Furthermore, there are many local paralyses and atrophies which may 
occur in any region of the body. 

The more or less slight paralyses of the muscles of the eye which occur 
so frequently in the early stages have already been mentioned ; but in all stages 
of the affection severe and permanent paralyses of varying nature and combi- 
nation may be observed. There may be complete paralysis of individual nerves, 
of the oculomotor and abducens, either unilateral or bilateral, and with or with- 
out ptosis, with marked strabismus, with paralytic contracture, also paralysis 
of associated muscles: The lateral rotators, the raisers and depressors of the 
bulbus, of both internal muscles, etc., and this, which unquestionably indicates 
a nuclear origin, may develop into a more or less complete ophthalmoplegia 
externa in one or both eyes. The details cannot be described here. 

Not rarely we note in addition an ophthalmoplegia interna, a complete 
paralysis of the muscles of the iris, usually with mydriasis and paralysis of the 
muscles of accommodation. These are usually persistent and incurable. I 
must reiterate that the variations in, and the possible combinations of, these 
symptoms are extraordinarily great. 

It is remarkable that in tabes the facial nerve is scarcely ever attacked by 
paralysis, although it has been noted in rare cases (syphilitic facial paralysis?) ; 
usually it is localized in the muscles of the lips as a partial phenomenon of 
"bulbar paralysis." 

More frequently, but still quite rarely, do the muscles of mastication become 
paretic and atrophic; this is usually only in combination with other bulbar 
forms of paralysis. 

Paralyses of the muscles of the larynx (therefore of the vago-spinal acces- 
sory region) are much more common, and have lately been thoroughly studied; 
of the most frequent form, posticus paralysis in tabes, there is already quite 
a voluminous literature. It may be unilateral or bilateral; in the latter case 
it shows itself by a disturbance of the respiration, by dyspnea, and by an 
inspiratory stridor which appears upon exertion, and not infrequently may 
lead to laryngeal crises. These may be so serious and menacing to life that 
tracheotomy becomes necessary ; I saw a patient of this kind, with his tracheal 
cannula, who lived for six years afterward; the laryngoscope at once reveals 
the affection. Other paralyses of the muscles of the larynx are also noted: 
Unilateral or bilateral paralysis of the vocal cords with hoarseness and aphonia, 
and also unilateral or bilateral paralysis of the recurrent laryngeal nerve. 



SYMPTOMATOLOGY 489 

(But little has been reported of anesthesia of the larynx in such cases.) 
Very rarely isolated paralysis of the external branches of the spinal accessory 
with paralysis and atrophy of the trapezius and sternocleidomastoid is observed. 

Finally, we come to the rare paralyses of the hypoglossal nerves in tabes. 
Unilateral atrophy of the tongue and its drastic consequences (atrophy, wrin- 
kling, and decreased size of the affected half of the tongue, deviation of the 
tongue to the atrophic side, fibrillary contractions, and probably DeE) are 
known and have been described. But bilateral atrophic paralysis of the tongue 
occurs, as a rule, as an accompanying phenomenon of bilateral bulbar paralysis. 

Combined, distributed, and usually progressive, paralysis of the cranial 
nerve is by no means rare in tabes : Sometimes in the form of nuclear paralysis 
of the muscles of the eyes, sometimes combined with paralysis of the muscles 
of deglutition, etc., sometimes as typical progressive bulbar paralysis (tongue, 
lips, and pharyngeal paralysis), but also occasionally combined with paralysis 
of the muscles of mastication and those supplied by the spinal accessory. And 
even these numerous forms may be so interwoven with each other that the 
pathologic process appears to implicate all of the motor nerve nuclei of the 
trunk of the brain (III to VII-X-XI1). The previously mentioned unfortu- 
nate patient, who for the last six years of his life wore a tracheal cannula, not 
only had almost complete bilateral ophthalmoplegia externa and interna with 
ptosis, but also total atrophic paralysis of the muscles of mastication, severe 
progressive bulbar paralysis with extreme atrophy of the tongue, and, finally, 
complete paralysis of the muscles of the pharynx. During the last 14 months 
of his life he was nourished entirely through the stomach-tube. In addition 
to typical tabes he had partial atrophic paralysis in all four extremities. 

With increasing frequency recently paralyses of individual nerves of the 
extremities, with and without atrophy of the muscles or DeR, have been demon- 
strated; they may be transitory and curable, but are often permanent; they 
are most likely to be found in the peronei and radials, but also in the tibial, 
median, ulnar, and other nerves. They chiefly impress us as a form of periph- 
eral neuritic paralysis, but may sometimes indicate disease of the gray anterior 
columns. 

Little is to be said of the irritative motor phenomena in tabes; I have 
already stated that contractures and spasmodic conditions are usually absent. 
Here and there we note a certain unrest of the limbs which prevents the 
patients from keeping their arms and legs quiet for a moment, or other marked 
spontaneous movements may appear which in some cases assume the charac- 
teristics of athetosis ; but in the main this is rare. 

A few notes in regard to disturbances of other cranial nerves in tabes may 
here be included. Symptoms on the part of the olfactory nerve are very rare, 
but unilateral and bilateral loss of smell and derangements of the sense of 
smell have been described. 

Taste is scarcely ever disturbed; the unfortunate patient with bulbar paral- 
ysis, previously referred to, retained his " wine tongue " to the last. Disturb- 
ances in taste and perversions of taste are only now and then reported 
(Klippel). 

More significant and common are disorders of hearing; difficulty in hear- 
ing and deafness not due to disease of the conduction apparatus have repeat- 
edly been described, and have been attributed to atrophy of the nerve of hear- 



490 TABES DORSALIS 

ing, also permanent tinnitus aurium with or without vertigo and difficulty in 
hearing; this sometimes occurs in paroxysms; now and then Meniere's symp- 
tom-complex has been observed in association with tabes. 

Finally, as a common symptom probably referable to the pneumogastric, 
tachycardia may be mentioned; this is manifested by a more or less rapid 
increase in the pulse rate from 90 to 120 per minute ; it is permanent, causes 
no distress, and is not explained by any objective finding in the heart. 

A few nervous symptoms of cerebral origin are now to be mentioned: 
Migraine is here included, provided it occurs for the first time during the 
course of tabes; but this is most uncommon, and its intimate relation with 
tabes doubtful ; a simultaneous arteriosclerosis is much more likely. 

The same is also true of occasional epileptiform and apoplectiform attacks ; 
they probably do not belong to the pathologic picture of tabes, but are more 
likely co-effects of the same cause (syphilis), or are to be regarded as inci- 
dental complications essentially due to arteriosclerosis. The patient mentioned 
frequently suffered from tinnitus aurium combined with attacks of vertigo, 
which occasionally caused a prolonged loss of consciousness and somnolence; 
here an intimate connection seemed likely. 

Polyuria and glycosuria, now and then observed, are of no great signifi- 
cance. They are both probably due to neurogenous alimentary glycosuria; 
but an accidental complication with true diabetes mellitus is however possible. 

We now come to exceedingly important and remarkable trophic disturb- 
ances. The most common are the changes in the joints and bones: Arthro- 
pathia tabidorum and spontaneous fractures of the bones due to their abnormal 
friability. We chiefly owe our accurate knowledge of these to the researches 
of Charcot and his school based upon the rich material of the Salpetriere ; but 
in Germany, as well as other countries, much was done to pave the way to an 
accurate recognition of these conditions. 

Tabetic arthropathy is an extremely peculiar joint affection which is char- 
acterized clinically by the most marked objective arthritic changes — in the 
acute stages by enormous swelling of rapid development, in the chronic stage 
by extreme deformities depending upon atrophy of all the parts of the joint, 
sub-luxations, and luxations — as well as by the almost total absence of pain. 

In fact it makes a remarkable impression upon us when we discover in a 
tabetic that his knee-joint — which during the night has become a tense, fluc- 
tuating, unsightly tumor of the size of a man's head, and from which marked 
edema extends far up the thigh and down the leg — is painless to touch and 
motion, without redness or fever; or when, later, the joint becomes loose, and 
sub-luxation and colossal deformities appear such as are scarcely ever seen 
in any other arthritic affections. 

The arthropathy usually occurs in the early stages of tabes, usually long 
before the ataxia, the onset being often acute, without prodromes, without 
pain, and without fever. There is a marked swelling of the whole joint 
and its surroundings, due to a profuse serous, sero-fibrinous, or hemorrhagic 
effusion, as well as to a doughy, tense, cutaneous, or subcutaneous edema. The 
chronic form begins more insidiously, there is less effusion, and either slowly 
or rapidly the subsequent coarse changes develop. In both forms of the disease 
the later course is the same : Deformities appear in the joints, there are creak- 
ing and crackling sounds, the atrophy of the joint surfaces and cartilage 



SYMPTOMATOLOGY 491 

produces luxations and sub-luxations and various kinds of proliferation, osteo- 
phyte formation, and connective tissue thickening, result in unsightly altera- 
tions in form, and more or less impair the usefulness of the joint. 

This is most conspicuous in the knee-joints: Enormous hyperextension, 
luxation of different degrees, and unsightly swelling produce a typical picture. 
Similar abnormalities appear in the joints of the shoulder, the elbow, and the 
hands. These changes involve many joints of the tarsus and the other bones 
of the foot, and not rarely cause a loss of the natural curve of the foot, a 
shortening of its middle portion — the different varieties and forms of which 
have been described by Charcot and Fere as the " tabes foot." 

The combination here presented of serous exudation, atrophy, erosion of 
the cartilage and epiphyses, destruction or proliferation of the ligaments, tear- 
ing of the tendons and ligaments, osteophyte formation, loosening of portions 
of the bone, luxation and loosening of the joints, and atrophy of the para- 
articular muscles will be minutely described under the pathologic anatomy. 
These changes make up a picture of the arthritic affection such as is never 
observed in ordinary arthritis, neither in arthritis deformans, nor in ordinary 
chronic articular rheumatism, nor in gout. From this clinical picture, there- 
fore, arise the peculiarities which led the first observers to regard tabetic 
arthropathy as a special type, as a tropho-neurotic arthritis. And this view 
is confirmed by the fact that the same arthropathies are found in only one other 
spinal disease, syringomyelia (and here chiefly in the upper extremities). 

This tabetic arthropathy is most common in the knee-joint (among a 
large number of cases'Ivredel found it here 104 times, in the hip- joint 56 times, 
in the shoulder- joint 36 times, in the ankle-joint 25 times, in the elbow-joint 
15 times, in the toe- and finger-joints 10 and 8 times respectively). The 
condition is not rarely bilateral in the same joints; occasional^, however, it 
implicates various joints of the upper and lower extremities side by side, and 
this indicates a widely distributed disturbance of the nutrition of the joints 
and bones. 

This is also distinctly evident from the fact that these arthropathies are 
frequently combined with an analogous disturbance in the diaphysds of the 
long bones which leads to spontaneous fractures upon the slightest cause. This 
sometimes appears in the early stages, not rarely, however, in the ataxic stage. 
These fractures either occur spontaneously on any brisk movement, such as 
turning in bed or putting on the shoes, or they follow insignificant trauma. 

They run their course without pain and without reaction, are readily rec- 
ognized, and under appropriate treatment unite rapidly and completely, al- 
though there is sometimes a marked callus formation, but they may soon 
reappear at some other point. Not rarely we find multiple fractures in the 
same patient. 

They are more common in the thigh, and especially at the neck, than in 
the leg, forearm, or upper arm, and are found in decreasing frequency in the 
clavicle, pelvis, etc. ; they are apparently due to an abnormal friability of the 
bone, probably to a rarefying osseous degeneration, with an increasing deposit 
of medullary substance. Their analogon is the spontaneous tearing of tendons, 
such as has been observed in the Achilles and the patella tendons ; this also is 
unaccompanied by pain. 

In addition we nute in some cases atrophy of the alveolar processes of the 



492 TABES DORSALIS 

jaws with consequent falling out of the teeth. Within a few weeks or months 
all the teeth of one jaw, or of both jaws, may fall out without causing pain; 
or the entire alveolar process, having become necrotic, sloughs away, and in 
comparatively young people the jaws appear as atrophic as those of the aged. 

An illustration of the not uncommon trophic disturbance of the skin is 
the condition known as mat perforant (perforating ulcer of the foot). This 
appears in the early stages, usually upon the sole of the foot in the region 
of the metatarso-phalangeal joint underneath the great toe, but it may appear 
in other areas of the sole, on the heel, or on the dorsum of the foot. It is 
a small, round, cutaneous ulcer which penetrates more and more deeply, even 
into the bone; it is chronic, torpid, only slightly if at all painful, and heals 
with the greatest difficulty. It is not remarkable that many persons regard it 
as a manifestation of syphilis. I have known it to heal most readily under 
specific treatment — the use of mercurial plaster. 

Among other cutaneous disturbances the first to be mentioned is herpes 
zoster which — otherwise rare — is very common in tabetics in the usual (trunk) 
and in unusual localities; we can scarcely doubt that it is attributable to the 
recently discovered anatomical changes in the nerve roots and spinal ganglia; 
this is confirmed by the lymphocytosis which has also been demonstrated 
in herpes zoster. Atrophy of the skin, falling out of the nails and of the hair, 
pigmentary changes, cutaneous hemorrhages, anomalous sweat secretion, ery- 
thema, chronic edema, etc., have all been considered in connection with tabes, 
whether correctly or not cannot be stated. 

Valvular lesions of the heart have also been referred to tabes; particularly 
aortic insufficiency which has been by far most frequently noted, and to which 
great significance has been attached; but it is certainly not frequent. In 400 
cases I found it only 7 times ; others have observed it in a greater proportion. 
After discussion it seems likely that this is either a purely accidental compli- 
cation (due to articular rheumatism) or it is due to a syphilitic aortitis which 
causes disease of the aortic valves. 

In conclusion, we will cast a glance at the numerous psychical disturbances 
which appear in tabes. 

In our former conception of tabes as an affection of the spinal cord, we 
expected to find no psychical disturbances in the symptom-complex, but, having 
seen that tabes produces symptoms in nearly all parts of the nervous system 
as well as in the entire body, it does not appear remarkable that this affection 
should influence the cerebrum, particularly its psychical functions; hence, 
from the earliest periods psychical disturbances have been mentioned in the 
pathology of tabes, and recently its undoubtedly close relation to severe psy- 
chical disease and to progressive paralysis has been especially discussed. 

Leaving this last affection out of consideration for the time being, there 
is no doubt that in many cases of typical tabes psychical disturbances are 
lacking: Intelligence, memory, and the general mental capacity of tabetics 
remain entirely intact; so far as mental work is concerned the patients may 
follow their occupations for decades without hindrance. In temperament they 
are often extremely jolly in contrast with the severe disease from which they 
suffer; at other times they are depressed, irritable, moody, or distressed by 
their symptoms, are sleepless, etc. On the other hand, accurate observation 
has shown that quite a series of psychical disturbances sometimes appear in 



SYMPTOMATOLOGY 493 

tabes, which, however, must be regarded as incidental s3 T mptoms or complica- 
tions: Severe neurasthenic disturbances or slight dementia, hysterical depres- 
sion, in rare cases actual paranoia, and the like appear ; later there are psychi- 
cal disturbances combined with alcoholism or morphinism, and, finally, what 
is not altogether rare, those due to syphilitic diseases of the brain. These 
conditions are, as a rule, easily recognized, but it is sometimes difficult to 
estimate them at their true value; in practical importance they are far behind 
progressive paralysis. 

After Westphal had demonstrated how common is degeneration of the pos- 
terior columns in progressive paralysis, after the symptom-picture of paresis 
(reflex rigidity of the pupil, absence of the patella tendon reflex, Eomberg's 
sign, lancinating pain, certain disturbances in gait, etc.) had been many times 
described among the important symptoms of tabes, and especially since we 
learned that progressive paralysis, like tabes, is undoubtedly a syphilogenous 
affection, among other causes this being in both the important one which de- 
velops the disease, and since the one disease is exceedingly apt to follow the 
other, the conviction was more and more strongly forced upon us that tabes 
and progressive paralysis are related to each other, that they represent analo- 
gous forms of disease produced by the same conditions, that they have the 
same anatomical foundation, and differ only in their localization. Some au- 
thors (Mobius, Eaymond, Xageotte, Mott, and others) have not hesitated to 
declare that tabes and progressive paralysis are really one disease, a meta- 
syphilis, the former chiefly or exclusively attacks the spinal cord, the latter 
implicating the brain. The combination of these affections might then be 
designated " tab o paralysis" But this is going too far: The identity of the 
actual pathological process in these two diseases has not yet been proven; the 
combination of the affections is not so common that they must be identical; 
but they are closely related, and their consideration from the same points of 
view is undoubtedly justified. 

Clinically the condition presents itself by the addition of progressive paral- 
ysis to a tabes which has for a long time existed, or tabetic symptoms are 
demonstrated in the pathologic picture of a progressive paralysis already pres- 
ent. The first case appears to be the rarer ; the addition of progressive paralysis 
to a long existing tabes is by no means frequent ; it is interesting to know that 
among the numerous tabetic inmates of Bicetre Dejerine observed this condi- 
tion only twice in 8 years; in the tabetic the psychical disturbances of pro- 
gressive paralysis are subsequently observed, chiefly as those of simple dementia, 
with paralytic attacks, disturbances of speech, etc., and the patients succumb 
in the usual way. 

The other condition — progressive paralysis to which tabes is added — is 
probably more common; among a number of patients with progressive paral- 
ysis Xageotte observed that two-thirds had symptoms of tabes; this is prob- 
ably over-estimated. Besides the typical symptoms of progressive paralysis, 
we find in these patients the sjonptoms of tabes; besides pupillary differences 
there is reflex rigidity, instead of being increased, as is the rule, the tendon 
reflexes are absent, there are lancinating pains, paresthesias, the typical sensory 
disturbances belonging to tabes (Eomberg), marked bladder symptoms, and 
probably also ataxia ; but these symptoms must be searched for, and are usually 
secondary to the symptoms of progressive paralysis. 



494 TABES DORSALIS 

Of course, this " taboparalysis " is seen in many varieties and subdivisions, 
the symptom pictures being of unequal formation. It is unnecessary to de- 
scribe this more minutely, but in practice the combination in itself is exceed- 
ingly important, serious, and of theoretic interest. 

From this comprehensive description of the many-sided symptom-picture 
of tabes in its early stages, we may proceed more rapidly to the final act in 
this pitiful drama. 

The duration of the first stage is prolonged by many variations, many 
arrests of the affection, and is exceedingly long, yet for many a one desirous 
of living not long enough; it may be 5, 10, 15, 20, or more years. But the 
suffering, the helplessness which constantly becomes greater, the pains and 
the recurring crises, the amaurosis, the incontinence of the bladder, the cys- 
titis, the decreased nutrition, the obstinate constipation, and many other evils 
exhaust the strength of the patient. The terminal stage of the disease, " para- 
lytic " or " paraplegic " as it has been called, appears. The motor power 
diminishes more and more, the greatly emaciated legs become paralyzed and 
stiff, contractures and deformities appear, the digestion suffers, appetite is lost, 
constipation becomes more obstinate, and incontinentia alvi becomes more 
serious; to the increasing paralysis of the bladder, cystitis and its sequelae, 
pyelitis and pyonephritis, are added. Bed-sores appear, and increasing ca- 
chexia, marasmus, and general exhaustion finally terminate the sufferings of 
the patient. 

Fortunate are those in whom an intercurrent affection (enteric fever, pneu- 
monia, influenza, pulmonary phthisis, dysentery, or the like) early brings about 
the final act. 

In some cases the disease terminates with a peculiar nervous prostration, 
a deep somnolence, with increased pulse, Cheyne- Stokes respiration, and a rise 
or fall in temperature which may last from one to three days — apparently a 
general paralysis of the cerebral functions. The duration of the disease — 
counting its three stages — may be calculated at from 20 to 30 years. Of 
course some cases terminate much earlier, even in 5 or 10 years, especially 
when there are grave complications (cerebral syphilis, progressive paralysis, 
severe pyonephritis, etc.). 

Before minutely analyzing the principal symptoms and their pathogenesis, 
it is most necessary to study the pathologico-anatomical changes in tabes. 

IV. PATHOLOGIC ANATOMY 

It is almost impossible, from our present knowledge, to give a satisfactory, 
conclusive, and didactic description of the pathologic anatomy of tabes. 

More than 40 years of painstaking study and innumerable investigations 
carried out with most exact methods have gradually taught us the localization 
of the process and how to explain it, but not its actual starting-point or its 
nature. In regard to these two points there has been much discussion in the 
last few years, old and long-forgotten views have been revived, and supported 
by new and important findings, the seemingly positive explanations generally 
accepted are combated by others, one theory annulling another, all being more 
or less opposed to each other, and for the objective investigator no single one 
to be regarded as certain. After such long and patient study this is really 



PATHOLOGIC ANATOMY 495 

an admission of which to be ashamed ! The subject is, however, extremely 
intricate, many-sided, and complex, the investigation of each individual case 
requires so much time and research, so much insight and exactness, that we 
readily comprehend why we cannot arrive at a satisfactory explanation, espe- 
cially as each individual case presents its own peculiarities and variations. 

We shall attempt briefly to describe the essential and positive facts, and 
will refer the reader to innumerable treatises and compilations which have 
been published in the last few years by v. Ley den, Dejerine and Thomas, and 
Collins, and especially to the excellent description given by Schmaus in his 
" Lectures upon the Pathological Anatomy of the Spinal Cord." Of course 
it is impossible for me to discuss fully the literature of this difficult subject. 

Upon microscopic examination of the spinal cord in recent and mild cases, 
we observe externally little or nothing; in all more advanced cases, however, 
it is evident that the spinal cord as a whole, especially its lower sections, is 
somewhat smaller than normal, and that this change affects mainly the poste- 
rior half, especially the posterior columns. These are gray, narrower, some- 
what sunken, and of coarse consistence; they are most noticeable in the lum- 
bar cord, and decrease as they extend upward toward the cervical cord. 

Decided changes take place in the posterior roots, these usually being most 
conspicuous in the cauda equina, but extend upward to the thoracic or 
cervical cord; the roots become atrophied, are of a grayish luster, are often 
reduced in size to the diameter of a thread, and differ markedly from the well- 
retained, round, whitish, and glistening anterior roots. 

As a rule, the meninges show distinct but, as a rule, insignificant changes ; 
in some cases they may be marked. There is usually slight turbidity and 
thickening of the soft membranes, with firm adhesion to the spinal cord to 
the roots and to the connective tissue threads, etc. ; a mild leptomeningitis 
posterior, generally confined to the posterior surface of the spinal cord and its 
lumbo-dorsal portion. 

In fresh, unstained, cross sections the gray discoloration of the white pos- 
terior columns becomes most obvious, and their transparent, coarse consistence. 
The posterior columns appear to be depressed beneath the posterior surface 
and decidedly shrunken, hence they approximate one another; they also, as 
a rule, appear to be decreased in size and atrophic. The lateral and anterior 
columns, and the gray anterior columns, present a normal appearance. 

This "gray degeneration (sclerosis) of the posterior columns" is most 
conspicuous and complete in the lumbar cord, it decreases in the upper thoracic 
cord, and is less marked in the cervical cord; in these regions it usually attacks 
only GolPs columns, and Burdach's tract. 

This change is rarely uniform — and in individual cases it varies extraordi- 
narily according to the duration of the disease and the varying height in the 
spinal cord. In tabes superior the process is chiefly localized in the cervical 
and upper thoracic cord, being merely indicated in the lumbo-dorsal region. 
The intensity and distribution of the posterior column degeneration is also 
irregular. 

In the later stages, here and there in the posterior lateral columns, pyram- 
idal columns, and cerebellar lateral columns, an indistinctly limited gray 
discoloration is noted. 

Accurate knowledge of these topographical relations is only to be obtained 



496 TABES DORSALIS 

by hardening and examination of the stained cross sections (after Weigert, 
etc.), taken from various heights in the spinal cord; and should be done with 
low as well as high magnification. 

We need not minutely discuss the exact liistological details of the process. 
It aids but little in explaining the pathological condition as a whole. Every- 
where in the degenerated areas we find the same simple processes : More or 
less advanced decay of the nerve fibers with a decided increase of the glia tissue. 
The nerve fibers degenerate, and more or less uniformly lose their medullary 
sheath ; some of them become swollen and thickened, they subsequently atrophy 
with the axis cylinders, and finally perish. The proliferated glia appears as 
a fine fibrillary tissue, in which the spaces left by the nerve fibers which have 
disappeared are frequently visible; later it changes into a fine, wavy, striated, 
and tough connective tissue, which shows many nuclei and neuroglia cells, 
and often numerous corpora amylacea. The vessels are usually but slightly 
changed ; they show thickening, hyaline formation, a narrowed lumen, and here 
and there profuse nuclear deposits. In the early cases which show a rapid 
progress granular cells have been seen. 

In the posterior roots of the nerve fibers we find exactly the same degen- 
erative, simple, atrophic, processes, varying in intensity in different areas of 
the same fiber; a conspicuous increase of the connective tissue structure and 
thickening of the vessels is also noted, and here and there profuse small-cell 
infiltration. 

These findings permit us to conclude with some degree of certainty that 
primarily the nerve elements are destroyed, and secondarily there is prolifer- 
ation of the glia — therefore a primary degeneration and a secondary sclerosis 
take place. The process, as a whole, cannot be differentiated from secondary 
degeneration of the nerve tracts due to other causes. This is recognized by 
most recent authors, and Schmaus expressly maintains that the areas of possi- 
ble primary degeneration cannot be distinguished from the areas of secondary 
ascending degeneration. 

It is certainly remarkable that the degeneration of the extramedullary pos- 
terior roots is often much less advanced and less intense than that of the intra- 
medullary continuations. 

But these histologic details are less important than the localization of the 
tabetic process in the posterior columns. 

In typical and slowly progressing cases, the process begins in the lumbar 
cord by two symmetrical fields of degeneration in Burdach's columns, which, 
at about their centers or a little more anteriorly, lie alongside of the posterior 
columns, and correspond to the so-called root entrance zones ("bandelettes 
externes " of Pierret) ; in the higher segments these fields advance toward 
the center, and finally enter Goll's columns, while new fields of degeneration 
appear beside them in Burdach's column; thus, a large portion of the trans- 
verse section of the posterior column in the upper lumbar and the lower dorsal 
cord may be implicated, while in the middle and upper thoracic cord the 
degeneration is limited to Goll's columns (the middle portion). 

If the process advances higher, new fields of degeneration in Burdach's 
column constantly appear in the various segments of the thoracic cord, and 
those fibers originating from the lumbar and sacral cord, and degenerating 
upward, more completely fill the posterior portion of Goll's columns — until 



PATHOLOGIC ANATOMY 497 

at last almost the entire transverse section of the posterior columns in the 
thoracic cord has degenerated. 

This process is then repeated in the cervical cord — the same advanced 
degeneration of Goll's columns that is seen in any conspicuous secondary 
ascending degeneration — and new fields of degeneration corresponding to the 
entrance zone of the roots in Burdach's columns are constantly added, until, 
finally, the morbid process gradually terminates in the upper cervical cord 
at the height of the calamus scriptorius. 

This is the typical course; it may, however, show variations as the indi- 
vidual root bundles are not always uniformly diseased from below upward ; for 
instance, when the process skips individual segments, or when it begins in the 
cervical cord ; then there is at first no degeneration of Goll's column, but the 
change is limited to the cervical root fibers and the higher areas, etc. 

Closer investigation teaches us that in the earlier stages this degeneration 
is chiefly localized at the entrance of the posterior root fibers. In the first 
place it directly affects the ascending coarse fibers which pass through Bur- 
dach's column into Goll's columns, then the finer root fibers situated in Lis- 
sauer's marginal zone (peripherally from the tip of the posterior columns) ; 
in tabes this zone almost invariably degenerates; it also affects the coarse 
fibers which pass into the posterior columns in an arch, on one side into 
Clarke's columns (a constant decrease of the fibers which enter these columns 
while the cells are retained), on the other side anteriorly through the posterior 
columns to terminate in the vicinity of the large ganglion cells of the anterior 
columns, the so-called reflex collaterals. There is a decrease of spongy and 
gelatinous substance in the fibers, and in the posterior columns in general, 
which permits the recognition of a corresponding glia proliferation. 

Hence, certainly in the early stages and for a long time, there is no degen- 
eration in the so-called ventral posterior column field (at the tip of the poste- 
rior columns), nor in the semilunar posterior external field (throughout the 
entire spinal cord) and, in the lumbar cord, only in the so-called dorso-medial 
bundle (a small oval field at either side of the posterior longitudinal space). 

It is assumed that these fields are chiefly composed of endogenous fibers, 
therefore they do not come from the posterior root fibers or their collaterals. 
But, in the later course of older cases of tabes, these also may completely 
degenerate, so that, finally, especially in the lumbar and thoracic cords, the 
posterior columns appear to have completely and uniformly degenerated. 

Flechsig attempted to prove that the tabetic fields of degeneration corre- 
spond with the embryonal 'fields of the posterior column which he discovered ; 
as a matter of fact there is a close analogy, but some authors doubt this, or 
consider it to be a mere coincidence. 

In many cases it has been found that the shorter intramedullary root fibers, 
probably also their collaterals, are most completely degenerated, and from 
this, as well as from the longer exemption of fields which are still included 
within the posterior root tracts, it has been concluded that the condition is 
one of selective disease of the root fibers, some being earlier, some later attacked, 
but this is still a matter of doubt. 

Observations and the preceding description have, however, shown with cer- 
tainty that tabetic disease of the posterior columns is nothing more than a 
segmental disease of the posterior root areas ascending from below upward. 
33 



498 TABES DORSALIS 

The lesion of the tabetic posterior column is, therefore, unquestionably radicu- 
lar, and is exactly the same as in a secondary degeneration after compression 
of the root (for instance, by a tumor of the cauda equina, and the like) ; the 
atrophy of Burdacrr's columns and GolPs columns is generally in proportion 
to the position and number of the degenerated posterior roots. 

This picture reveals the nature and the development of the process which 
must finally lead to columnar degeneration of nearly the entire posterior 
column. Segments of the individual posterior root bundles are attacked one 
by one in an upward direction; each field passes through a characteristic 
degeneration, first in Burdacrr's, and then in Goll's columns; these fields are 
situated one above the other in Burdach's columns, and they elongate and 
distribute their processes in Goll's columns, until from below both pass far 
upward, and appear as columnar degeneration in the vicinity of the medulla 
oblongata. In this sense, therefore, tabetic disease, in so far as it affects the 
posterior columns, is a systematic lesion; it affects the various fiber systems 
which are situated in the posterior roots. 

The process usually begins symmetrically in individual root bundles, con- 
stantly implicating more and more of these, and ascending and progressing 
with varying rapidity; it may begin in the lumbar region, almost invariably 
in the upper lumbar, sometimes also in the lumbodorsal, more rarely in the 
sacral cord or the conus medullaris, and quite as rarely in the cervical cord, 
in which case it gives rise to tabes cervicalis or superior, to which we have 
frequently referred. 

Occasionally the process appears to be confined to a few roots (then clin- 
ically differentiated as tabes incompleta) or even to an individual root bundle, 
in which case it has been called mono-radicular tabes. Such cases, of course, 
only accidentally become subject to histologic investigation. 

Therefore, we are dealing primarily with a strictly limited disease of the 
peripheral sensory neuron (ganglion cells in the spinal ganglion), and chiefly 
of its spinal axon. 

Before discussing this view, we must mention other anatomical changes 
which have been found in the spinal cord itself and the other parts of the 
nervous system. 

In the earlier stages we invariably find in the spinal cord the decided 
degeneration of the gray posterior columns which has already been described. 
Not infrequently, but always later, the gray degeneration implicates the lat- 
eral columns, there is usually marginal degeneration of the cerebellar lateral 
column tracts and Gowers' bundles, which is not strictly- confined to these 
boundaries but extends even beyond them to the pyramidal columns. Occa- 
sionally, especially in long-standing cases, marked changes may be widely dis- 
tributed and included within the combined system diseases (observations of 
Kattwinkel and others). 

Changes are rarely found in the gray anterior columns. Only in isolated 
cases of amyotrophia has degeneration of the cornual cells, etc., been noted. 

In the meninges there is cloudiness and thickening with connective tissue 
adhesions; this is most marked between the posterior roots, rarely encircling 
the entire spinal cord. In quite a number of cases published in Germany 
(Hoffmann, Dinkier, etc.), there was conspicuous syphilitic leptomeningitis. 
Later, however, French authors (Nageotte, Dejerine, etc.) attached special 



PATHOLOGIC ANATOMY 499 

significance to the almost invariable occurrence of mild meningitis with pro- 
liferation of small embryonal cells in the membranes and the vessels, being 
more marked in the veins than in the arteries ; Xageotte maintains that these 
are syphilitic. They are identical with the meningitis accompanying myelitis 
syphilitica, but are usually confined within moderate limits. 

The constant degeneration of the posterior roots, as well as their histo- 
logic condition, has already been described; in the anterior roots there are 
usually no degenerative changes or these are but slight, and are found in cases 
complicated by amyotrophia, and in degeneration of the gray anterior columns. 

On account of certain views which have been discussed, the spinal ganglia 
have recently been thoroughly studied, but they show no invariable changes; 
what little has been found in the cells (vacuoles, contraction, atrophy, pig- 
mentation, tigrolysis, etc.) is unimportant, and does not permit the assump- 
tion of constant or great anatomical change, but neither does it exclude a 
functional disturbance. In 1901 Thomas and Hauser again announced the 
great significance of these changes in the pathogenesis of tabes ; they invariably 
found them to be more or less intense. 

The fact — noted by most investigators — is very remarkable that the affer- 
ent posterior root fibers (following the physiologic direction of conduction) 
are markedty degenerated above the ganglion, while the afferent peripheral 
nerve fibers are intact or but slightly changed. 

It has been known for some time (v. Leyden, 1863) that the peripheral 
nerves sustain degenerative neuritic lesions, etc.; these are most severe in the 
sensory nerves, especially in the small distal cutaneous branches, being less 
severe in the nerve trunks and certain nerves, and least so in the proximal 
parts (Westphal, Pierret, Dejerine, Oppenheim and Siemerling, Xonne, and 
others), but this is by no means constant. It is certainly remarkable that the 
peripheral sensory nerves near the ganglion, as well as the ganglion itself, 
should show such insignificant changes, while the posterior roots belonging 
to them have degenerated to an extreme degree. 

The peripheral motor nerves rarely show degenerative change; here we 
must except the nerves of the ocular muscles which are frequently altered, 
and show neuritic change. Sometimes other cranial nerves are affected: The 
hypoglossal, the pneumogastric and spinal accessory, the recurrent laryn- 
geal, etc. 

Especially important and common is atrophy of the optic nerves, which is 
evidently a primary parenchymatous degeneration, usually combined, it is 
assumed, with changes in the nerve-cells of the retina. Degenerative changes 
have occasionally been noted also in the olfactory and auditory nerves. 

Here must be mentioned degenerations in the nuclei of the cranial nerves 
in the trunk of the brain, the nuclei of the muscles of the eye, the bulbar 
nuclei, etc., with the clinical disturbances belonging thereto. 

In the sympathetic nothing positive has been found; a degeneration of the 
fine medullary fibers has sometimes been noted, the coarser ones remaining 
intact. 

In the orain, in the cerebrum as well as in the cerebellum, cortical fiber 
atrophy has been occasionally demonstrated, and unless the frequent combina- 
tion with paresis is excepted, this appears to be incidental and to have but 
little in common with tabes. What importance is to be attached to the 



500 TABES DORSALIS 

changes in the molecular layer of the cerebellum, which appear to be constant 
and to which C. Weigert called attention (Neurolog. Centralbl, 1904, p. 738), 
remains to be seen; it may be considerable. 

Therefore, the essential feature of tabes is undoubtedly a segmentary dis- 
ease of the posterior root tracts, both intramedullary and extramedullary. 
There is almost complete unanimity of opinion concerning this. But the 
questions arise, What is the nature of the process? What produces it? Is it 
a primary or a secondary degeneration? If the latter, what is the actual 
starting-point of the process ? 

All these questions have been much considered of late, much investigated 
and discussed, but the answers have differed greatly; important facts have 
come to light, new views have arisen — but unanimity has not yet been attained. 
The questions are still mooted, and at present no conclusive opinion can be 
reached. Hence only a brief report concerning the various views is possible ; 
a critical and comprehensive review of all the facts, views, theories, etc., that 
have been advanced, would lead us too far from our subject. 

The old view that tabes is a chronic inflammation of vascular nature with 
a connective tissue sclerosis and secondary degeneration of the nervous ele- 
ments, and that this inflammation essentially depends upon the vascular divi- 
sion of the posterior columns and their localization, was long ago disproven 
and generally discarded. But, in a modified form, a medullary theory depend- 
ing especially upon the lymphatic tracts of the posterior columns has lately 
been promulgated by P. Marie and Gruillain. 

The view has steadily become more prominent, and in the last few years has 
become the dominant one, that the condition is one of primary parenchymatous 
degeneration of the actual nerve tracts (the posterior roots), and that this 
is due to unknown deleterious influences, and, with increasing certainty based 
upon clinical experiences, this baneful factor is assumed to be the action of 
certain poisons generated by syphilis. The analogous action upon the spinal 
cord of other poisons, of ergotin, pellagra, lathyrus poison, lead, and alcohol, 
also of certain autointoxications as in severe pernicious anemia, etc., which 
preferably affect the posterior columns, and produce lesions very similar to 
tabes, appears to support this view. But this theory is disputed, and, at pres- 
sent, is merely a hypothesis. 

Anatomical findings have gradually forced us to recognize in the tabetic 
change a secondary degeneration of the posterior root tracts; although these 
do not exactly correspond with the common secondary degeneration caused 
by coarse root lesions (in fractures of the vertebrae or tumors of the cauda 
equina) it does not appear remarkable that in such an exceedingly chronic 
process in which the individual root bundles either simultaneously or success- 
ively become diseased (and the origin of this secondary degeneration was 
eagerly sought for) a dynamic or mechanical action, which must be found, 
had at some point interrupted the nutrition and conduction of the posterior 
root tracts in such a way as to lead to secondary degeneration. 

Under the domination of the neuron theory, and after the trophic centers 
of the posterior root tracts were recognized in the ganglion cells of the spinal 
ganglion, what was more natural than to think of these spinal ganglia, and 
to search in them for the actual starting-point of the tabetic posterior column 
lesion? This highly plausible theory, unfortunately, could not be proven, 



PATHOLOGIC ANATOMY 501 

although many special investigations were undertaken for the purpose; the 
histologic changes in the cells of the spinal ganglion are not sufficiently dis- 
tinct nor constant enough to be regarded as the anatomical starting-point of 
severe changes in the roots which go on for years. The forcible objections 
have been raised that, besides the almost intact ganglion, the centripetal root 
fibers close to the ganglion are markedly degenerated, the centrifugal periph- 
eral nerve fibers, however, being but little altered, and that even the degen- 
eration of tabes is often entirely absent in the peripheral nerves. 

The same facts controvert the otherwise plausible hypothesis that the con- 
dition is essentially one of functional disturbance of the spinal ganglion cells, 
and this produces the trophic changes in the axons, particularly the spinal 
ones. The theory was first suggested by me 20 years ago (and has since 
then been generally accepted) that functional disturbances in the trophic cen- 
ters (neuron cells) which are microscopically unrecognizable may produce 
visible and more or less decided trophic disturbances (degeneration and 
atrophy) in the motor nerves and the muscles innervated by them. It seems 
plausible that a definite toxic action which does not histologically damage the 
cell may, nevertheless, disturb its function to such a degree that the axon, 
first at its distal ends, and subsequently even the cell itself, may degenerate. 
It is unnecessary to discuss this possibility; whether it is true in this case 
cannot be stated; hence the conception of a functional or anatomical change 
of the spinal ganglion as the origin of tabes — of course only in so far as it 
is manifested in the posterior columns and posterior roots — is merely a hypoth- 
esis which must be proven. 

The same is true of the view proposed some time ago by Leyden, and 
lately also by Goldscheider, that tabes begins in the pcriplieral sensory nerves 
(chiefly in the cutaneous nerves), that here the first degenerative changes 
appear, and that these extend centripetally through the spinal ganglion to the 
posterior roots and the spinal cord. The anatomical facts (inconstant affec- 
tion of the peripheral nerves, slighter degeneration of these than of the poste- 
rior roots, and exemption of the ganglion itself), the extent, the localization, 
and the development of the tabetic process, the early implication of the reflex 
collaterals, etc., are opposed to this view, while for the etiologic factors (cold, 
trauma, etc.) which certainly do not play a prominent role, as well as for 
the appearance of affections of the spinal cord after amputations, all grounds 
of support are removed. Therefore, this theory of the development of tabes, 
which v. Leyden maintains with some modifications, is rejected by almost all 
recent authors. 

Finally, the starting-point of the tabetic lesion was sought in the meninges, 
in a chronic meningitis. This obsolete, and apparently long forgotten, view 
has recently been restored to a place of honor, naturally in a new form. The 
fact is certain that severe meningitis — that form leading to coarse indurative 
thickening, or even syphilitic meningitis — as a rule produces no tabetic root 
lesion. The same may be occasionally said of syphilitic meningitis, but in 
this there may be coordinative consequences of syphilis. It is also certain 
that in tabes there are never any decided and invariable, although mild, menin- 
gitic changes. Therefore, stronger warrant for this assumption must be found. 

Obersteiner and Eedlich demonstrated that the entering posterior root 
bundles on the side of the somewhat thickened pia mater suffer a slight con- 



502 TABES DORSALIS 

striction, consequently they show intramedullary degeneration. The point at 
which the roots pass through the pia offers less resistance, therefore facilitates 
this process. There is much in opposition to this view; the most important 
argument, in my opinion, is that this hypothesis does not explain the invariable 
degeneration of the posterior roots, and the changes in the pia are too slight 
to explain the severe compression, etc. But even if the condition seen in most 
cases is not an artifact (due to hardening), and subsequent examinations have 
shown it to be by no means constant, this hypothesis is nowhere accepted, and 
we may say, has been withdrawn even by its originators. 

On the other hand, ^"ageotte 1 believes that he lias found another invariable 
starting-point for the tabetic root degeneration — this upon the basis of accu- 
rate and, apparently, very important investigations which we must consider 
somewhat more minutely. 

In his opinion tabes is the result of a local affection of the spinal roots at 
the height of the " nerfs radiculaires " (by this Nageotte means that portion 
of the roots from their common entrance into the dura mater up to the spinal 
ganglion; see diagram) ; this disease sometimes extends as far as the spinal 
ganglion. It consists in an interstitial transverse root neuritis (perineuritis 
and endoneuritis with interstitial and parenclrymatous changes) which is due 
to a mild but chronic syphilitic meningitis, its development and course being 
determined by the arrangement of the lymph tracts. This root neuritis is also 
syphilitic. 

According to Nageotte, this diffuse syphilitic meningitis which may be 
recognized by a slight thickening of the pia, with diffuse or focal cellular infil- 
tration (especially profuse in recent cases!), with specific vascular changes in 
the arteries, capillaries, and especially in the veins, and which upon lumbar 
puncture invariably shows lymphocytosis, always precedes tabes, but tabes is 
developed only by its combination with root neuritis. It may be demonstrated 
by cytodiagnosis from the earliest symptoms of tabes (isolated reflex pupil- 
lary rigidity), and even prior to its appearance. 

Neuritis of the posterior roots consists in a more or less severe interstitial 
change which finally leads to sclerosis ; it always permits us to recognize recent 
cellular infiltration (the persistent activity of the process corresponding to the 
progressive character of tabes) ; it also consists in degeneration of the root 
fibers without a tendency to regeneration; finally, the fibers are completely 
destroyed, some sooner, some later, and then secondary ascending degenera- 
tion of the posterior roots and their spinal continuations results. The fact 
that the degeneration at the beginning often shows only an intramedullary 
character Nageotte explains by the assumption that the short root fibers (col- 
laterals?) degenerate first at their distal ends, the process then extending 
celluKpetally (apparently a retrogressive change). 

Strange to say, the motor roots are not exempt; but their injury is less 
severe, is usually higher than the focus in the posterior roots, and the degen- 
erated fibers show a distinct tendency to regeneration. (This may possibly 
account for the absence of motor symptoms.) 

The " nerf radiculaire " is a channel for the lymph circulation in the cen- 
tral nervous system, and lesions or irritation in the cerebrospinal fluid may 

i " Pathosrenie du Tabes dorsalis " 1903. 



PATHOLOGIC ANATOMY 



503 



perhaps at this point have a more concentrated effect. In syphilitic menin- 

root neuritis here establishes itself; this is not 



gitis a syphilitic interstitial 
necessarily present in all 
cases of syphilitic menin- 
gitis, but it is constant in 
tabes. Nageotte found it 
in 11 cases which he care- 
fully investigated. 

It may be present in 
many roots, sometimes in 
the lumbosacral, at other 
times in the dorso-cervical 
roots; or some of the roots 
may at various heights be 
separated by normal ones, 
sometimes limited to a few, 
to one, or to two pairs of 
roots — therefore in very ir- 
regular distribution. 

In the main the fore- 
going embodies the findings 



Anterior root - 



Neuritis radic 
anterioris 



'to 

and the 



Nageotte. 




^ Spinal cord 



- Arachnoid 



Posterior root 



Dura mater 



Nerf radiculaire 



Neuritis radic. 
posterioris 



Ganglion 



Fig. 173. — Diagram of the Course of the Spinal 
Roots and of the Nerf Radiculaire Nageotte. 



views of 
It is evident that all sorts 
of objections may be raised 
to this theory, but it seems 
satisfactorily to explain 

the anatomical as well as the clinical picture of tabes — certainly its spinal 
lesions. 

But we have as yet no proof that these findings are invariable; hence we 
cannot at present decide in favor of this pathogenesis. 

From the same base the assumption of a " syphilosis " of the meninges, 
therefore of a chronic syphilitic meningitis, P. Marie and Guillain 1 have 
recently arrived at a quite different opinion ; they invariably found in tabes a 
posterior meningitis, especially in the dorsal region, and assumed the exist- 
ence of a peculiar and highly important lymphatic system in the posterior 
roots, in the pia, and in the posterior columns, and that syphilitic inflamma- 
tion of this system is the cause of tabes. They point to the strict root local- 
ization of tabes in the posterior column, and believe that there is often a merely 
pseudo-radicular distribution dependent upon division of the lymph tracts; 
these authors regard tabes as nothing more than a syphilitic lymphangitis in 
the posterior lymphatic system of the spinal cord. 

I am unable to understand how these authors reconcile the conditions histo- 
logically, and how they can deduce therefrom the entire tabetic lesion; here 
also we must await developments. 

Thomas and Hauser have recently propounded another theory according 
to which tabes is a toxic neuritis (a "dystrophy," as they call it) of the 
peripheral sensory neuron, chiefly existing in the central fiber distribution of 



Revue neurologique," 1903. 



504 TABES DORSALIS 

the neuron. They rigidly hold to primary degeneration in consequence of 
toxic influences, but make all sorts of concessions concerning other related 
views. 

These are the most important of the recent theories as to the pathogenesis 
of tabes; others which have been promulgated we may ignore. At this time 
it does not appear to me proper to declare positively in favor of one or the 
other. In my opinion, Nageotte's views based upon carefully ascertained facts 
most deserve confidence. I have seen some of his preparations, and they are 
very convincing; whether or not his views are correct depends upon whether 
or not his findings are constant, and whether or not his root neuritis is the 
co-effect of the same (toxic?) cause as the degeneration of the posterior root 
tract; therefore whether or not the intermediary link of a root neuritis is 
required. 

In spite of numberless investigations the secondary disease of the posterior 
root tracts is still undetermined, and it is possible that in tabes we are deal- 
ing with a primary degeneration of the root tracts, which at the onset of their 
intramedullary course are due to toxic action in combination with certain aux- 
iliary causes (Schmaus). And with this we come at last to the question of the 
actual remote cause of tabes, and its relation to the anatomical findings. Here 
everything revolves about the question whether syphilis is this ultimate cause, 
and whether it can produce these anatomical changes. This will be briefly 
discussed. 

Anatomical facts (aside from clinical facts) which favor the syphilogenous 
origin of tabes have recently accumulated. The French (Nageotte, P. Marie, 
Guillain, Dejerine, and others) speak merely of a " syphilosis " of the me- 
ninges, a syphilitic root neuritis, etc., in tabes, and with good reason; for 
these authors the question has undoubtedly been decided; they also, in the 
old sense, find " specific " changes in the meninges and roots. 

That these typical changes, the gray degeneration of tabes, are in this 
sense " not specific " is certain. That they may depend upon syphilis I no 
longer doubt. Even the fact that, in a large number of cases of tabes, besides 
the " typical " tabetic lesion we recognize quite a series of " specific " changes 
in the meninges and vessels, in the form of small cell infiltration, gummata, 
etc., renders it very likely ; I have recently compiled these cases in an article 1 
in which I attempted to prove the syphilogenous nature of these simple and 
apparently non-specific changes by the same reasoning and facts (the only 
decisive ones ! ) as the so-called specific gummatous changes. 

I believe that no one to-day will seriously maintain that these conditions 
cannot be of syphilitic origin. But we have no scientific proof that they are 
actually syphilitic, nor for the assumption that the " gummatous " changes are 
invariably specific. 

I shall refer only to the views of Leredde 2 who maintains with great 
warmth, and for the reasons indicated by us, that the tabetic lesions are cer- 
tainly syphilitic ; also to the recent view of Fritz Lesser 3 that tabes belongs 
to the fourth stage of syphilis, that the interstitial chronic inflammatory 

1 " Bemerkungen zur pathologischen Anatomie der Syphilis des centralen Nerven- 
systems." Deutsche Zeitschr. f. Nervenheilk., 1902, XXII. 

2 Leredde, " La question de la parasyphilis." Progres medical, 1902, Nr, 14, 

3 Berliner hlin, Wochenschr., 1904, Nr, 4, 



PATHOLOGIC ANATOMY 505 

processes are directly produced by the syphilitic virus, a view which at present 
I do not share, and which has been rejected by the competent scientists of 
Berlin; proof of this is still lacking; time will disclose whether there is any 
truth in the opinion. 

Notwithstanding this — and considering the impossibility of pathological 
anatomy leading us to any decision — there is no well founded objection to 
our recognizing the tabetic changes as actually syphilogenous ; on the con- 
trary, it is in a high degree likely that they depend directly upon syphilis, and 
are in some way produced by this affection. 

This conclusion is borne out by the recently recognized results of cy to diag- 
nosis of the cerebrospinal fluid, as elaborated by Widal, by numerous authors 
in France (Sicard, Babinski, Nageotte, Raymond, Brissaud, Pierre ]\Iarie, 
Ravaut, Meige, and others) and also in Germany (Schonborn, Frenkel, D. Ger- 
hardt, Mayer, and Xissl) ; this is a subject of absorbing interest, to which a 
few words will be devoted. 

While but few lymphocytes are found in the normal cerebrospinal fluid, 
even by minute methods of investigation — i. e., not more than 2 to 4 in a 
microscopic field — in cases of active syphilis of the meninges, of the spinal 
cord, or of the brain these are decidedly increased, and from 20 to 200 may 
be counted in a field; this same increase is found almost invariably in recent 
and old cases of tabes and in progressive paralysis, therefore in so-called meta- 
syphilitic affections, while in all other chronic diseases of the brain and spinal 
cord it is either absent or only occasionally met with (as in multiple sclerosis). 
A similar lymphocytosis has been observed only in herpes zoster and in certain 
acute forms of infectious meningitis, especially the tuberculous, in which it is 
combined with a polynuclear leukocytosis. It is a specially interesting fact 
that the same leukocytosis is found in isolated cases of reflex pupillary rigidity, 
which is almost pathognomonic of syphilis, in the headache of chronic syphilis, 
and in hemiplegia with a luetic basis. How often it may occur in. the sec- 
ondary and tertiary stage of syphilis without nervous symptoms can only be 
determined by further researches. In his investigations of syphilis in all 
stages of the disease, Ravaut claims to have found lymphocytosis without even 
a trace of disease of the nervous system, and it is absent only in syphilis with 
mild cutaneous and mucous membrane implication. 

These reports were tested by Dr. Schonborn ! in a large series of cases in 
my Clinic, and in the main were confirmed; among 35 cases of tabes, lympho- 
cytosis was found in every instance. 2 

If these findings continue to be corroborated — and there can scarcely be 
doubt of this — they are certainly of paramount importance in the conception 
of tabes (and of paresis) as a syphilogenous affection. Lymphocytosis may 
therefore be included among the positive pathologico-anatomical findings, and 
with syphilis of the meninges is probably a chief factor in the pathogenesis 
of tabes. 

Of course, I am far from regarding these investigations as final. The 
time has been too short, and the number of cases observed is not large enough. 

i " Die Cytodiagnose des Liquor cerebrospinalis." Neurol. CentralbL, 1903, Nr. 13. 

2 In the last case, just investigated, in which syphilis could not be demonstrated, 
lymphocytosis was not present ; the diagnosis is not quite clear, and we incline some- 
what to the view of a neuritic pseudo-tabes. 
34 



506 TABES DORSALIS 

But I believe them to be important auxiliaries for those who regard the patho - 
logico-anatomical changes in tabes as most likely the sign of a syphilitic infil- 
tration and a late syphilitic manifestation. We will no doubt come to a conclu- 
sion in the near future. 

Of the pathologico-anatomical processes not referable to the nervous system 
but little need be said; probably the only noteworthy ones are the changes 
in the bones and joints. Tabetic arthropathy I shall briefly sketch, refer- 
ring the reader for details to text-books on pathological anatomy. It is a 
peculiar form of arthritis deformans which sets in with an enormous effusion 
of serous or slightly hemorrhagic fluid into the joints and their surroundings 
(subcutaneous edema), with swelling and proliferation in the synovial mem- 
brane, and in all cases leads with comparative rapidity to destruction of the 
joints, atrophy and loosening of the cartilage, flattening, atrophy and rare- 
fication of the head of the joint, of the epiphyses, and of the floor of the 
joint, to loosening of the osseous parts, etc. ; there is also accompanying but 
slight osteophyte formation. The ligaments undergo atrophy and tear, the 
surrounding muscles also atrophy, and sub-luxations, luxations, loose joints, 
pseudo-arthroses, and anchyloses are the consequences. Suppuration or tuber- 
culosis of the joints is rare. The joints most frequently implicated are those 
which have been mentioned. 

The abnormal friability of the bones which leads to spontaneous fractures 
is the result of a rarefying osteitis and of osteoporotic processes, of an increase 
of the medullary substance, of atrophy of the solid bony tissue, and of osteo- 
malacic softening. The tabetic foot, atrophy and necrosis of the jaws, the 
falling out of the teeth, and mal perforant, etc., which result from similar con- 
ditions, require no description. 

Every possible form and degree of atrophy is present in the muscles, in 
part simple diffuse emaciation and atrophy, in part more localized forms and 
degenerative atrophy of the usual type, as is evident from the clinical de- 
scription. 

In the internal organs — the respiratory, circulatory, digestive, urinary, and 
sexual systems — as a rule nothing characteristic of tabes is found, certainly 
nothing more than those conditions which appear in the terminal stages of any 
chronic spinal affection. 

Fritz Lesser's pathologico-anatomical investigations are noteworthy, for 
he demonstrated that every fifth tabetic has an aneurysm, and this has some 
bearing on the syphilitic etiology. His figures, however, have been disputed. 

We must still devote a few words to the special pathogenesis of the ana- 
tomical changes, i. e., their deduction from the causes of tabes, and, owing 
to our limited knowledge of these affections, we may be brief. 

We believe that under the discussion of the etiology we proved that syphi- 
lis is by far the most important and most common, perhaps even the invariable, 
cause of the development of tabes, that the anatomical changes may be regarded 
as directly or indirectly produced by syphilis. Therefore, they may represent 
a third or, as was recently proposed by Fritz Lesser, perhaps a fourth stage in 
the manifestations of syphilis, or are certainly closely related to them. Many 
hypotheses have been suggested to explain the nature and manner of this inti- 
mate relation. 



PATHOLOGIC ANATOMY 507 

Unfortunately we have as yet no actual knowledge of the pathogenic cause 
of syphilis, but it is most likely of bacterial nature (somewhat analogous to 
the pathogenic agent of tuberculosis, but having quite different effects) ; we 
know nothing of its directly irritating and directly damaging action on the 
tissues, nor whether the pathologic agent itself, its products of metabolism 
(toxins), or the poisonous products produced in the tissues themselves and 
their antitoxins — immunization products, etc. — play a role in the secondary 
and tertiary syphilitic manifestations, or, if so, to what extent. We cannot 
understand as yet how the syphilitic virus or its products can so often remain 
latent in the body for years and decades, yet preserve their vitality, then sud- 
denly become active, and produce new forms of the disease. 

We are, therefore, absolutely restricted to theories, and it remains a mooted 
question whether an investigator shall regard as the cause of the tabetic changes 
the actual syphilitic virus, or its products of metabolism, or the products of 
an interchange of tissue by which the syphilis present in the body produces 
its pathologic effects. 

Nevertheless the resemblance, already referred to, of the tabetic changes 
to certain inorganic, organic, and bacterial toxic effects, makes it seem likely 
that in tabes there is such a poisonous effect, and that it probably originates 
from syphilitic infiltration. This thought was first developed in a most 
explicit way by Strtimpell (syphilitoxin). 

Whether these poisons are to be sought for among the actual syphilitic 
toxins, or in the toxins and antitoxins developed in the organism, possibly 
in the superabundance of the products of immunization which have developed, 
is still unknown. The unending complexity of this realm which has been 
revealed by modern bacteriology scarcely permits an opinion, much less a satis- 
factory conclusion. I refer here to my detailed description of this condition 
in a clinical lecture. "The Etiology of Tabes/' published in 1892. 

Therefore, except for the fundamental fart of its sijpliiJogcnous origin, 
everything is still uncertain. 

The rarity of tabes among syphilitics, and the undoubted effect in certain 
cases of a so-called predisposing cause, necessitate the brief consideration of 
this form of development. We must, however, bear in mind that these condi- 
tions in themselves never give rise to tabes except when there is a preceding 
syphilitic infiltration. 

We cannot comprehend how a single, or repeated, exposure to cold could 
produce such a prolonged and progressive affection, which, after decades, impli- 
cates different regions of the nervous system ; if it damages the tissues at all, 
it may in this way form a starting-point for the syphilitic poison, but scarcely 
more. 

This is more readily comprehended in the case of trauma, the damage done 
to the tissue having been determined with certainty, and it may also occasion- 
ally give rise to chronic progressive changes in the nervous system. But 
trauma is very rare as a preceding cause of tabes, and in most cases it pro- 
duces an effect simply because it forms a point of attack, a port of entrance, 
for the syphilitic poison which leads to the development of tabes. 

The effects of over-exertion and sexual excesses can be clearly understood 
from Edinger's masterly substitution hypothesis ; in this we assume processes 
of metabolism, increased consumption, etc., which, with insufficient substitu- 



508 TABES DORSALIS 

tion, may very likely lead to demonstrable degeneration; but the fact that 
these conditions are operative only in the case of syphilis decidedly militates 
against its general or decisive importance. That these processes in other dis- 
turbances of metabolism, in anemia, cachexia, faulty nutrition, etc., rarely 
cause tabes, and only when there is preceding syphilis, decidedly favors the 
fact that they merely open the way to syphilitic tissue disturbance, and, com- 
bined with imperfect substitution, produce the progressive tabetic disease. 

We must consider in a similar way the etiologic effects of certain poisons 
(alcohol, tobacco, ergotin) and of some infections (influenza, etc.), also a 
damage to tissue which in itself rarely leads to the disease but only when it 
is associated with the syphilitic poison; in all of these cases there is a com- 
bination of toxic effects, among which the syphilitic is the most important and 
decisive. 

Therefore, much is still uncertain, and the hypothesis of intoxication ap- 
pears to me to be the most plausible one ; in many cases, so far as syphilis is 
concerned, it appears to be quite sufficient; in other instances we must also 
consider disturbances of metabolism and a loss of regenerative power in the 
used-up material. The latter can only wisely be assumed with limitations, since 
it appears that the causes without syphilis probably do not produce tabes or 
only in the rarest cases. It is really most remarkable that tabes does not more 
commonly result from other toxic and infectious causes, such as debilitating 
influences, anemia, cachexia, etc. ; the fact that it does not is an impressive 
confirmation of the assumption that it has a definite and specific cause. 

We will now attempt to show, as far as possible, the relations of the 
pathologico-anatomical changes to the symptoms of tabes. 

V. PATHOLOGIC PHYSIOLOGY OF TABES. PATHOGENESIS 
OF THE DIFFERENT SYMPTOMS 

In spite of their apparently very simple relations, the deduction of the 
individual symptoms of tabes from the anatomical changes, and the minute 
analysis of their development and form, still occasions perplexity. This be- 
comes greater rather than less with increasing knowledge, and while our for- 
mer views, based upon comparatively crude physiologic tests, simply main- 
tain that " tabes is essentially a disturbance of the sensory centripetal functions, 
the conduction of which takes place in the spinal cord, particularly in its pos- 
terior half, and that in tabes we find the posterior columns degenerated, there- 
fore its symptoms depend chiefly upon this degeneration," yet, when the com- 
plicated structure of the posterior columns was disclosed, and the extraordi- 
narily manifold disturbances of sensation occurring in tabes were considered, 
it was recognized that this simple statement of the facts did not sufficiently 
explain the subject under discussion. Considering the enormous difficulty 
of obtaining from animal experiments any results regarding sensory conditions, 
it is evident that the physiology of sensory conduction in the posterior columns 
is much more likely to be solved by tabes than that the pathogenesis of the 
sensory disturbances in tabes can be explained by physiology. 

The certain demonstration that the posterior columns are chiefly composed 
of the nerve tracts brought to them by the posterior roots, that the pathologico- 
anatomical changes in tabes are unquestionably confined to the posterior root 






PATHOLOGIC PHYSIOLOGY OF TABES 509 

areas, and that the initial symptoms of the same indicate a radicular arrange- 
ment, sustains the former views, although with slight modifications. Of 
course, our insight into these processes is still far from satisfactory; we will, 
therefore, be brief in our presentation. 

Undoubtedly various disturbances of cutaneous sensibility may be* deduced 
from the progressive degeneration of the posterior root tracts. That pares- 
thesia as well as simple hypesthesia, the radicular and localized disturbances 
of the tactile sense and pain sense, probably also the manifold symptoms of 
hyperesthesia, may in general be referred to these degenerations can hardly be 
doubted. But in the individual case it is difficult to explain the many varia- 
tions in the sensory disturbances: Why in one case the tactile sensation, in 
another that of pain, almost exclusively suffers; and why isolated hypalgesia 
and thermo-hypesthesia appear ; where the retardation of pain conduction and 
the peculiar localized hyperesthesia to cold, etc., originate, cannot be stated 
with certainty. Is it the different localization of the process in the individual 
root bundles, or the varying intensity or nature of the process, or the localiza- 
tion at various heights of the intraspinal tracts, or the implication of the gray 
substance to which, we attribute it ? Xothing but theories can be adduced in 
answer to these questions. 

The implication of the peripheral sensory nerves in this disturbance is also 
quite obscure. 

The attacks of lancinating pain are exceedingly difficult to explain on 
account of their singular variations as to intensity, frequency, and localization. 
Naturally, we locate them in the sensory root tracts, but what process causes 
them? To what is their paroxysmal character due? Must we consider marked 
but transient inflammatory irritation, or toxic irritation, or a seeming dis- 
charge of nervous energy similar to that in neuralgia, or are they to be referred 
directly to the peripheral nerves? For the present all these are matters of 
doubt. 

Disturbance of the so-called muscle sense, the entire gamut of symptoms 
arising from a decrease of sensation in individual areas of the skin, the joints, 
the ligaments, the bones, the muscles, etc., and which early manifests itself 
as Romberg's sign, undoubtedly can be attributed to a disease of the centripetal 
tracts of conduction. But whether these are situated in the roots and spinal 
cord, whether in special tracts, or whether they possess different nerve fibers, 
no one can state with certainty. 

The sensation of fatigue, so often present at the onset, is best included 
here, as well as the occasional absence of the sensation of fatigue. 

We have more accurate knowledge regarding the pathogenesis of the reflex 
disturbances, especially of the tendon reflexes. The apparently positive fact 
that the root fibers which come from the lateral root zone, radiating into the 
gray posterior column, and perhaps anteriorly into the anterior column, the 
so-called reflex collaterals, have been found degenerated, furnishes the sim- 
plest explanation, although it is not entirely satisfactory. Concerning the 
tendon reflexes, we know more definitely the area of the spinal cord in which 
the respective root tracts are situated; for instance, for the patella tendon 
reflex it is the region from the second to the fourth lumbar segment, for the 
tendo Achillis that of the third to the fifth sacral segments, disease of the cor- 
responding root zones being indicated if the reflexes are abolished. For the 



510 TABES DORSALIS 

tendon reflexes of the upper extremities, the localization must be sought in 
the sixth and seventh cervical segments. 

The conditions are different with the cutaneous reflexes; since these are 
by no means parallel with the tendon reflexes, and they may possess other 
reflex arcs — complicated tracts of reflex processes — we cannot maintain that 
the reflex collaterals are situated at a height the same as, or similar to, those 
of the tendon reflexes. Clinical experience leads us to consider the con- 
duction tracts (the long ones?) and probably also their collaterals which 
transmit the tactile sense; but where are these situated? We do not 
know. It is very likely that the conspicuous hypotonia of the muscles is 
also due to disturbances in the reflex tract, but this cannot be positively 
proven. 

We are still in doubt as to the tracts implicated in bladder and sexual dis- 
turbances; we know that their centers are situated in the lumbar and sacral 
portions of the cord, also that at many points these must be connected with 
the higher portions of the spinal cord and of the brain. That the centripetal 
tracts must also be primarily affected is self-evident; but there is no doubt 
that in these disturbances motor and vasomotor (therefore centrifugal) tracts 
must also be considered. In regard to their situation (in the sacral segments 
and root regions?) we know but little (I shall not discuss the theory recently 
suggested that some of them lie external to the spinal cord in the sympathetic), 
and still less of their implication in tabes; hence it is useless to propose com- 
plicated hypotheses to support this view. 

In a small number of cases we demonstrate the cause of the spinal motor 
disturbances (pareses, paralysis, atrophies) to be degeneration of the gray ante- 
rior columns and atrophy of the ganglion cells, the gray anterior roots, etc.; 
in a large proportion of the cases, however, only peripheral neuritic changes 
in the motor and mixed nerves can be proven, but these furnish a satisfactory 
explanation. 

For the more widely distributed atrophy of the muscles (Dejerine) no 
anatomical foundation is as yet known, but this also is probably a chronic 
neuritic change. Whether a lesion of the anterior root, of the nerf radicu- 
laire, discovered by Nageotte, is in any way connected with it, is still a matter 
of conjecture. 

For the many trophic disturbances which we have learned to recognize 
among the symptoms of tabes there is as yet no rational anatomical founda- 
tion; it is obvious that we must assume a neurotic origin for the typical 
processes of arthropathy, osteoporosis, etc.; there are sufficient, and almost 
forcible, clinical reasons for such assumption ; anatomical facts, however, upon 
which to base it are not at hand. Therefore we need not minutely discuss the 
various hypotheses, some of which refer the lesion to the posterior columns, 
through this to the gray posterior columns, and even to the anterior columns ; 
some to the spinal ganglia or to the peripheral nerves. 

It is quite unlikely that these processes, such as chronic syphilitic arthritis, 
traumatic or gouty arthritic changes, are all to be traced to the nervous sys- 
tem. Perhaps, as Vulpian assumes, an entire series of deleterious effects of 
nervous or accidental nature is here combined. 

According to all experience, however, herpes zoster, which often occurs in 
tabetics, depends upon a degeneration of the posterior root or of the spinal 



PATHOLOGIC PHYSIOLOGY OF TABES 511 

ganglia, primarily in a peri- and endoneuritis of Xageotte's " nerf radicu- 
laire/' and this is sometimes -communicated to the spinal ganglion. 

The anatomical foundation of the so-called visceral crises is quite obscure. 
They have, perhaps correctly, been regarded as analogous to attacks of lan- 
cinating pain; in spite of some variations, this may be admitted. But, in 
this respect, we have no more accurate knowledge, for we also refer these 
symptoms to the posterior root regions, and, on the other hand, the sympa- 
thetic nerve has also been considered without our being able to adduce any 
actual proof of its involvement; changes in the pneumogastric and spinal 
accessory, or in their bulbar nuclei, have been considered to be the cause 
of gastric, laryngeal, bronchial, and cardiac crises, but this has not been 
proven. 

We now come to changes in the cranial nerves, which cannot be regarded 
as forming part of the spinal changes, but which can nevertheless be desig- 
nated as localizations of the tabetic process. 

Amblyopia and amaurosis are always due to the well known gray degenera- 
tion and atrophy of the optic nerve, and this can invariably be demonstrated. 
Similar conditions are found in the olfactory and auditory nerves, but these 
disturbances, as a rule, are quite rare. 

Paralysis of the ocular muscles, which, in the first stage, is usually transi- 
tory, is referred to neuritic or syphilitic processes in different nerves of the 
ocular muscles. In the later stages extreme peripheral degenerations, espe- 
cially those in the nuclei of the nerves of the eye upon the floor of the third 
ventricle, have been demonstrated (nuclear paralysis of the muscles of the 
eye) ; analogous to these are bulbar nuclear degenerations which lead to paral- 
ysis of the facial, trigeminal (motor portion) and hypoglossal nerves, and to 
actual bulbar paralysis ; furthermore, occasional atrophy of the Gasserian gan- 
glion and of the trigeminal nerve has been found in the same symptom-com- 
plex; finally, degenerations of the pneumogastric, spinal accessory, and recur- 
rent laryngeal have been noted in paralyses of the muscles of the larynx. All 
of these conditions, however, are quite obvious. 

Two of the most constant and important symptoms of tabes are still to be 
analyzed and localized: reflex pupillary rigidity with myosis, and ataxia. Both 
have been the subject of comprehensive study and much discussion. 

Reflex pupillary rigidity and myosis are two independent phenomena, but 
they occur in association with extraordinary frequency. The former usually 
precedes the latter, therefore more frequently occurs alone, or may long be 
an initial symptom. The anatomical localization of reflex pupillary rigidity 
may very easily be determined theoretically, but practically it has not yet been 
demonstrated. It is clear that this disturbance cannot be located in the 
peripheral optic (centripetal pupillary), nor in the peripheral oculomotor 
(centrifugal pupillary), tracts; it must therefore be sought in an intermediary 
tract, probably in the first optical, and its adjacent oculomotor, centers, or in 
a special center. This was supposed to be due to changes in the central gray 
of the cavities or in Westphal-Edinger's nucleus, and the region of the cilio- 
spinal center in the cervical cord was also formerly thought of; the ciliary 
ganglion too. for weighty reasons, has been considered. Eecent investigations, 
however, have shown such manifold localizations, and so many modes of devel- 
opment, as to make it almost impossible for us to obtain accurate knowledge 



512 TABES DORSALIS 

of these conditions. The researches of Bach x clearly portray these difficulties 
and obscurities. It is impossible for us here to discuss this question, which 
has engaged the attention of Majano, Bernheimer, Bach and H. Meyer, Cas- 
sirer and Schiff, Ruge, Levinsohn, Axenfeld, Uhthoff, v. Hippel, and others; 
moreover, it is unnecessary, since no conclusive opinion is possible, and the 
condition remains obscure. 

It is now assumed that there are contained pupillary fibers in the optic 
nerve which produce a reflex, and these are subsequently detached from the 
original optic tract (in the neighborhood of the geniculate ganglion). Noth- 
ing is more obvious than the assumption that primary degeneration of these 
fibers (which may be regarded as analogous to the posterior root fibers and 
their reflex collaterals) produces pupillary rigidity; but it was found in reflex 
pupillary rigidity that if the optic tract was clear, as is usually the case, the 
nerve of sight was absolutely normal. Moreover, no conclusive clinical or 
anatomical reason could be assigned for the localization of the lesion in the 
region of the corpora quadrigemina, the nuclei of the oculomotor nerve (West- 
phal-Edinger's nucleus, etc.) ; therefore this localization was not accepted. 
This is opposed by anatomical, and chiefly by experimental, investigations 
(particularly those of Bach and H. Meyer), which clearly show that there are 
anatomical relations between the medulla oblongata — that is, the upper por- 
tion of the cervical cord — and the reaction of the pupils. 

Bach's experiments first led him to assume a reflex center in the upper 
cervical cord, also an inhibitive center at the spinal end of the fourth ventricle 
near the median line for the reflex of light (contraction of the pupils), as 
well as for dilatation of the pupils, and he attempted to explain the tabetic 
reflex pupillar}^ rigidity, as well as the myosis, by a persistent irritation of 
this inhibitive center. He is not exactly enthusiastic over this explanation, 
and it appears to me that the assumption of an irritation of these centers which 
lasts for years and decades, and which never produces paralysis, controverts 
everything that we know of tabes and its anatomical foundations; therefore 
it is absolutely inconclusive. 

Bach found by somewhat contradictory experiments that division of the 
cervical cord somewhat below the medulla oblongata leaves the pupillary reac- 
tion intact; destruction of the same close to the medulla at once arrests the 
light reflex; section of the medulla at the spinal end of the fourth ventricle 
immediately results in rigidity to light ; irritation of the medulla at its spinal 
end also causes rigidity to light with myosis, which, however, disappears at once 
if the medulla is divided in the center of the fourth ventricle, or even some- 
what higher. In my opinion these findings permit the conclusion that an im- 
portant, and not a subordinate, reflex center for the pupil is situated ante- 
riorly and above the medulla, but that the main reflex center is to be found 
in the upper cervical cord; further experiments will decide. Most of these 
researches were made in cats, and, considering the peculiar pupillary reactions 
of these animals, they can be applied to man only with the greatest reserve. 

The fact that, in a few cases of reflex pupillary rigidity, changes in the 
posterior column in the upper cervical cord have lately been found confirms 

i L. Bach, " Pupillenstudien." v. Grafe's Arch., 1903, LVIL — " Was wissen wir 
fiber Pupillenreflexcentren/' etc. Zeitschr. f. Augenheilk., 1904, XL 



PATHOLOGIC PHYSIOLOGY OF TABES 513 

Bach's views. Eeichardt (1904) has even declared them to be almost invaria- 
ble, and he locates them in the ventral part of Bechterew's intermediate zone 
at the height of the second and third cervical segments. 

The changes which Marina found in the ciliary ganglion in tabes and paresis 
must be further investigated to demonstrate their significance. 

Taking all into consideration, we may state that the anatomical lesion 
which is the cause of reflex pupillary rigidity is not yet positively known, but 
that it is most likely to be found in the posterior columns of the cervical 
cord close to the spinal end of the fourth ventricle. 1 

The same is true of the common, but by no means invariable, accompany- 
ing myosis. Since cocain causes a prompt dilatation of the pupil, we cannot 
unreservedly attribute this myosis to a paralysis of the sympathetic fibers, 
although this is the most likely cause. Bach explained it as due to an irrita- 
tion of his hypothetical reflex inhibitive centers for contraction and dilatation 
of the pupil. The absence of these two reflexes produces myosis, because the 
tonus of the stronger sphincter is decidedly greater than the tonus of the 
dilator. Xeither has this view been as yet confirmed. 

At all events the mechanism of pupillary movements and the tonus of the 
pupillary muscles appear to be much more complicated than was previously 
supposed; by continued research, especially in tabes, some unexpected conclu- 
sions may be reached. 

Ataxia still remains to be described, and this is undoubtedly the most 
interesting, as well as the most difficult, chapter in the pathological physiology 
of tabes. 

Since the accurate formulation of Duchenne's conception, endless discus- 
sions have taken place and innumerable clinical observations and animal 
experiments have been made, without, however, yielding any definite results, 
nor leading to a final unanimity of opinion in place of these diametrically 
opposite views. The nature and pathogenesis of ataxia are primarily of 
most theoretic importance for the normal and pathologic physiology of the 
spinal cord; but they have recently become of practical significance because 
the relief of this symptom dominates the treatment. We would digress 
too far were we to make at this point a comprehensive investigation of the 
question of ataxia. Volumes could be filled. We shall therefore attempt 
to be brief. 

As a rule, we understand by ataxia the disturbed coordination of volun- 
tary movements; but even the conception "coordination of movements" is 
difficult to understand. We may say that for either simple or complicated 
movements, also in steadying the extremities and the trunk for the purpose 
of maintaining the equilibrium, for balancing, for maintaining certain posi- 
tions, etc., carefully graduated innervation of more or less numerous muscles 
is necessary, provided that actual coordinated impressions with the hast possi- 
ble expenditure of strength and minimal sensory impressions is to result. 
For this the correct choice of the many innervated muscles and muscle groups, 
the correct degree, rapidity, and sequence of their innervation (including the 

i Compare W. Uhthoff , " Augenveranderimgen bei Erkrankung des Xervensystems," 
in Grafe-Samisch's Handb. der Augenheilk., 1904, XI, 2. Aufl. ; and Bumke, "Die Pupil- 
lenstorungen bei Geistes- und Nervenkrankheiten," Jena, 1904. 



514 TABES DORSALIS 

antagonists) is necessary. If, in any of these essentials, there is even a slight 
disproportion, " disturbance of coordination " results. 

This faculty of coordinating innervation is chiefly the result of long-con- 
tinued practice in the accumulation of certain impulses of movement, which 
are then gradually exerted upon a certain tract, and the conjoint effect of 
impulses of the will is the result ; but we assume that these are dominated 
by certain parts of the central apparatus which are designated coordination 
centers, these having for a long time been referred by physiology to the brain 
(the trunk of the brain, especially the cerebellum; some of them recently to 
the cortex of the cerebrum). It has been determined that the existence of such 
centers is absolutely necessary for the impulses by which we maintain the 
equilibrium and the erect posture in space. They may perhaps be only areas 
for the transmission of stimulations of the will, only a meeting point for the 
various innervation tracts, an apparatus for the reception of centripetal sen- 
sory stimulations, and a point of communication between these centripetal 
and centrifugal tracts ; but for the comprehension of fine and complicated proc- 
esses they can scarcely be dispensed with. 

These so-called " centers " apparently act upon the motor apparatus only 
through centrifugal tracts — the spinal and peripheral conduction tracts and 
muscles — which may possibly unite in the brain or the pons, some of them 
perhaps in the spinal cord, with the direct motor conduction tracts. Appar- 
ently the spinal cord itself does not transmit combinations of voluntary move- 
ments, but by means of complicated reflexes, and by the assumption of so-called 
" coordinated reflexes," which are to be differentiated from the other spinal 
(tendon and cutaneous) reflexes, this is not inconceivable. 

The exact relations of the centers of the will to these coordination centers 
are still obscure; undoubtedly the latter are influenced and set in motion by 
the will, but a clear conception of this is impossible. 

Moreover, there are numerous centripetal impressions and stimuli which 
reach the apparatus of coordination from the periphery, and stimulate or 
modify its activity for every possible kind of movement; impressions which 
transmit to the external world the relations of the body, the relations of the 
internal parts of the body to each other and to the central organ (by no 
means always the organ of consciousness!), every possible position of the 
joints, the starting-point of movements and much besides, and thus aid greatly 
in the regulation of voluntary and involuntary movements — their production, 
duration, and increase, as well as their decrease, or entire inhibition. Among 
these are the centripetal stimulations originating from the sensory nerves of 
the skin, of the joints, of the muscles, of the tendons, of the ligaments, etc., 
some not extending to consciousness at all, and some being active only reflexly. 
Some of the areas from which these centripetal stimulations are transmitted 
to the motor ganglion cells and conduction tracts may be situated in the 
spinal cord ("spinal coordinative reflex tracts"), some in the cerebellum 
("cerebellar coordination"), some in the cortex of the cerebrum, or in the 
motor cortical regions ("cerebral coordination"). To this must be added 
the important sensory stimulations which either by the optic tract and the 
optic centers, or by the semicircular canals of the labyrinth of the ear and 
the tract of the auditory nerve (vestibular nerve) exert a, powerful influence 
upon the coordination of movements; these are the visu-cerebral and the ves= 



PATHOLOGIC PHYSIOLOGY OF TABES 515 

tibulo-cerebellar centripetal mechanisms, which are undoubtedly of vast im- 
portance for coordination. 1 

Centripetal sensations are absolutely necessary to explain the nature of 
most of the complicated movements. The child at birth is in truth ataxic, and 
it learns its first coordinate voluntary movements, such as touching, grasp- 
ing, standing, walking, and those involved in speech, solely by persistent at- 
tempts at movement, and by exercises which are regulated by the cutaneous, 
arthritic, and muscular sensibilities of sight and hearing. For the rapid and 
apparently automatic movements which have been acquired — manipulations, 
walking, writing, piano-playing, etc. — this regulation seems to be no longer 
necessary, or only to a slight degree; there is no longer time for it, especially 
if the movements are performed quickly or by a single energetic action (such 
as throwing, jumping, etc.). But accurate analyses, such as Frenkel and 
0. Forster have made, of these various movements show that both movement 
conceptions and coordinate movements are invariably dependent upon certain 
centripetal impressions. If these are new, unfamiliar, or rarely experienced, 
the movements in the normal person will again resemble the incoordinate 
movements of the child or the tabetic (for example, in walking upon rough 
or strange paths, slippery ice, narrow stairs, in the dark, etc.). 

To maintain the equilibrium and an erect posture in space (balancing the 
body, especially the trunk, under various circumstances) these centripetal 
impressions are probably always necessary; they regulate most accurately and 
rapidly, and entirely without the exercise of the will, the numerous innerva- 
tions of the muscles of the trunk, of the vertebral column, and of the legs, 
which are necessary for the performance of these acts. The slightest disturb- 
ance of these sensations (for instance, of the muscular sense) on closing the 
eyes (Eomberg) betrays itself by faulty and uncertain muscular action in the 
form of swaying. 

Frenkel recently demonstrated conclusively that the maintenance of the 
normal muscular tonus, particularly in certain muscle groups of the legs and 
vertebral column, is decidedly important for the correct coordination of vol- 
untary movements. That certain reflex effects may also be instrumental in the 
performance of these complicated functions is clear, but we cannot minutely 
discuss them at this point. 

It is therefore obvious that a disturbance in the coordination of move- 
ments may take place in very different areas, tracts, and centers. In addi- 
tion to disturbance of the organs of volition, derangement of the coordinating 
central apparatus (anomalous stimulation and activity) comes into question; 
first, disturbances in the centrifugal coordinating conduction tracts originat- 
ing from them, some of which are still hypothetical ; next, those disturbances 
(especially a decrease of the stimulus conveyed by the centripetal sensory 

1 The ingenious diagram of Otfried Forster. which clearly explains the centripetal 
coordinative stimulations and transmissions, gives us a comprehensive, although com- 
plex, picture of the extremely involved processes of coordination which here take place. 
Unfortunately we have no proof of their actual existence and correctness : much con- 
tained in the diagram is hypothetical. But it must be admitted that it furnishes a 
plausible conception, and still leaves room for the assumption of centrifugal coordina- 
tive tracts which appear to be indispensable for the conduction of stimulations, especially 
the cerebello-fugal and cerebro-fugal, in the processes of coordination. 



516 TABES DORSALIS 

nerves) some of which are transmitted by paths in the spinal cord, some in the 
cerebellum, and some in the cerebrum; then disturbances of certain reflexes; 
finally, disturbance of the muscle tonus, hypotonia of the muscles, which is 
invariably present, and is certainly of vast significance in regulating the meas- 
ure of individual movements. 

Obviously the question now arises which of these disturbances is especially 
responsible for tabetic ataxia, whether it is only one, whence the ataxias would 
be of uniform origin, or, if several act simultaneously, which are they, and 
do they act in different combinations. Our entire clinical and anatomical 
knowledge of tabes at once makes it evident that we are dealing with compli- 
cated and obscure processes, which in different cases may vary greatly or may 
be combined. Apparently complex conditions act together, these being modi- 
fied by different circumstances, and dependent upon a number of conditions 
and coincidences. 

Actual disturbance of the coordinating centers in tabes, which is regarded 
as a disease of the spinal cord, is scarcely likely. It is true that a form of 
ataxia occurs in disease of the cerebellum, also of the pons (the so-called 
cerebellar ataxia), and of the corpora quadrigemina — perhaps also in disease 
of the frontal brain. But both in its nature and symptoms it distinctly 
differs from spinal, tabetic ataxia; therefore it need not be considered. The- 
ories which attempt to refer tabetic ataxia to certain lesions in the cortex 
of the cerebrum and cerebellum have been rejected, and must be regarded as 
untenable. 1 

We must now investigate true tabetic spinal ataxia. For its form and 
mode of expression, as well as for a number of important details, we refer 
the reader to the description in Frenkel's book 2 which forms an original and 
comprehensive study of the whole subject of ataxia and its associated condi- 
tions, varieties, and complications; for more specialized instruction the work 
of Otfried Forster, 3 which treats the entire condition most exhaustively, is 
valuable. This author first studied each individual joint and each individual 
muscle group of the lower extremities — some for the relation of the " differ- 
ent factors " — namely, sensation, tendon reflexes, tonus, passive tension, etc. — 
then the behavior of the muscles as agonists, agonistic synergists, as antago- 
nists (that is, also as collateral and rotary synergists), finally the resulting 
anomalies in position and movement of the affected portion of the members. 
He described and analyzed these, and was inclined to attribute them to a 
simultaneous disturbance of centripetal irritation. He then analyzed similarly 
the processes of standing, walking, rising, sitting down, ascending stairs, etc. 
Subsequently he examined in the same way the muscles of the trunk, the indi- 
vidual muscle groups, and the joints of the upper extremities, particularly 
during the act of writing; finally, the muscles of the head, face, tongue, 
larynx, and eyes. 

i Time will show whether the communications of C. Weigert (published after the 
untimely death of the author) in regard to invariable changes in the molecular layer 
of the cerebellum are of any value. 

2 H. S. Frenkel, " Die Behandlung der tabischen Ataxie mit Hilfe der Uebung." 
F. C. W. Vogel, Leipzig, 1900. 

s Otfried Forster, " Die Physiologie und Pathologie der Coordination." G. Fischer, 
Jena, 1902. 



PATHOLOGIC PHYSIOLOGY OF TABES 517 

This author compiled an enormously rich mass of material which shows 
how many-sided and complicated, and how pathogenetically different, the 
ataxic movements are. His investigations will bear close study, and are cer- 
tainly invaluable for the sensory theory of ataxia. 

If we remember that tabes is essentially a disease of the spinal posterior 
columns — the spinal root areas — and therefore represents centripetal conduct- 
ing tracts, that theory will appear to be the most rational which deduces tabetic 
ataxia from a disturbance of the centripetal sensory conduction tracts. 
Frenkel, who has recently made a most thorough study of its clinical aspects, 
states that ataxia is the reaction caused by disturbances of sensation in the 
arthritic and muscular systems, and to some extent in the skin. 

This is the sensory theory of ataxia which was first promulgated by Ley- 
den in 1863, and has since then been repeatedly and zealously advocated, being 
somewhat modified by numerous clinical and experimental facts. 

This theory is chiefly based upon clinical observation, upon the appar- 
ently invariable disturbance of cutaneous sensibility, of conscious and uncon- 
scious sensations, above all of the sensations conveyed by the joints, muscles, 
and tendons ; this Goldscheider and others stanchly maintain. The constancy 
and proportional relation of these disturbances to ataxia have been, and are 
still, doubted. Frenkel has recently strengthened this view by asserting that 
tabetic ataxia is usually combined with a derangement of cutaneous sensation, 
alv:ays with disturbance of arthritic sensation, and in severe cases with dis- 
turbance of muscular and tendon sense, and he declares that the assumption 
that tabetic ataxia is not accompanied by sensory disturbance depends upon 
erroneous and incomplete researches. He further declares that the degree of 
ataxia is always in proportion to the degree of sensory disturbance; that is, 
the most markedly hypesthetic joint, the extremely hypesthetic extremities, 
also show the most profound ataxia. This author refers the statement that 
distinct sensory disturbances are occasionally present in tabes without ataxia 
to inaccurate deductions (for the investigation of ataxia he has proposed a 
series of exceedingly minute tests!). 

An effort was made to base this theory upon experimental findings. For 
these sections were made of the posterior roots of one or more extremities, 
either unilateral or bilateral, in frogs, dogs, and recently in apes ; with regard 
to the questions to be solved, there has been an increasing refinement in the 
operation. 

It is impossible here to discuss minutely these miscellaneous investiga- 
tions ; it is evident that only those performed upon apes will apply to human 
pathology; and here we refer the reader to the numerous subsequent investi- 
gations of H. E. Hering, Mott and Sherrington, A. Bickel, P. Jacob and 
A. Bickel, Korniloff, Merzbacher, and others, from which it is at least cer- 
tain that the arrest of sensation after the severance of the posterior roots results 
in a decided disturbance of motility. Whether this is related to tabetic ataxia, 
or perhaps identical with it, is difficult to prove. It is certainly not true in 
the case of the lower animals ; in dogs it must be accepted with some reserve, 
and probably also in the case of apes. 

It is not my purpose to discuss these investigations at length. I am under 
the impression that they substantiate the sensory theory of ataxia, although 
many doubts arise, and under minute criticism, some of the findings are 



518 TABES DORSALIS 

susceptible of other interpretation. It is remarkable that many of these ex- 
aminations have shown that the disturbance of motion after some time disap- 
pears, although there is never reestablishment of the sensory conduction tracts. 
Vicarious compensatory stimulations appear to be set in motion (by way of 
the collateral sensory tracts, or of the auditory and optic tracts, from the 
sensory sphere of the cortex of the brain, or the like), and after their suspen- 
sion " ataxia " once more manifests itself. 

To the adherents of the sensory theory the occurrence of ataxia (or, more 
correctly, a disturbance of motion resembling ataxia) in peripheral neuritis 
— so-called neurotabes peripherica — was of great significance, and quite prop- 
erly so; but only when actual sensory disturbances existed, and secondary 
changes in the central nervous system were absolutely excluded. These condi- 
tions were certainly difficult to prove. 

The foregoing facts, combined with the thorough consideration of the 
sensibility of the skin, of the joints, of the muscles, and of the tendons, make 
it appear extremely plausible that what we call tabetic ataxia chiefly depends 
upon sensory disturbances. It is certain that when there is anesthesia of the 
soles of the feet their position and movements upon the floor become uncer- 
tain; that with decreased sensation in the joints the excursus of their move- 
ments becomes more extensive and rapid, so that the production of movement 
is apparent to the consciousness; that for the same reason when muscle and 
tendon sensations are decreased the movements become uncertain, exaggerated, 
more marked, or weaker; that with a defective appreciation of the starting- 
point for the members the intended motions are incorrect, misdirected, or 
accelerated; that individual movements for which the tabetics have lost the 
necessary appreciation of sequence, knowledge necessary for movements made 
with a definite purpose, are grossly impeded, etc. It is doubtful, however, 
whether these facts sufficiently explain the fully developed ataxia of tabes. 
We know that disturbances of sensation, in the widest sense of the term, 
are compensated for by sensory control, especially on the part of the eyes; 
but tabetic ataxia in a greater or less degree unquestionably appears when the 
eyes are open, and there is rapt attention. 

Some adherents of the sensory theory appear to have noted this weak 
point in its construction; and one of its most earnest supporters, Frenkel, 
has quite properly utilized a significant factor to explain many of the ataxic 
phenomena : hypotonia of the muscles, which this author has carefully studied. 
He holds this responsible for the jerky, aimless, irregular movements of 
tabetics, for the conspicuous disturbance of the gait, for the anomalies in the 
most important movements of the trunk when walking, and much besides. 
Are we justified in referring this hypotonia to the sensory centripetal disturb- 
ance ? This seems doubtful ! Is the hypotonia to be chiefly attributed to a 
reflex disturbance of tonic stimulation ? It would seem so ! But Frenkel 
has shown that the hypotonia, which he declares to be the initial and almost 
pathognomonic symptom of tabes, is not strictly parallel with the disturbance 
of sensation or with the reflex disturbances. In his opinion, ataxia is by no 
means the consequence of hypotonia, which may exist without ataxia — but it 
undoubtedly has an influence upon the form of the ataxia. 

Otfried Forster, the latest investigator of this subject, after most com- 
prehensive studies and close consideration, comes to the conclusion that tabetic 



PATHOLOGIC PHYSIOLOGY OF TABES 519 

ataxia is due to a disturbance of the sensitive stimulations (" factors ") which 
are indispensable for coordination, of the "subcortical" (spinal, cerebellar, 
that is, unconscious) as well as " cortical " (conscious) sensations. 

Hence he finds the anatomical foundation for ataxia to be a degeneration 
of the reflex collaterals in the spinal cord — the collaterals passing to Clarke's 
columns which penetrate to the cerebellum — and of the long posterior column 
fibers (for the cerebrum) ; its path ologico- physiological foundation, however, 
is a disturbance thereby produced in the spinal sensitive factors (arrest of the 
tendon reflexes, the coordination reflexes, the spinal reflex tonus), in the cere- 
bellar factors (arrest of the cerebellar reflex tonus), and in the cerebral factors 
(conscious sensations). These may individually or collectively be unequally 
affected and thereby produce different degrees and modifications of tabetic 
ataxia. 

Plausible as these deductions seem, and rational as the sensory theory of 
ataxia thus formulated appears, many doubts still confront the candid investi- 
gator. Many have never accepted this theory, and some have designated it 
as untenable. Friedreich did this, upon the basis of his minute researches 
in "hereditary ataxia/' Other authors as well as I (in my text-book) fol- 
lowed him. It is true we know that this theory is suggested for a disease 
which must be sharply discriminated from tabes, and that hereditary ataxia 
differs in many ways from true tabetic ataxia. This may be accounted for 
by the fact that in Friedreich's ataxia marked disturbances of conscious sensa- 
tion do not appear for a long time ; yet undoubtedly " ataxia " exists, and — 
what is of especial importance — also gray degeneration of the posterior col- 
umns, so that, from a scientific standpoint, tabetic ataxia cannot at once be 
eliminated, as the adherents of the sensory theory so naively require us to do. 

For true tabes a number of circumstances may be urged in opposition 
to the unrestricted adoption of the sensory theory, although this, as now 
becomes apparent, is not based merely upon disturbance of conscious sensa- 
tion. We need only mention the following: the occasional occurrence of 
ataxia without any evident sensory disturbance, and the absence of ataxia 
with distinct disturbances of sensation, this being not seldom observed in the 
so-called preataxic stage; the late appearance of ataxia, in contrast to the 
other symptoms, when there is most certainly a lesion of the posterior root 
tracts; and, when both are present, the common disproportion between the 
degree of sensory disturbance and the ataxia. 

These reasons formed sufficient ground for the opponents of the sensory 
theory to reject it; for it is quite evident that the concurrence of sensory dis- 
turbances and ataxia by no means proves that the latter is produced by the 
former; they may be parallel, but entirely independent, symptoms of ataxia; 
but every case in which ataxia without sensory disturbances can be certainly 
proven, and also every case in which distinct sensory disturbances appear 
without ataxia, must help to shatter the sensory theory. Frenkel has attempted 
to nullify these objections by the supposition that the absence of sensory dis- 
turbance in the one case, as well as the absence of ataxia in the other, is only 
a seeming one, such absence not having been observed by the investigator 
because the examination was not thorough, and owing to the assumption that 
sensory disturbances and ataxia are always in exact proportion to each other. 
I must, however, express my doubts of the correctness of these views, and 



520 TABES DORSALIS 

shall suspend my opinion until these statements have been confirmed by others ; 
unfortunately want of time has so far prevented me from investigating them, 
but I shall devote my attention to them in the near future. We are greatly 
indebted to Frenkel for formulating correct methods for testing sensation, 
ataxia, and hypotonia in tabes. 

As an additional objection to the exclusive importance of the sensory 
theory we may briefly mention the persistence of ataxia during sensory control 
by the eyes (as well as by the semicircular canals of the labyrinth) ; these cases, 
in which, when sensory " factors " are absent, the optical control is insuffi- 
cient to prevent ataxia, have lately been especially emphasized by 0. Forster. 
He expressly points out in such cases the apparent dissociation of motor im- 
pulses in primary voluntary innervation which is quite independent of the 
secondary sensitive factors, the exaggerated, aimless, waddling movements in 
all extreme ataxia in spite of well-retained motility and coarse power, and the 
presence of compensatory sensory control; the undoubted appearance of pro- 
found ataxia, not unlike the spinal form, in diseases of the cerebellum, of the 
trunk, of the brain, etc., without any sensory disturbance (the same condi- 
tion being noted in Friedreich's hereditary ataxia). He also maintains the 
development of extremely severe spinal sensory disturbances in other diseases 
of the spinal cord (syringomyelia) without any trace of ataxia; the familiar 
cases of Spath-Schuppel, Engesser, and others, cannot so readily be ignored ! 
Finally, the want of unanimity and the unquestioned rarity of ataxia in neu- 
ritis and polyneuritis (so-called neurotabes peripherica) — in my long prac- 
tice I have certainly seen numerous cases of polyneuritis, yet no typical case 
of this kind was ever observed — lead me to think there must be something 
else, some central or other change, which in neurotabes peripherica (which 
usually depends upon alcoholism) certainly cannot be excluded, but is very 
difficult to demonstrate. 

In the decision of this question too much importance must not be attached 
to animal experiments. The deranged motion produced bears at most but a 
very indistinct resemblance to tabetic ataxia, and the results in animals, whose 
movements of the limbs are much more automatic than those of man, cannot 
readily be applied to human pathology; this is usually permissible only in 
experiments upon apes, in which, it is true, the results are quite remarkable. 

Therefore the sensory theory of ataxia is even to-day incomplete; at all 
events, tabetic ataxia cannot be exclusively, and in a wide sense, attributed 
to tabetic sensory disturbances, although we do not absolutely reject this 
standpoint. 

We must, therefore, seriously consider the possibility of a motor theory, ac- 
cording to which disturbances in the centrifugal (so-called coordinating) con- 
duction tracts are held responsible for the ataxia, the so-called coordinate cen- 
ters being combined with the peripheral motor neuron. These tracts must be 
situated somewhere in the spinal cord. But where ? This we do not know, and 
hypotheses amount to little. It is certainly conceivable that by the tabetic proc- 
ess, irritation and degeneration in these tracts, which usually appear only in 
the further course of the disease — influence " coordination " of movements and 
more or less decidedly disturb it. Y\ T e must view the disease from many stand- 
points in order to ascertain the components of ataxia. In the present state 
of our knowledge we are forced to admit the possibility, and even the likeli- 



COURSE, TERMINATION, AND DEFINITE FORMS OF TABES 521 

hood, of such a process. That the motor theory of well-developed ataxia com- 
bined with degeneration of the posterior columns is the only tenable one, at 
least for Friedreich's ataxia, appears to be established. In how far true tabetic 
ataxia is due to this cause remains a mooted question. We believe the motor 
theory to be more hypothetical and less well founded than the sensory 
theory, but, as it cannot be absolutely denied, the subject must be further 
investigated. 

Special consideration of the reflex theory of ataxia is superfluous. That it 
bears no close relation to disturbances of the ordinary cutaneous and tendon 
reflexes no one doubts. What other factors may be operative in these unknown 
reflex influences — hypothetical " coordination reflexes," etc. — has been dis- 
cussed under the sensory theory, and in our remarks concerning the influence 
of hypotonia; certainly disturbance of the reflexes alone will not fully explain 
tabetic ataxia. 

This shows that our knowledge of ataxia is still obscure; probably there 
are complicated conditions which cannot be understood from one point of 
view; it may be that sensory disturbances, especially of the joints, the mus- 
cles, and the tendons, play a role. But later disturbances in the motor (co- 
ordinating) conduction tracts cannot be excluded, and hypotonia of the mus- 
cles may play an especially important part in ataxic motor disturbances. In 
how far these depend upon centripetal reflex disturbances, or whether or not 
they are to be reckoned among the motor disturbances, therefore are of vast 
significance for the motor theory, cannot be stated. 1 

At all events, ataxia is one of the most interesting symptoms in the rich 
clinical picture of tabes, and one of the most difficult to explain; it furnishes 
to physiologists, neurologists, and experimental pathologists many problems 
which await elucidation. 



VI. COURSE, TERMINATION, AND DEFINITE FORMS OF TABES 

The course of tabes varies greatly in different cases ; usually one type pre- 
dominates, forms the rule, and is found in the majority of cases, although with 
many modifications and peculiarities (the typical form of tabes). We have 
attempted to portray this variety in the description of the symptoms. 

It may be simply divided into three stages : First, the initial neuralgic 
stage, the most important objective and subjective symptoms of which have 
been described above, including a number which are rare. The affection has 
a chronic and slowly progressive development. The first symptom is usually 
lancinating pain which is more or less frequent and intense, is usually regarded 
by the patient as rheumatism, to which but little attention is given; by and 
by other symptoms appear (paralysis of the muscles of the eyes, disorders of 
sight, paresthesia, fatigue, weakness of the bladder and sexual functions, etc. ) ; 
the affection may actually begin with any of these symptoms, but others in- 
variably follow, and may be complemented by many rare phenomena (gastric 
and laryngeal crises, arthropathies, partial paralysis, etc.), while objective 

1 In regard to this Strumpell's interesting report may be consulted : " Ueber die 
Storungen der Bewegungen bei vollstiindiger Anasthesie eines Armes," etc., in the 
Deutsche Zeitschr. f. Nervenheilk., Bd ( XXIII, p. 1, 1902. 



522 TABES DORSALIS 

examination reveals typical symptoms in the pupils, the tendon reflexes, in 
sensation, etc. 

Just as the disease begins slowly and insidiously, so it progresses, usually 
becoming aggravated ; but decided improvement, a long arrest, or rapid aggra- 
vation, may take place. With the gradual or rapidly developing disturbance 
of coordination which soon or late occurs, the patients reach the second, the 
ataxic, stage of the disease, and here a slow but progressive aggravation is the 
rule. For a long time ataxia is limited to the legs, then it gradually involves 
the arms, while the legs become so ataxic that the patients require a cane, or 
two canes, or crutch support ; finally they are compelled to use a rolling chair. 
At the same time the symptoms of the first stage usually increase in severity 
the pain becomes more intense, the attacks more frequent, the paresthesias, 
the bladder disturbance, the sexual weakness, etc., increase, and new symp- 
toms appear; yet the constitutional condition may for a long time continue 
good, or from the onset may reveal severe disturbance. 

After a long but somewhat variable duration of this stage, in which the 
patients are often still able to follow their occupations, the mental powers 
being quite active, they become more helpless. Ataxia of the hands renders 
these incapable of use, the extreme ataxia of the feet, to which paralysis, 
atrophy, and contracture are soon added, prevent the use of the legs, and the 
patients are confined to a rolling chair or to bed, while the general condition 
begins to suffer seriously. This is the third stage of the disease, usually desig- 
nated the paraplegic, although true paraplegia, actual paralysis, is not always 
present, but only an extreme degree of ataxia, with anesthesia, debility, atrophy, 
and contracture of the legs. The distress of the patients is usually increased 
by their helplessness, by pain, and the visceral crises, by paralysis of the blad- 
der to which cystitis is soon added, by bed-sores, and by general cachexia, until, 
in different ways which are soon to be mentioned, death relieves the miserable 
sufferer. 

Each of these stages may last a long time, especially the first two, which 
are sometimes prolonged through years or decades, so that the disease may 
last 25 to 30 years, or even longer; sometimes death takes place much sooner — 
in from 5 to 10 years. It is difficult to prognosticate concerning this point. 1 

In regard to rapidity of development, there are marked deviations from 
this course; in the first place, some cases are so exceedingly slow that the 
patients never progress beyond the first stage of the typical symptoms, hence 
we might almost speak of an abortive form of tabes ; I have had under treat- 
ment for several years a patient, in whom Charcot in 1881 made a diagnosis 
of tabes, who presents all the classical symptoms of the first stage (spinal 
myosis, absence of the tendon reflexes, lancinating pains, laryngeal crises, pre- 
ceding syphilis), yet who even to-day is not ataxic; another patient with prior 
syphilis has had lancinating pains for 26 years, with transitory paralysis of the 
muscles of the eye and of the bladder, but even now the tendon and pupillary 
reflexes are retained, and show no sign of ataxia. Similar cases of appar- 
ently very benign and insidious types have been reported in literature. 

In contrast to these are the extreme cases in which the different symptoms 

i Pierre Marie has proven by statistics that about 83 per cent, of tabetics attain 
the age of 50 years, 51.5 per cent, over 60 years, and some 75 to 80 years. 



COURSE, TERMINATION, AND DEFINITE FORMS OF TABES 523 

of the affection appear in rapid sequence, the first stage lasting but a few weeks 
or months, being quickly, we might almost say in a night, followed by a high- 
grade ataxia; the patients soon become helpless; as a rule, there are extreme 
weakness and decided constitutional disturbance. These cases with rapid 
course are not rarely characterized by a profusion of symptoms, a combina- 
tion of rare phenomena, of the various crises, arthropathies, etc., as well as an 
aggravation of the ordinary symptoms. They may be aptly designated the 
malignant forms of tabes ; upon what this course depends we do not know. 

Besides these chief forms of the disease — the ordinary, the benign, and 
the malignant — other varieties of tabes are readily differentiated by the symp- 
tom-complex of the individual case. In my text-book (1878) I enumerated 
such types, and Dejerine and Collins have recently described quite a series. 
This is of practical value, because the type appears to have a definite influence 
upon the course and duration of the affection. We shall, therefore, briefly 
sketch a few of them. 

Types of Tabes. — First, there is a form in which from the onset lancinating 
pain forms the chief symptom of the affection (tabes dolorosa), the intensity 
often reaching an extreme degree, the painful attacks being very frequent, 
while the other symptoms are mild or wholly secondary ; but the opposite also 
occurs, and represents one of the most severe forms, for in this the patient 
almost invariably becomes addicted to morphin. 

Closely related to this form is another in which the sensory disturbances, 
such as disagreeable paresthesia and almost constant pain, become prominent 
(tabes paresthetica). Such patients often complain of a disagreeable girdle 
sensation, as though the trunk were enclosed in armor, there is difficulty in 
breathing, persistent and irksome sensations in the back; very often the trunk 
shows marked hyperesthesia to cold. In this form lancinating pains, gastric 
and laryngeal crises, and similar conditions are frequently noted ; the remain- 
ing symptoms vary. In this type it is a question whether the meningitic irri- 
tative symptoms, the " syphilosis " of the meninges, do not form the most 
prominent feature. In other cases motor disturbances are most evident, the 
sensory being less marked. Such patients are either decidedly ataxic or they 
soon present paretic and paralytic disturbances (tabes atactica or tabes para- 
lytica). 

An especially important form is that in which atrophy of the optic nerve 
appears as the first and earliest symptoms, and with it amaurosis (tabes amau- 
rotica). If the amaurosis is the earlier or prodromal symptom, the other symp- 
toms are remarkably mild; the sensory disturbances are usually slight, but 
they can be objectively demonstrated, the tendon reflexes are absent, pupillary 
rigidity exists, and — a remarkable fact — in spite of almost total blindness, 
sometimes Romberg's symptom is present ; such patients usually do not become 
ataxic, or only after a very long period of time; therefore the course of the 
disease is prolonged but mild. Benedikt, Charcot, Dejerine and Martin, Marie 
and Scwitalski, and others, have described and thoroughly discussed such cases, 
and I have seen several. What produces them cannot be positively stated, and 
I shall not enter upon hypotheses. If optic atrophy appears in the later course 
of tabes, it seems to have no influence upon the course of the affection. Bene- 
dikt maintains that, under these circumstances, the ataxia is less marked ; but, 
according to Marie and Leri, this is frequently not the case. 



524 TABES DORSALIS 

In other instances visceral symptoms, gastric and other crises, bladder 
symptoms, and the like (tabes visceralis) are more prominent; it is reported 
that these are frequently coincident with trophic disturbances, arthropathies, 
spontaneous fractures, etc., so that the extensive implication of the sympa- 
thetic and its spinal roots is suggested. 

Special significance attaches to a form of tabes which begins in the upper 
portions of the spinal cord, being localized in the cervical cord, not rarely 
somewhat higher, and implicating the bulbar and cerebral regions (tabes supe- 
rior, cervicalis, bulbaris) : the first symptoms are noted in the upper extrem- 
ities (lancinating pains, ulnar anesthesia, and ataxia of the hands), and to 
these laryngeal crises and posticus paralysis, bulbar symptoms, atrophy of the 
tongue, a difficulty in mastication, paresthesia of the face and of the neck, 
nuclear ocular paralysis, etc., are added; tabetic symptoms in the legs appear 
later, and are not so prominent. It becomes obvious that, with these compli- 
cations, the course is more or less unfavorable and more rapid. Another 
type, tabes infantilis or juvenilis, such as occurs in children from 5 to 10 
years of age, or in adolescents from 13 to 18 years of age, calls for a special 
description. In these cases there is almost always a history of hereditary 
syphilis, or of tabes and paresis among the brothers and sisters, or among the as- 
cendants. In most cases the classical symptoms of tabes appear, while sensory 
disturbances and ataxia are relatively less conspicuous. Unfortunately, there 
are as yet no autopsy reports of such cases, but a great number of them have 
been clinically diagnosticated. 

Finally, we must mention another important symptom of tabes which is 
associated with severe changes in the brain, as a rule with paresis, and to 
which we have previously referred (tab o paralysis) . That this complication, 
which is common in the early stages of tabes and rare in the later, influences 
the course unfavorably and greatly shortens life, is obvious. It is unnecessary 
to describe its symptom-picture. 

The number of these " types " may be easily increased ; in spite of all its 
peculiarities, to say nothing of the distinctiveness of its fundamental type, 
tabes is an extremely many-sided disease, richer in symptoms than any other, 
and I wish expressly to emphasize that the t}^pes here described are not con- 
clusive, but they form transitional stages and manifold gradations from one 
fundamental form; they are very different from those irregular forms of 
tabes which are transitional stages of the combined system diseases, or which 
are combined with other syphilitic diseases of the spinal cord and of the brain 
(with meningitis, meningomyelitis, meningoencephalitis, endarteritis, etc.). 
Their description would lead us too far from our subject. But it must be 
expressly stated that these varieties form a small minority in comparison with 
the regular, typical, fundamental forms of tabes. 

Except for the relatively few cases in which tabes is of rapid devel- 
opment, its course is extremely chronic, slow, but progressive; i. e., the disease 
shows a tendency to slow aggravation. But it must be borne in mind that to 
this there are numerous exceptions. Decided variations and more or less im- 
provement may appear, the condition of the patient may change with the 
seasons, improvement may lead to a prolonged arrest of the affection, or to 
a condition which is almost absolute recovery, so that the patients may be 
practically regarded as cured, being able to follow their occupations, to enjoy 



COURSE, TERMINATION, AND DEFINITE FORMS OF TABES 525 

life, and to fulfil its duties; such arrests may occur even in the ataxic stage, 
and the patient, who had been almost confined to a rolling chair, may find that 
his disease remains stationary for many years ; not infrequently, however, these 
arrests are disturbed in a most undesirable way by the effects of extraneous fac- 
tors (by exposure to cold, excitement, over-exertion, sexual excesses, by unsuit- 
able and exhausting therapeutic measures, such as hot baths, too prolonged use 
of the gymnastic method, and the like, often by influenza, occasionally by 
other intercurrent diseases), and the disease then rapidly progresses toward its 
terminal stages. 

The ordinary outcome of the affection is death. Not that tabes in itself 
is always fatal, for during its entire course the vital functions may be exempt. 
But it always shortens life by the sequels which it produces, by its reaction 
upon the entire organism, by many complications which are incidentally 
brought about. In some cases — often after a duration of 25 to 30 years — the 
disease is fatal because in the third stage it leads to paraplegia, to cystitis 
and pyelonephritis, or to bed-sores with all of their consequences, and thus 
the powers of the patient are exhausted, and, after a final stage of coma, 
delirium, acceleration of the pulse, respiratory disturbance, and great misery, 
the patient succumbs. 

Or the affection itself may cause death. The patients may suffocate from 
paralysis of the posticus, or from severe laryngeal crises; or they may suc- 
cumb to the consequences of fulminant gastric crises, either in collapse or from 
inanition; or the bulbar paralytic symptoms, difficulty in deglutition, or ina- 
nition, may have the same effect; or paralysis may rapidly bring about the 
catastrophe; all these are comparatively rare. 

Finally, an incidental and intercurrent disease — pneumonia, influenza, 
diphtheria, enteric fever, cholera, erysipelas, or pulmonary disease — frequently 
causes death; or syphilitic manifestations, cerebral syphilis, apoplexy, aneurysm, 
a lesion of the aortic valve, or the like, may terminate the life of the patient. 

But the termination is not always lethal. A few well developed cases of 
tabes (perhaps 4 to 6 per cent.) terminate in recovery, or, at least, the affec- 
tion is so far arrested, and the patients so far improve, that they are "prac- 
tically cured," they no longer have subjective difficulties, are able to perform 
their work, and to enjoy life in the ordinary way. If the patella tendon reflex 
is absent, or if the pupils are rigid, this but slightly affects the general condi- 
tion. I know of a number of such cases, of course not cases of tabes incom- 
pleta or tabes abortiva, which are soon to be discussed, in which a permanent 
arrest of the affection usually occurred spontaneously; hence we can hardly 
speak in these cases of " cure." 

Experience shows that in a certain proportion of the cases of tabes, unfor- 
tunately a very small one, actual or relative cure takes place, the duration of 
which may be limited. 

We have heretofore spoken of typical tabes which has a full symptom- 
picture, the most important subjective or objective symptoms being present 
in greater or less, but naturally very varying, numbers. Undoubtedly many 
cases belong to tabes, yet the symptom-picture is not completely developed, 
and may give rise to doubts. In a short article 1 I some time ago called 

1 " Zur Friihdiagnose der Tabes." Hiinchener med. Wochenschr., 1900, Nr. 29. 



526 TABES DORSALIS 

attention to such cases, and designated them as tabes incompleta (the French 
term these " formes frustes "). I have since seen many such cases, and other 
authors have called attention to them. 

These cases are invariably observed in persons who were previously syph- 
ilitic, in whom we note prominent subjective difficulties (lancinating pain, 
paresthesia, a sensation of fatigue, slight bladder disturbance, and the like), 
but we recognize no objective symptoms or these are indistinct (unilateral or 
bilateral pupillary rigidity, slight hyperesthesia to cold, hypesthetic areas on 
the trunk or the feet, indications of Romberg's sign, with retained patella 
tendon reflexes, perhaps unilateral absence of the tendo Achillis reflex and of 
the triceps reflex, etc.). 

In these cases, while all or nearly all subjective symptoms are absent, and 
the persons believe themselves to be quite well (possibly there may be ulnar 
paresthesia or a slight bladder weakness), nevertheless we find distinct objective 
symptoms of tabes (myosis with reflex rigidity, absence of the tendon reflexes, 
or the like). 

Finally, there are cases in which symptoms not in themselves character- 
istic, especially the onset of gastric crises, dyspepsia, salivation, intestinal 
disorders, cardiac neurosis, constitutional disturbances or the like, may exist, 
but examination reveals one or another of the objective symptoms of tabes 
(most often reflex rigidity of the pupil, paresis of an ocular muscle, unilateral 
decrease of one or the other tendon reflex, perhaps hyperesthesia to cold). 

These combinations of the individual subjective, and individual objective, 
symptoms of tabes are very numerous, much more so than was formerly sup- 
posed ; the habit of investigating all cases which are at all suspicious, of exam- 
ining the pupils and the reflexes, of seeking for Romberg's sign, and preced- 
ing syphilis, has shown them to be common. 

I do not doubt that these cases all belong to tabes, and that when present 
in this form they, must be grouped as tabes incompleta; moreover, in the 
last few years, I have often traced their development into tabes completa. 
In individuals who have previously been syphilitic, we will, as a rule, not be 
wrong in this assumption. Early in the discussion of the diagnosis we will 
return to this point, and I shall especially emphasize the value of cytodiagnosis 
in judging such cases. 

There is, however, a group of cases which present even fewer of the symp- 
toms of tabes, perhaps only one or two, and under subsequent and prolonged 
observation — 10 to 20 years — nothing further is noted. These are patients 
who have had syphilis, but upon examination they show nothing but reflex 
pupillary rigidity ; they have perhaps suffered for years from typical lancinating 
pains without any objective symptoms, or they now and then have a suspicious 
gastric attack, but during this time there is no tendo Achillis reflex, and 
nothing further develops. Following Mobius these cases may be regarded as 
abortive tabes, and, if we choose, we may await their subsequent development. 
We are certainly justified in considering these cases to be tabes which has 
become stationary, or has run its course, or, better, to be " syphilosis of the 
meninges." 

But all these observations teach us how extraordinarily the pathologic pic- 
ture of tabes may vary — from the mildest indications to the severest forms 
rich in symptoms — and how extremely full and varying is the symptomatology. 



DIAGNOSIS 527 

They warn us that in tabes, as in so many other diseases of the nervous sys- 
tem, especially in the toxic, infectious, and hereditary forms, Ave should not 
be too schematic, but should maintain a free and impartial attitude in con- 
sidering the nature and appearance of the affection. We may state with some 
satisfaction that here, under all circumstances, carefid clinical observation will 
yield most abundant fruit. 

VII. DIAGNOSIS 

In the present state of our knowledge and diagnostic advancement, ordi- 
nary typical tabes, even though in the first stages, is not difficult to recognize. 
If the affection has advanced further, and most of the subjective and objective 
phenomena are present, non-recognition of the disease can occur only in conse- 
quence of gross ignorance on the part of the physician, and a very defective 
examination. That this even now not infrequently occurs — every year I see 
cases which for some unaccountable reason have not been correctly diagnos- 
ticated — is a fact which mortifies me. 

The not uncommon cases in which the affection sets in with some rare 
symptom, such as a gastric crisis, arthropathy, or weakness of the bladder, 
are, as a rule, easily recognized on careful investigation if the physician takes 
tabes at all into consideration. 

The typical form of tabes should at once be diagnosticated by every physi- 
cian. Among its almost pathognomonic symptoms the most conspicuous are 
the lancinating pains, the reflex rigidity of the pupils, the absence of tendon 
reflexes, Romberg's sign, the initial atrophy of the optic nerve, and the ataxia ; 
less typical yet quite characteristic are the paresthesia, the girdle sensation, 
the local circumscribed hypesthesia, the analgesia, the retarded pain conduc- 
tion, hyperesthesia to cold, bladder and sexual weakness, various paralyses of 
the ocular muscles, gastric crises, and arthropathies. 

The diagnosis of tabes may be considered positive whenever we note merely 
a few, perhaps only one or two, of these symptoms (for example, lancinating 
pain, sensations of fatigue, reflex pupillary rigidity; or lancinating pain and 
the absence of the tendon reflexes ; or weakness of the bladder, reflex pupillary 
rigidity, or girdle hypesthesia ; or arthropathy, absence of the tendon reflexes, 
pupillary rigidity, etc.). 

Naturally in a few cases the diagnosis may be perplexing in the first stages 
of the affection, or when it irregularly develops as in tabes incompleta, or is 
combined with other disturbances, partly organic, partly functional; in these 
cases, the diagnosis may for some time be doubtful or even impossible. If, 
however, these conditions are kept in mind, and the development is traced with 
care, as a rule a decision soon becomes possible by the increasing distinctness 
of the symptoms, "or the appearance of new ones. But this is merely what 
takes place in all diseases, and requires no further explanation. 

But the recognition of tabes in its first stages, its so-called early diagnosis, 
appears to me to be of the greatest practical importance. This alone permits 
the favorable influence upon the disease of treatment, an effect which in fully 
developed tabetic degeneration is almost impossible. For this reason neurolo- 
gists have in the last few years devoted their attention to this finer and early 
diagnosis of tabes. It depends chiefly upon ascertaining the subjective diffi- 



528 TABES DORSALIS 

culties by a refined analysis, upon demonstrating that they indicate a beginning 
tabes, upon an exhaustive and most minute study of the different objective 
phenomena — although these may be only rudimentary — and such methods fre- 
quently lead to success. 

Of the subjective symptoms, the typical, lancinating pains are usually the 
most suggestive, even though these may be transitory, and may appear only 
as isolated sudden stitches. All forms of paresthesia are to be noted, espe- 
cially girdle sensation, localized paresthesia, hyperesthesia in the mammary 
and cardiac regions, ulnar sensation, paresthesia in the perineum, the anus, 
and the genitalia, the slightest bladder disturbances, decrease of sexual power, 
transitory diplopia, sudden dilatation of the pupils, etc., and the " rudimentary 
crises " which have lately been particularly described by Determann, and are 
as follows : gastric acidity, salivation, tickling sensation in the throat, a sense 
of burning in the esophagus, nausea, retching, anomalous fecal evacuation, 
attacks of cardiac palpitation, tachycardia, cardiac asthenia, marked emacia- 
tion, a sallow complexion, etc.; if these occur without a recognizable under- 
lying cause, and recur with a certain persistency, they are especially significant. 

Naturally the objective symptoms are more important, and chief among 
these are changes in the pupils: beginning sluggishness of reaction, although 
of one eye only, distinct pupillary rigidity either unilateral or bilateral, inequal- 
ity of the pupils, and unilateral mydriasis and paresis of accommodation. 

As long as the tendon reflexes are retained, slight differences between the 
two sides are of importance. We should always test the tendo A chillis reflex, 
for its unilateral or bilateral absence often long precedes the absence of the 
patella tendon reflex, and forms one of the most constant early symptoms of 
tabes. The triceps reflex should also be tested, although its absence is of less 
consequence; here differences between the two sides are often of great signifi- 
cance. Of course we must bear in mind the fact that the tendon reflexes may be 
absent in other diseases (paresis, atrophy, neuritis, and sciatica),, and these 
must always be excluded. 

Finally, a careful and early investigation of sensation leads us to diagnostic 
conclusions. Here the demonstration of circumscribed hyperesthesia in the 
trunk (mammary region, perineal region, lateral abdominal region) and in 
the extremities (ulnar region, soles of the feet, the toes, peroneal region, etc.), 
of analgesia and retardation of pain conduction in the leg, of hyperesthesia 
to cold upon the trunk, aids in the diagnosis; finally Romberg's sign, that 
often appears early. Rarely can ataxia be demonstrated in the early stages 
of the disease, and only by the finer tests. The accurate investigation of the 
eyes for double vision, and of the eye-ground for beginning atrophy of the 
optic nerve, is sometimes of use. 

If, moreover, we are dealing in such cases with individuals who are par- 
ticularly "susceptible to tabes" (as Mobius correctly expresses it), with 
middle-aged men in whom deleterious factors can be demonstrated, especially 
a preceding syphilis, the importance of these signs is decidedly enhanced. 

We have recently acquired an exceedingly important diagnostic aid in 
Widal's cytodiagnosis of the cerebrospinal fluid, which deserves the most com- 
prehensive investigation. Marked lymphocytosis appears to occur even in the 
earliest stages of tabes. Milian (1904) found it to be absent only in the cases 
of " tabes f ruste " ; in all other forms it was more or less marked. 



DIAGNOSIS 529 

My own cases, which have been quite numerous, confirm this. When, 
therefore, in doubtful cases we exclude those affections in which lymphocytosis 
also is present (tuberculous meningitis, herpes zoster, and paresis) — and this 
is usually easy — the proof of lymphocytosis is of great, even decisive, impor- 
tance. 

In illustration I quote the history of two cases recently under my obser- 
vation : 

(1) A land-owner, aged 35, had syphilis in 1889, and was under thorough treatment 
by Hg; in 1897 he had paresis of the muscles of the eye, dimness of vision in the left 
eye, girdle sensation, and neurasthenia. In May, 1898, objectively, general absence of 
tendon reflexes was noted, and was said to have existed for some time ; the pupils were 
normal. He took a thorough inunction treatment in Wiesbaden, and was greatly debili- 
tated. Objectively nothing was to be found; there was gradual improvement. In 1901 
he took part in military exercises; objectively everything was normal, even the pupils; 
all tendon reflexes were absent. In February, 1904, there was again for a few weeks a 
disturbance of the eye. Right pupil larger than the left, left reflex showing rigidity, the 
right still reacting; on the right side micropsia — otherwise, no signs of tabes. March, 
1904: Lumbar puncture revealed marked lymphocytosis. Diagnosis of tabes decidedly 
more positive. Hg treatment. 

(2) A manufacturer, aged 35, syphilitic infection 12 years ago. For 3 years he lfad 
moderate but characteristic lancinating pain in the legs, with hyperesthesia both in the 
legs and arms; there was somewhat decreased sexual power, but no other symptom. 
Objectively there was neither ataxia nor Romberg's sign; all the tendon reflexes were 
retained and active (the left patella reflex was perhaps weaker than the right), and 
the pupils reacted promptly; there was distinct slowing of the pain conduction without 
hypalgesia, also mild hyperesthesia to cold in the trunk, and testicular analgesia, but 
no local hypesthesia. Lumbar puncture: High-graded lymphocytosis ; therefore unques- 
tionably tabes incipiens! 

It is to be hoped that this means will further advance our knowledge. At 
all events, with our present refinement of diagnosis minute investigation will 
enable us to recognize tabes in its early stages, and incomplete and abortive 
forms will be more clearly and correctly differentiated. 

As has been maintained by Xageotte and others, cy to diagnosis of the cere- 
brospinal fluid opens up a further perspective, by permitting us to recog- 
nize cases in which tabes is threatened but is not yet present. If " syphilosis 
of the meninges " forms a prerequisite for the development of tabes, and if 
this, as can scarcely be doubted, is revealed by cytodiagnosis, a diagnostic lum- 
bar puncture should from time to time, perhaps every few years, be made in 
syphilitics, especially if they present any nervous symptoms; if lymphocy- 
tosis be positively found we should institute an energetic specific treatment 
to avert the threatening danger. 

Differential Diagnosis. — We must now briefly consider the differential 
diagnosis of tabes from other nervous diseases, especially the spinal forms. 

The differentiation of tabes from functional neuroses, from neurasthenia 
and hypochondriasis, in those patients who come to the physician with all 
sorts of subjective difficulties and consequent anxieties (especially in physi- 
cians, and in syphilitics who have heard of tabes), the diagnosis of the affec- 
tion merely from the subjective symptoms and with the absence of all objective 
phenomena (in the pupils, in the reflexes, in sensation, etc.) is, as a rule, easy. 
In isolated cases we must be very guarded in our opinion, and await the fur- 
ther development of the affection. The absence of syphilis here plays an 
35 



530 TABES DORSALIS 

important role; but in all persons previously infected we should be cautious 
in expressing an opinion. 

The disease may be readily distinguished from hysteria, which occasionally 
also presents " tabetic symptoms " (pains, anesthesia, bladder disturbance, 
ataxia, astasia-abasia, etc.) ; minute investigation and the objective considera- 
tion of the disturbances will reveal the true nature of the affection; one or 
two objective symptoms (rigidity of the pupils, absence of the tendon reflexes, 
etc. ) may render the decision easy and positive ; but we must remember that 
tabes and hysteria may occur simultaneously. Close analysis of the symptoms 
appertaining to these affections will dispel all doubt — at least for the expert 
neurologist. 

Among chronic diseases of the spinal cord we must primarily consider 
the purely motor system diseases (spastic spinal paralysis, amyotrophic lateral 
sclerosis, spinal amyotrophies, etc.) ; they form a diametric contrast to tabes 
by the absence of sensory and bladder disturbances and changes in the pupils, 
by the occurrence of paresis, by muscular tension and muscular contractures, 
increased tendon reflexes, Babinski's sign, and typical atrophies, so that con- 
fusion becomes possible only because of great ignorance. 

Following these syphilitic spastic spinal paralyses, transverse myelitis, 
tumors of the spinal cord and of the meninges, and gradual compression of 
these membranes, must be considered. Here, too, we usually note pareses and 
paralyses, spastic symptoms, increased tendon reflexes, Babinski's sign, etc., 
so that in spite of the presence of pain, and of sensory and bladder disturb- 
ances, the decision, as a rule, presents no difficulty. Intricate cases some- 
times form exceptions, but with close attention (normal pupils, no ataxia, no 
typical lancinating pains, sharp upper limitation of disturbance) the expert 
cannot long be in doubt. 

This is more likely to occur in multiple sclerosis which, if the foci are 
chiefly localized in the posterior columns, may at first produce tabetic symp- 
toms; the intention tremor may also resemble ataxia, or actual ataxia may 
occur. But multiple sclerosis presents a more complicated symptom-picture 
than tabes, and the diagnosis will chiefly depend upon the presence of cephalic 
symptoms (vertigo, headache, nystagmus, scanning speech, intention tremor, 
etc.), and upon the almost invariable increase of some of the tendon reflexes, 
upon paralysis, contractures, and the like. The absence of syphilis from the 
history may be utilized, as well as the age and sex of the patient ; nevertheless 
in some rare cases a diagnosis is only possible after prolonged observation 
of the case. 

Syringomyelia in its typical and ordinary form and localization (in the 
cervical cord with amyotrophy and absence of the tendon reflexes of the upper 
extremities, with dissociated sensory paralysis, with trophic disturbance, 
mutilations, with spastic symptoms and increased tendon reflexes in the lower 
extremities) will rarely cause diagnostic perplexity; but this may readily be 
the case when the affection has an unusual localization in the lumbo-dorsal 
portion of the spinal cord; here the absence of lancinating pains, of pupillary 
symptoms, of a specific etiology, etc., will lead to a decision. 

Among spinal diseases we must mention Tuczek's ergotin tabes which in 
fact bears a strong resemblance to true tabes. Its epidemic appearance, 
its etiology, its rapid development, the absence of pupillary rigidity, the 



DIAGNOSIS 531 

accompanying psychical and convulsive disturbances, sufficiently charac- 
terize it. 

In combined system diseases of the spinal cord (ataxic paraplegia, etc.) 
the differentiation will usually be easy, provided spastic symptoms and disease 
of the lateral columns dominate the clinical picture; it may be difficult or 
impossible when disease of the posterior column is the most prominent feature. 
Combined system disease has not infrequently been found in undoubted cases 
of long-standing tabes (lately by Kattwinkel). 

Friedreich's disease, hereditary ataxia, which was once regarded as a form 
of ordinary tabes, but which must unquestionably be differentiated from it, 
is now usually considered a combined s} r stem disease; it differs from tabes 
(also the infantile form) by its appearance in families even in childhood, by 
the absence of pain, of sensory and bladder disturbances, and of pupillary 
rigidity ; by the peculiar nature of the ataxia, its early appearance in the upper 
extremities, in the eyes, and in the organs of speech, and by the absence of 
syphilis. " Heredo-ataxie cerebelleuse" closely related to it, and first described 
by P. Marie, is differentiated by the activity of the tendon reflexes, by a pecul- 
iar club-foot, etc. 

Diseases of the cerebellum (tumors, atrophy, etc.), in which the "cere- 
bellar ataxia " produced resembles tabes, is usually differentiated by the course, 
by the form of the ataxia (swaying and staggering), by cerebellar symptoms 
(vertigo, vomiting, and choked disc), by the absence of all typical tabetic 
symptoms (rigid pupils, WestphaPs and also Eomberg's sign, lancinating 
pains, and typical sensory disturbances) ; in fact it so completely differs from 
tabes that it scarcely needs mention. 

The differentiation from chronic spinal meningitis, syphilitic root neuritis, 
and the like, rarely causes difficulty; true symptoms of meningitis and the 
absence of typical tabetic phenomena may make this possible, but these forms 
of disease may be combined with tabes. 

A number of affections remain to be considered, to which, because they 
produce some tabetic symptoms — such as paresthesia, anesthesia, pain, absence 
of the tendon reflexes, and especially ataxia — the inappropriate name of 
"pseudo-tabes" has been attached: to this category belong certain cases of 
multiple neuritis (peripheral tabes), especially in alcoholics (pseudo-tabes 
alcoholica) ; of sciatica with absence of the tendo Achillis reflex; of post- 
diphtheritic paralysis (with atrophic paresis, anesthesia, absence of the tendon 
reflexes, ataxia, ocular symptoms, etc.), perhaps also some cases of leprosy 
and toxic neuritis (from arsenic, nicotin, carbon bisulphid, etc.), and of dia- 
betes mellitus combined with acute pain in the legs (but usually due to sci- 
atica) and absence of the patella reflex. Although some of these cases may 
have an important bearing on the theory of ataxia, yet, as a rule, close inves- 
tigation shows that they have nothing in common with true tabes, but may 
occasionally be combined with it (for example, tabes in alcoholism, tabes in 
diphtheria, or tabes in diabetes, etc.). Cases of this nature which actually 
resemble tabes are exceedingly rare; in my long experience I have seen but 
few. They are to be differentiated, as a rule, by demonstrating the etiologic 
factors, by the presence of atrophic paralysis and simple non-tabetic sensory 
disturbances, by the absence of pupillary rigidity, and bladder disturbance, etc. 

That diseases of the brain can never be confounded with tabes is self-evi- 



532 TABES DORSALIS 

dent. We therefore briefly refer to the relatively frequent combination of 
tabes with progressive paralysis, because the early recognition of this condi- 
tion is often of great practical importance. The demonstration of tabetic 
symptoms in the picture of an already existing paresis is easy, but the recog- 
nition of signs of beginning paresis in tabes is not always so simple; here, 
occasionally, slight rudimentary symptoms of paresis appear which are not 
followed by the severe pathologic picture of progressive paralysis. Paresis 
can always be differentiated by the familiar early symptoms, such as a change 
in temperament, mild psychical debility, inequality of the pupils, tremor of 
the lips and tongue, slight disturbance in speech, etc. 

VIII. PROGNOSIS 

Half a century has passed since Romberg remarked, " For none of these 
patients is there hope of recovery ; all are condemned to death." In our knowl- 
edge of the disease, its nature, and its causes, we have advanced greatly. Many 
new therapeutic aids and processes have been tested, but the verdict quoted 
above has never been reversed, the prognosis of tabes has not become much 
more favorable. 

Naturally, this applies only to the cases of advanced tabes with well marked 
ataxia and typical symptom-complex. We know to-day that under any cir- 
cumstances tabes is a serious and usually progressive disease; that, however, 
the prognosis is decidedly more favorable in those initial cases of tabes in 
which the affection is recognized at the onset, cases of slowly progressive or 
mild tabes, of incomplete and abortive tabes, and we now have at command 
a certain number of therapeutic agents, although we cannot always state that 
they are valuable. 

At an interesting session of the Societe de Neurologie of Paris (Rev. 
Neurol., 1902, No. 1), the view promulgated by Brissaud that tabes has as- 
sumed a milder form, develops more slowly, and is frequently arrested, was 
thoroughly discussed. Brissaud leaves it uncertain whether this is the result 
of energetic treatment with mercury or of an attenuation of the syphilitic virus. 
P. Marie, Raymond, and Babinski agree with Brissaud, and attribute the 
benefit to specific treatment, while Joffroy doubts this effect, and Gilb. Ballet 
believes the subject not yet ripe for discussion; it is probably true that fur- 
ther investigation is necessary. 

Our general description of the disease and of its course indicates the prog- 
nosis : tabes, as a rule, is incurable ; only in a small proportion of the cases 
may recovery, or an arrest which amounts to recovery, be expected. Most 
cases run a slow course, many are extremely mild and benign ; in these, there- 
fore, the prognosis in regard to life and ability to follow an occupation is 
favorable, and many of these patients carry on their work for years ; in others, 
on account of the rapidity or malignancy of the disease, the many severe 
symptoms, the definite complications, the prognosis is much more grave. 

But at the onset of the affection, when the patient has been but a short 
time under observation, it is often difficult to give a prognosis that is at all 
reliable. Sometimes insidious and mild cases suddenly take a rapid and 
rather malignant course; in some cases which begin rapidly, and quickly lead 
to ataxia, improvement is noted; the disease is arrested for a long time, or 



PROGNOSIS 533 

its progress is extremely slow. But this foretells little concerning the indi- 
vidual case. 

It ma} r be stated that the prognosis is influenced favorably by the follow- 
ing factors : a very slow development, moderate intensity of the symptoms, 
the absence of severe trophic disturbances and of visceral crises, and a slight 
systemic implication; by a previously robust constitution, by favorable exter- 
nal circumstances, by the benign nature of the etiologic factors, by the remote- 
ness of the syphilitic infection, by the somewhat advanced age of the 
patient, etc. 

On the other hand, it is unfavorably affected by a marked neuropathic 
predisposition, rapid progress of the affection, early ataxia, a combination of 
grave symptoms ( such as severe pain, gastric and laryngeal crises, arthropathy, 
cystitis, and other bladder disturbances, etc.), an improper mode of life, ineffi- 
cacy of therapeutic measures, etc. 

In regard to individual symptoms, it must be stated that even the early 
recognition of the affection does not always avail, because the disease is of 
substantive character, and is not always amenable to treatment and a regu- 
lated mode of life. 

The cases with slow progress and but few symptoms, especially if these 
have existed for a long time, are comparatively hopeful. 

The forms designated as tabes dolorosa are prognostically very grave, both 
as regards this symptom and the course of the disease. The same is true of 
cases with gastric, intestinal, laryngeal, and bladder crises; these are not infre- 
quently combined in the same patient, and severely damage the entire con- 
stitution, thus profoundly affecting the course. Cases in which arthropathy 
and spontaneous fractures appear belong to the same category. 

Early and extreme ataxia is prognostically unfavorable, but we have learned 
to modify this symptom by appropriate treatment. Ataxia which comes on 
rapidly and acutely is almost always serious, although it may rapidly and 
wholly disappear. 

Cases of tabes cervicalis and tabes bulbaris give little reason for hope, 
because the implication of the cranial nerves (difficulty in deglutition, etc.) 
considerably shortens life. 

While tabetic amaurosis in itself is of exceedingly grave import, never- 
theless it considerably modifies the subsequent course of the affection, which 
usually proves to be mild. A patient whom I treated in 18TG for tabetic 
amaurosis remained free from ataxia for decades, and is still living to-day. 

The cases of tabes incomplete/, and of abortive tabes are comparatively 
benign. As a rule, they remain stationary or progress very slowly, but to this 
there are, of course, exceptions. 

Severe complications (such as syphilis of the brain, apoplexy, cystitis, 
pyelonephritis, contracted kidney, aneurysm, valvular lesions, myocarditis, 
etc.) manifestly render the outlook unfavorable. 

Tabo-paralysis is absolutely hopeless; it usually causes death in a few 
years. 

Altogether, the prognosis of tabes is very uncertain, and it must be cau- 
tiously made. Only after prolonged observation can we attain any degree of 
certainty; but the patient should always be encouraged to hope for an arrest 
or improvement, and in any case we should refrain from expressing an unfa- 



534 TABES DORSALIS 

vorable prognosis, for most astonishing improvement sometimes takes place 
in apparently hopeless cases. 

IX. TREATMENT 

It is self-evident that a disease which is relatively so common, appearing 
among all classes of people, and, as a rule, threatening to interfere with the 
earning of a livelihood during the most active period of life — a chronic affec- 
tion of the central nervous system, such as tabes, which lasts many decades — 
must from the onset have awakened intense professional interest, and inspired 
many therapeutic endeavors. 

But the difficulties presented in the treatment, and, still more, in estimat- 
ing the results of treatment, are almost incalculable; the tenacity with which 
this disease often resists all therapeutic measures, its slow development, the 
variations and peculiarities in its course, the occurrence of incomplete, mild, 
benign, or stationary forms, the appearance of unexpected remissions and 
exacerbations, all render it extremely difficult correctly to appreciate the re- 
sults of treatment ; our imperfect understanding of its actual nature and cause 
prevents a recognition of the precise indications; and although our knowl- 
edge of the etiology of tabes is steadily increasing, nevertheless its pathologico- 
anatomical foundations are still subject to change. Even recently they have 
again been constructed upon a new basis, yet they lack clearness and cer- 
tainty. Comprehensive clinical investigation and innumerable therapeutic 
efforts have failed to bring about decided amelioration, to establish positive 
methods of treatment, and thereby brighten the prospects of the unfortunate 
tabetic. 

Nevertheless a relatively favorable course of the disease, continued activ- 
ity, and a tolerable existence are often observed — apparently owing to thera- 
peutic measures; but this effect is uncertain, and only in a minority of the 
cases are we able favorably to influence the course, to arrest the disease, or 
to bring about improvement. 

And if we bear in mind the changes produced in the nervous system as 
soon as tabes has manifested itself, the length of time the process usually re- 
mains latent, the number of nervous structures which degenerate beyond recov- 
ery or have been destroyed, the slight hope of restoration, from the onset there 
is little hope of success, and we will entertain no sanguine expectations ; only 
too frequently these end in disappointment. 

This is evident at the beginning ; nevertheless, notwithstanding our unfor- 
tunate ignorance of the nature, cause, and course of the affection, as well as 
our inability to effect a cure, we should never desist from trying to relieve 
the patient, and should utilize the results of these efforts. Our knowledge 
of the etiology of tabes, which is definite and conclusive, to a great extent 
indicates the treatment. 

We will, therefore, briefly consider the individual indications which result 
therefrom. 1 

Undoubtedly we may refer to the prophylaxis of tabes; since we know to a 

i For a comprehensive description I refer to my article ; " Pie Therapie der Tabes," 



1 For a comprehensive description I refer 
Volkmmn's Sammlung, N. F. ? Nr. 150 ; 1896, 



TREATMENT 535 

great extent its cause, it is quite possible but exceedingly difficult, to prevent 
the disease, yet the attainment of this object is most desirable, for when the 
disease is once present, it is hard to cure it or to arrest it, even in its first 
stages. 

Tabes is probably due, in the main, to the combined action of a number 
of deleterious factors, the most important and constant of which is syphilis. 
Tabetics are almost exclusively persons who have previously been syphilitic, 
but even in these there may be other predisposing causes, such as a neuro- 
pathic predisposition, congenital or acquired weakness of the nervous system, 
refrigeration, bodily over-exertion, sexual excesses, trauma, abuse of alcohol 
and tobacco, excessive mental exertion, excitement and emotion, etc. This 
shows what the prophylaxis of tabes should embrace. 

First of all, the prophylaxis of syphilis should be attempted. With the 
extinction of this disease, tabes also would in great measure disappear. This 
is not the place for a minute discussion of this point. In spite of all the 
measures recently brought before the public for the study and prevention of 
sexual diseases, there is practically but little hope of success in this direction, 
and we shall long have to reckon with the fact of the prevalence of syphilis, 
especially among the population of a large city, and among those who visit it. 

Therefore, the principal condition for the prophylaxis of syphilis is the 
timely, thorough, and sufficiently long-continued treatment of syphilis in its 
first stage. Experience has proven that even the most vigorous and most 
careful treatment of syphilis is not certain to prevent the subsequent appear- 
ance of tabes. But the researches of syphilographers in regard to the influ- 
ence of specific treatment by Hg in the earlier stages of syphilis and for the 
later tertiary forms, among which we may mention the voluminous statistics 
of Fournier, Xeisser, and others, unanimously and convincingly prove that 
the energetic treatment of syphilis in the first stages decidedly decreases the 
probability of the later appearance of tabes. At the same time, it proves the 
absurdity of the foolish statement, so often made, that mercurial treatment 
favors the subsequent appearance of tabes, or even directly causes it. 

How this treatment is to be conducted, how often repeated, and how 
long continued, need not here be discussed. Unfortunately in practice many 
of our methods are wrong. Treatment is often too lightly regarded by physi- 
cians, the patients are not sufficiently instructed in regard to the dangers 
of neglect of treatment, they are not impressed with the serious nature of 
their disease, and this levity on the part of the patient often baffles descrip- 
tion. It is unnecessary to make our patients syphilophobes and hypochondriacs, 
but in many of them explicit instruction within certain limits will not fail 
of its purpose; Stintzing (Handbuch der Therapie innerer Krankheiten von 
Penzoldt und Stintzing, V, 3. Aufl.) has given us some valuable advice in 
regard to this. 

But prophylaxis is not exhausted with the treatment of syphilis. Since, 
in syphilitics, various incidental causes may immediately produce tabes, we 
must issue a warning against these deleterious factors. Of course, when we 
consider the rashness of those who do not regard themselves as sick, we cannot 
hope to attain much ; but with some persons such a warning will bear fruit ; 
hence I have long considered it my duty to tell syphilitics expressly that their 
disease has so weakened them that they react more strongly to all deleterious 



536 TABES DORSALIS 

factors than do normal persons, and that they cannot expose themselves to these 
influences without danger, as others may do. This warning is especially needed 
by those who have a " hereditary or acquired " neuropathic tendency, those 
who perhaps exhibit all sorts of nervous symptoms (neurasthenia, etc.) They 
should guard against refrigerations, especially repeated ones, and over- exertion 
and fatigue — for example, in hunting, fishing, mountain climbing, rowing, 
bicycling, etc. They are to avoid sexual excesses, and the abuse of tobacco and 
alcohol, are to be exceedingly careful for a long time after trauma, carefully 
to avoid immoderate work, mental strain, emotion, and great excitement, and 
especially the combination of these harmful factors; after influenza or other 
serious infectious diseases, they should allow themselves a sufficient time for 
convalescence, and should not resort to immoderately hot baths, steam baths, 
nor the ridiculous use of cold ivater at extremely low temperature. 

Persons who are predisposed to nervous affections, and who have acquired 
syphilis, must guard against the development of tabes by a sensible mode of 
life, by tonics, and by air and water cures, etc. 

This about exhausts the prophylaxis of tabes, and we turn to the treat- 
ment of the causal indication. It is clear that any treatment qf tabes has a 
better prospect of success if begun early, therefore the early diagnosis of tabes 
is of paramount importance. 

In the causal treatment, antisyphilitic measures undoubtedly and almost 
exclusively first come into question, but their strict scientific applicability is 
at this time uncertain, and even impossible to prove. If tabes could be re- 
garded simply as a late manifestation, a special form, of tertiary syphilis, the 
foundation for this treatment would be clear ; and although many of the 
lesions and numerous tertiary gummatous manifestations in the meninges 
and vessels (especially the irritation of the meninges revealed by cytodiagno- 
sis) must undoubtedly be regarded as due to syphilis (" syphilosis "), and as 
"of extreme importance, this is not yet absolutely certain; the possibility that 
we are dealing with a specific toxic action, the effect of a syphilitic poison 
constantly furnished by old, latent, pathologic foci, and perhaps by abnor- 
mally increased antitoxins, immunization products and the like, necessitates 
a certain reserve, and the prospect of an effective syphilitic treatment becomes 
decidedly less. 

Notwithstanding these considerations, the majority of modern investigators 
have come to the conclusion that antisyphilitic treatment is indicated in tabes, 
and that this should be more or less energetic; some have made only brief 
trials, others have pushed iodin and mercury; many have obtained good re- 
sults, others only moderate, and with still others the results were chiefly nega- 
tive; among those who admit the connection between syphilis and tabes, some 
reject specific treatment absolutely, to say nothing of those who declare, what 
has been sufficiently disproven, that specific treatment is injurious to the tabetic. 

The justification of a specific treatment of tabes can be determined only 
by experience; and the difficulty of securing accurate reports has already been 
pointed out; this is increased by our newly acquired knowledge that some of 
the mild forms of tabes remain stationary, and that they follow a varying 
course which is difficult to determine beforehand; value can be attached only 
to extended series of observations, and to the impressions of experts. 

The experiences of numerous authors are at hand. They are almost united 



TREATMENT 537 

in the opinion that antisvphilitic treatment in tabes, especially if early re- 
sorted to, and with some necessary modifications, is undoubtedly beneficial in 
the majority of the cases, but in only a few is this benefit ver}^ decided. 

It would be a digression to detail reports from literature or personal expe- 
rience. I shall only state that in numerous cases in my own extensive practice 
I have often seen good results from a rational specific treatment several times 
repeated; that in a few cases these results amounted to complete recovery, 
for the patients were able to resume their occupations and to follow them for 
years (reflex pupillary rigidity and the absence of the tendon reflexes, etc., 
perhaps persisting) ; that in a series of cases the affection was often checked 
for years ; but I must add that in a large number of cases, especially those 
well advanced, no arrest of the disease could be brought about. 

At the same time my abundant experience has taught me that specific 
treatment — provided it is carried out rationally and carefully — is absolutely 
li armless to the tabetic, so that the loud warnings which have repeatedly been 
issued against this treatment are entirely uncalled for. It is self-evident that 
some individuals are susceptible to Hg or iodin, and do not bear this treat- 
ment well. But it is also true that most tabetics tolerate cautious and even 
energetic antisvphilitic treatment, and that it improves their general condition, 
and increases their weight. 

Therefore, considering that syphilis is the most common, most important, 
and, primarily, the chief cause of tabes, that the pathologico-anatomical 
changes in tabes certainly do not preclude the effect of specific treatment, 
that undoubtedly true, specific, gummatous diseases often introduce tabes, that 
lumbar puncture reveals an irritation of the meninges which is most likely 
syphilitic, that perhaps, somewhere in the body (glands, etc.) latent, luetic, 
pathologic foci continue to exist, the removal of which is beneficial, and, 
finally, that specific treatment is never injurious to the tabetic, it is evident 
that in tabes preceded by syphilis an antisyphilitic treatment is generally indi- 
cated. It is also certain that it should not be blindly resorted to, but that the 
indications in each case should be minutely considered, and the treatment in- 
stituted more or less powerfully, according to individual circumstances; some- 
times it yields no result whatever. 

As a rule, specific treatment is justifiable in the following conditions: 
1. In all recent cases of tales, especially if the syphilitic infection is not 
remote; 2. In all cases in which florid symptoms of syphilis are manifest 
somewhere in the body (in the skin, mucous membranes, bones, etc.), and 
when these are combined with symptoms of cerebral or meningeal syphilis, 
especially in those cases in which lumbar puncture reveals a typical lympho- 
cytosis; 3. In all cases, even long-standing ones, in which an ineffectual syph- 
ilitic treatment had been previously instituted, in which relapses had occurred, 
or in which there is marked lymphocytosis. 

Of course, when the disease is well advanced and the ataxia is prominent, 
when the cases have been associated with special conditions, for example, with 
recent relapses, with new tertiary symptoms, with decided lymphocytosis, we 
usually conclude to reinstitute specific treatment. Even if this is unsuccessful, 
we risk little, and I have repeatedly known astonishing benefit to follow. 

Specific treatment is contraindicated in very advanced and long-standing 
cases, in emaciated, cachectic, or dvspeptic patients, when the luetic infection 
3G 



538 TABES DORSALIS 

is remote, when specific treatment has been repeatedly unsuccessful, and Hg 
and KI have not been tolerated. 

The method of treatment must be chosen according to the fundamental 
principles by which the late forms of syphilis are treated ; primarily, we should 
consider Hg combined, or alternating, with ioclin treatment. Whether this 
exhausts the therapy of late syphilis is doubtful ; possibly the future may bring 
to light remedies more effective than these. I am under the impression that 
Hg and iodin are not so beneficial in these degenerative processes in the nerv- 
ous system as they are when secondary and tertiary manifestations are pres- 
ent. But advancing knowledge, or, perhaps, a fortunate accident, may fur- 
nish something better. 1 

We prefer the old, well tested inunction, treatment, but have no objection 
to other methods (internal administration and subcutaneous injections of sol- 
uble and insoluble preparations of Hg). It is our practice to institute a 
moderately active inunction treatment, and to repeat it at long intervals 
(30 to 50 inunctions, each of 4 to 6 grams, rarely more), observing all the 
precautions necessary to prevent stomatitis, including two lukewarm baths a 
week, plentiful nutrition, an outdoor life, the patient absenting himself from 
his business and occupation. The latter is most important, therefore we fre- 
quently combine this method with spa treatment (at Aix-la-Chapelle, Nau- 
heim, Wiesbaden, Baden-Baden, Eehme, Nenndorf, Baden in Switzerland, etc., 
but avoiding hot baths), or this treatment may be carried out in the hospital 
or sanatorium. I must issue an express warning against using this treat- 
ment while the patient follows his occupation or remains at his home. 

For years I have maintained that treatment with Hg first of all removes 
the underlying cause of tabes, and, so to speak, prepares the way for the 
action of other remedies — other measures (for instance, those usual in the 
treatment of meningitis) may be tried at the same time. But our chief 
reliance must be upon the former, hence the attention of the patient must be 
called to the fact that during mercurial treatment no marked improvement 
is to be expected; on the contrary, it may at first seem to aggravate the con- 
dition, the benefit appearing later. 

After treatment with mercury I have attempted to bring about improve- 
ment by a tonic process — a residence in the mountains, mild hydrotherapy, 
sitz baths, electric treatment, the administration of tonics (arsenic, cacodylin 
injections, glycero-phosphates, lecithin, etc.), or a spa treatment at Eehme or 
Nauheim, and I believe that I have thus secured excellent results. 

According to circumstances, mercurial treatment is to be repeated in from 
four to eight months, or even a year later, and thus an alternate specific and 
tonic treatment may be carried out for several years. The patients are usu- 
ally satisfied with the improvement which appears, and are willing to continue 
the treatment. 2 

i The combination of Hg with an organic preparation of arsenic, such as the so-called 
ennesol ( Salicylarsinate de Mercure, Coignet), promises to be a particularly active 
remedy for tabetic and similar syphilogenous degeneration. I have instituted some 
trials with it. 

2 Others have advised similar methods, but the principle is always the same — regu- 
lar treatment by mercury, continued for some time, with intermissions. 

Bockhart advises an annual treatment of 20 to 25 inunctions, each of 2.5-4.0 grms. 



TREATMENT 539 

In this form mercurial treatment is well borne by the patient, but whether 
it will effect a cure is another question. Some authors assert that the ineffi- 
cacy of the treatment which many physicians report is due to a want of energy 
and persistence in the administration of Hg. 

In France the institution of brisk and long-continued mercurial treatment 
in tabes and paresis has recently come into favor, many successes having been 
published. I feel very skeptical, for I have undoubtedly seen many ill effects 
from the " heroic treatment," which has been advised in Germany by 0. Ziems- 
sen (Wiesbaden). The result of these trials is still sub judice. 

Of course, in an Hg treatment, iodin therapy also comes into question. 
Undoubtedly it should be resorted to in many cases, and in some it is certainly 
beneficial. The administration of KI (or Xal) may be combined with that 
of Hg, or the drugs may be used alternately for from one to three months, 
1-4 grams or more daily, before or after a tonic. The use of iodin has been 
greatly facilitated by the introduction into treatment of iodipin, which is best 
given in the form of subcutaneous injections (10 and 25 per cent.), and is 
usually well borne. 

Of other antisyphilitic methods — Zittmann's treatment, root) laffectcur, 
diaphoresis, etc. — not much is to be said. Occasional trials of these have been 
unsatisfactory to me. 1 So much for the antisyphilitic treatment of tabes. 

Xow the question arises: How are the cases of tabes without preceding 
syphilis to be treated? Considering that some authors regard all tabes as 
due to syphilis, and the impossibility in many cases of positively excluding a 
syphilitic infection (because, from the nature of the case, the infection is 
not unlikely, at all events can neither be disproven nor demonstrated) it may 
be asked : Should, or may, these cases also be placed under antisyphilitic treat- 
ment? Some authors answer this question in the affirmative, and advise at 
least a trial with mercury and iodin; certainly this is very common in practice, 
and although we risk little with such an attempt yet I do not concur in it; 
every physician must, in the individual case, answer this question for himself; 
here cytodiagnosis may help us to a decision. If we believe that lymphocy- 
tosis of the lumbar fluid is positive evidence of the syphilitic nature of the 
infection, and if in beginning tabes without demonstrable syphilis we find a 
typical lymphocytosis, this is a very clear indication for syphilitic treatment. 

I shall briefly detail such a case which I recently observed: A man, aged 35, had 
suffered for 3 years from lancinating pains, ptosis, and bladder weakness, to which 
typical tabes indpiens, with all its objective symptoms (without ataxia) was added. 
He never had chancre or syphilis, but had one attack of gonorrhea. His children were 
healthy. When examined no signs of syphilis could be found (merely slight cicatrices 

with warm baths and complete rest. The method recently advised by 0. Rosenthal 
("Therapie der Syphilis," 1904) is worthy of notice. He reports a number of con- 
spicuous cures and of arrested cases under the following treatment: daily inunctions 
of 4 to 10 grams with 4 to 6 weeks' rest in bed (very important), also sweating, daily 
bathing, and injections of iodipin. The treatment is to be repeated after 3 months, then 
after an interval of 9 months. In the second year there are to be two treatments, and 
in each of the two following years one treatment. The diet is to be carefully regulated. 
but should always consist of roborant foods; no alcohol, no tobacco! 

i Whether, when demonstrable lymphocytosis indicates a beginning tabes, this should 
be treated with intradural injections (of preparations of iodin. mercury, or the like) I 
dare not decide, but the question certainly deserves consideration. 




540 TABES DORSALIS 

at the angle of the mouth which had no significance as the patient was an inveterate 
smoker). Lumbar puncture revealed high-graded lymphocytosis. I regarded this as a 
clear indication for treatment with Hg, which gave most excellent results. 

It will rarely be necessary to combat other causal agents than syphilis. 
The causes which the patient may mention are too remote, and are past coun- 
teracting; there remains, therefore, merely the question of removing exciting 
or permaent deleterious factors : the effects of cold, of over-exertion, of living 
or working in damp rooms, of sexual excesses, of stimulation, the abuse of 
alcohol and of tobacco, excitement, emotion, mental strain, of baths either 
too cold or too hot, etc. ; with the regulation of these much will be attained. 

For those who regard tabes as a late form of syphilis — i.e., an actual 
syphilitic disease — and if they desire to do justice to the indicatio causalis, 
the indicatio morbi coincides with the specific treatment just outlined. Self- 
evident as is this view to-day, nevertheless it has not been proven; specific 
treatment has its limits, and frequently yields only unsatisfactory results. 
There is still room for a non-specific treatment of tabes; at all events, the 
cases of tabes without a preceding syphilitic history should be directly treated, 
and in all cases the indicatio morbi should be fulfilled. Its purpose is to com- 
pensate for the degenerative processes going on in the posterior columns and 
other parts of the nervous system; at least to remove the functional disturb- 
ances caused by these. This is its most important object, although, in the 
strict sense of the word, a " pathologico-anatomical cure " is probably never 
attained; it is practically satisfactory to the patients if the disturbed func- 
tions are sufficiently compensated, and their complete activity restored. It is 
quite immaterial to them whether gray degeneration still remains or whether 
there are irrelevant symptoms which nowise disturb them — such as reflex pupil- 
lary rigidity, and the absence of the patella tendon reflex. 

The methods which nature provides to attain this goal vary. Nutritive 
disturbances in the nervous elements may be counteracted by an invigorated 
and improved metabolism, by the stimulation of the circulation of blood and 
of the lymph, perhaps also by the elimination of toxic products; but the 
slumbering powers of vicarious and collateral tracts may also be awakened, and 
may be utilized as substitutes for the lost functions. 

The general improvement of the nutrition, the stimulation of metabolism, 
as well as of the functions of the diseased nerve tracts, relief of the circulation 
iri these tracts, and kindred alterations may produce a change of chemism in 
the diseased nerve tracts; the stimulation and exercise of vicarious and com- 
pensatory functions will be supplementary. 

There are many methods for this purpose. Before enumerating and con- 
sidering these, a few introductory remarks are in order concerning the general 
dietetics and the mode of life of the tabetic. 

Generally speaking, tabetics must live a quiet, regular life, eliminating 
over-exertion, excitement, and excesses as far as possible. " Live as if you 
were an old man, simply, abstemiously, quietly, and regularly ! " is the advice 
that I give to all patients in the first stage of the affection ; later this is com- 
pulsory, but in the stage of onset it is profitable, although often difficult, to 
follow this direction. 

Therefore simplicity of life, with moderation in eating and drinking, in 
smoking, in social intercourse, in sexual and other pleasures, with proper alter- 



TREATMENT 541 

nations of work and rest, moderation in work, and sufficient recreation is 
important; simple pleasures and cheerful companionship are most desirable, 
but all excitement, emotion, and over-exertion are to be prohibited; an out- 
door life in the summer, 1 a residence near the woods or in a mountainous re- 
gion, and in the winter a warm climate, are to be recommended. Care should 
be taken to avoid excessive bodily exertion and over-fatigue; hence sports 
(hunting, mountain climbing, bicycling, and tennis) are to be prohibited or 
carefully regulated. In the first stage, the pre-ataxic, we may allow the gym- 
nastic exercises, especially in the room, rowing, moderate bicycling, and even 
horseback riding if not in excess. 

Naturally we must closely consider the individual conditions, and, espe- 
cially in recent and progressive cases, must be extremely cautious ; in the late 
or stationary stages, the patients should practise regular muscle exercises, and 
movements and gymnastics in the open air (see Frenkel's method). 

Edinger's theory of the development of tabes is of great importance, but 
very perplexing. Were it generally accepted we would advise for most tabetics, 
especially at the onset of the affection, in exacerbations, or in relapses, complete 
bodily rest or even rest in bed. But, of course, this cannot always be done; 
for a robust tabetic, who for years and decades may perhaps still be active, 
will not at the beginning of the disease submit to such a method. But for 
those forms in which ataxia progresses rapidly, with motor debilitative condi- 
tions and extreme emaciation, absolute rest is strongly to be recommended. 
I believe that the benefit of this method cannot be doubted, but in other 
forms of the disease it may do the patient harm by making him more flaccid 
and less energetic, by increasing the hypotonia of the muscles and the ataxia ; 
therefore in these it should not be continued. In regard to this it would be 
well to collect extensive reports, as Edinger did. Here, as everywhere, we must 
carefully individualize. 

Among the various remedies and curative methods, drugs must be first 
mentioned, but little can be said in their praise. There has been but slight 
advance or additional clearness of indications in the past 30 years, and no 
record of prompt and positive results. Nevertheless, in many cases their 
value cannot be doubted, and, with the endless duration of the disease, they 
certainly cannot be dispensed with. 

Silver nitrate still enjoys the highest reputation — its results in a certain 
number of cases are incontestable; it should be given for a long time, even 
months; or, with long intervals, may be continued for years (in pill form, 
3 to 5 centigrams daily, preferably with extract of nux vomica) until from 
10 to 12 grams of the remedy have been used. Inunctions of unguentum 
argent, colloid. Crede, need further trial (Determann). 

Ergot which was almost exclusively employed by Charcot (2 or 3 times 
daily for a few weeks, then resumed after an interval, etc.) has fallen into 
disuse; its affinity for the posterior columns (Tuczek's ergotin tabes!) makes 
its administration seem unwise, although its effect in small doses cannot be 
denied. I chiefly employ this drug (or, better, ergotin) in cases of extreme 
weakness of the bladder. 

i The treatment advised and elaborated by Determann combines hygienic, physical, 
and dietetic methods ; " treatment with rest in the open air " certainly merits con- 
sideration. 



542 TABES DORSALIS 

Even before syphilis was recognized as the main cause of tabes, potassium 
iodid was praised by many, particularly for the lancinating pain. Nowadays 
its effects do not seem to be remarkable. We have already referred to the 
use of KI, Nal, and iodipin. The sails of bromin have also been recom- 
mended, but I have never observed their directly curative effect in tabes. 
Arsenic also has been much praised; undoubtedly this is useful in various 
diseases, particularly those of the nervous system. I have used it often, appar- 
ently with very good effect; but I have never decided whether it acts simply 
as a tonic and blood-forming remedy, as a nervine, or whether it has a directly 
curative influence upon tabes; the first theory appears to be the most likely. 
I administer it in the usual forms, either as arsenious acid, as Fowler's solu- 
tion (generally in combination with tincture of nux vomica), as Levico water, 
or, lately, as cacodylate by subcutaneous injection. After abundant experi- 
ence I have formed a very favorable opinion of its tonic and alterative effects 
in the last mentioned form. 

The same may be said of strychnin which I have frequently used, and 
which undoubtedly has some effect in tabes, although this action is difficult to 
explain. Whether it directly affects the chemism of the spinal cord tracts, or 
is merely a general nervine, stomachic, or tonic, I shall not attempt to decide ; 
but it is certainly beneficial (the subcutaneous injection of 2 to 10 milligrams, 
or the administration of the various preparations of nux vomica, either tinc- 
ture or extract, and preferably combined with other remedies, such as arsenic, 
silver nitrate, wine of condurango, or other tonics). 

Among these I lay stress upon the preparations of iron, which, combined 
with arsenic, quinin, or strychnin, are undoubtedly valuable for their gen- 
eral tonic effect by which they restore the powers of the organism. I fre- 
quently employ with benefit my so-called tonic pill (see formula below 1 ), 
and alternately arsenic and the cacodylates. They form a very satisfactory 
after-treatment to Hg and iodin cures, and are well borne and highly praised 
by the patients. 

Of the numerous other remedies employed in tabes — the double chlorid 
of gold and sodium, quinin, antipyrin, phosphorus, and barium chlorid — 
nothing special can be stated ; experience alone will show whether the glycero- 
phosphates, the phosphorus-containing lecithin preparations, and phytin, which 
have lately been advised, give better results. 

The opotherapy of Brown- Sequard, treatment with organic extracts (of 
the brain and spinal cord, and of the testicles, also with spermin-Poehl, etc.), 
has found but few proselytes. I must, however, admit that I have seen a 
few cases in which injections of spermin apparently had a favorable and tonic 
effect; therefore this should not be entirely tabooed in the treatment of this 
tedious disease. 

Balneotherapy has a prominent place in the treatment of tabes. The views 



13 Ferr. lactic, j __ 4 Q5 Q 

Extr. chmae aqu. ) 

Extr. nuc. vom. spir 0.5-0.8 

Extr. gentian q- s. 

M. f. pil. No. 100. 
One or two three times daily after meals. 
The formula may be modified according to individual indications. 



TREATMENT 543 

in regard to this and its indications have changed but little in the last 25 
years. The exact action of baths, by means of their temperature and the salt 
and C0 2 they contain, is not quite clear, but there is no doubt of their effect 
upon the circulation and respiration, the skin, the centripetal nerve tracts, the 
general metabolism and nutrition, upon appetite and sleep, etc., although this 
is difficult to explain. To-day baths certainly belong to the therapeutic meas- 
ures which are indispensable in tabes. 

The thermal salt baths of Nauheim and Rehme-Oeynhausen, which are 
rich in C0 2 , are in the first rank. I have had abundant experience with these, 
and in many cases their beneficial effect is striking. But, when unsuitable, 
especially when forced, they may do harm, therefore we must exercise discre- 
tion in using them, and must strictly individualize. It is the duty of the spa 
physician, who fully understands the waters, to prescribe the exact temperature 
and duration of the bath, its amount of gas, and the kind of movement (douche 
baths, etc.) ; baths of medium or low temperature, containing a moderate 
amount of C0 2 and with a moderate motion of the water, appear to be the 
best adapted to all tabetics except those who suffer from severe attacks of 
pain, or who have areas upon the trunk which are hyperesthetic to cold, and 
whose nervous systems are very irritable. 

Those iron oaths which are rich in CO,, and some of which also contain 
salt (Schwalbach, Franzensbad, Tarasp-Schuls, St. Moritz, and others), have 
a similar effect and are resorted to for similar reasons, particularly when we 
desire to combine their tonic effect with mountain treatment. Peat baths have 
been sometimes praised, but the indications for their use are difficult to define, 
hence they are rarely ordered in tabes. 

Springs at varying temperatures ( Schlangenbad, Ragaz, Wildbad, Gas- 
tein, Teplitz, Wiesbaden, Baden-Baden, etc.) were formerly recommended; 
but baths at high temperature (above 33° C.) have proved rather injurious 
to the tabetic, and hold no important place in the therapy. In a few irritable 
and erethetic cases with great pain, hyperesthesia, and different forms of 
crises, etc., they appear to have been beneficial when cautiously given at a 
somewhat reduced temperature, when of brief duration, and not too frequently 
repeated. 

The same is true of sulphur springs (Aix-la-Chapelle, Nenndorf, Baden 
near Vienna, Baden in Aargau, etc.) ; they are highly advocated as auxiliary 
remedies during mercurial treatment, but we should be careful not to permit 
too high a temperature. 

The springs of La Malou, ordered exclusively in France, appear to possess 
no special advantages. 

Steam, air, and electric light baths can be used in tabes only with the 
greatest care, and are better omitted. 

On the contrary, the benefits from hydrotherapy are especially marked. 
The generally strengthening and refreshing effect of the water, its action 
upon the skin, the sensory nerves, the circulation, and the respiration, upon 
metabolism, upon the general nutrition, etc., sufficiently account for the benefit 
which tabetics experience under this treatment. I have had abundant experi- 
ence with hydrotherapy, and I consider it just as valuable as a treatment in 
Nauheim or Rehme; much depends upon the mode of application and its 
individual adaptation. All highly irritating procedures which cause a sudden 



544 TABES DORSALIS 

shock (very cold baths, douches, ablutions, or packs) are to be strictly avoided, 
as well as the Kneipp treatment; as a rule, the milder processes, carefully and 
gradually increased, such as tempered sitz baths (from 30° to 20° C), simple 
ablutions, tepid full baths, local spongings, as of the back, feet, etc., are pref- 
erable. Much depends upon the choice of an institution ; sanatoria in wooded 
or mountainous regions are the best, and the treatment should be by a special- 
ist. Under a suitable regime all tabetics bear this treatment well. Here and 
there a certain intolerance is noted in anemic or irritable persons who show 
a poor vasomotor reaction, hyperesthesia to cold, etc. 

In some cases river bathing is permissible in midsummer, while sea bath- 
ing, especially when the temperature of the water is low and the surf strong, 
must be resorted to very cautiously ; nevertheless, they are sometimes beneficial. 

Electrotherapy in tabes, as practised and advised by R. Remak, has under 
the weight of recent opinion and, it appears to me, unjust criticism, come 
under the ban. Naturally we do not share Remak's illusions; nevertheless, 
although electrotherapy in tabes has often been unproductive of good, in many 
cases it undoubtedly has led to improvement, amelioration, or even the arrest 
of the affection — in rare cases the results were quite remarkable. This is abso- 
lutely certain, and in the interest of tabetics I regret that the skepticism of 
most authors concerning the action of electricity has curtailed the use of this 
remedy. Since the influence of suggestion is so powerful, it might be well to 
treat the tabetic by hypnotic suggestion; but concerning this remedy no suc- 
cessful results have been published. 

Of course this treatment must be carried out scientifically by a physician 
who fully understands the conditions, and is specially versed in the method. 
But this principle is frequently overlooked; hence the many unsuccessful re- 
sults which have helped to displace electric treatment. Treatment by the 
patient himself, by his relatives, or by nurses should be absolutely forbidden. 
As a rule this prohibition amounts to nothing, and in extremely mild cases 
we may be obliged to permit persons who have been carefully instructed to 
carry it out. 

For details concerning the probable effects of electricity (stimulation of 
function, of the circulation, of nutrition, of the nerve elements, thereby 
checking degeneration), the strength of current to be employed, and the meth- 
ods of application, I must refer the reader to text-books on electrotherapy; 
here I shall briefly discuss the most important indications. 

The galvanic current is of prime importance. In a longitudinal (col- 
umnar) disease of the spinal cord, such as tabes, the longitudinal application 
of the current is most practicable. Both poles should be placed on the vertebral 
column, the large electrode (ascending or descending current or these alter- 
nately), the stabile current with the electrodes successively and directly cover- 
ing the different areas of the spinal cord; or the method, which I prefer, of 
simultaneous galvanization of the sympathetic (the cathode upon the cervical 
sympathetic, the anode at different points along the thoracic and lumbar verte- 
bral column, stabile, applied to each side for one or two minutes, then longi- 
tudinally to the back) or the application of either electrode to the sternum, 
the other to the back, successively covering the various portions of the spinal 
cord, stabile, in recent cases the anode, if of long standing preferably the 
cathode, or even both poles alternately may be applied to the back. Where the 



TREATMENT 545 

spinous processes are specially sensitive, and there is girdle pain, intercostal 
neuralgia, hyperesthesias of the trunk, or lancinating pain, this " polar " treat- 
ment of the back (of the nerve roots, intervertebral ganglia, etc.) is recom- 
mended. 

In addition, peripheral galvanization of the extremities and their nerve 
trunks may be useful (anode to the lumbar enlargement, cathode labile along 
the entire course of the nerves in the leg, the same to the upper extremities). 

The use of the large electrode with only a moderately strong current, the 
thorough moistening of the skin, the smooth and skilful management of the 
electrodes, and the not too-prolonged administration, are all enjoined. 

The faradic current usually serves a symptomatic purpose; the faradic 
brush which Eumpf applies systematically to the skin of the trunk and the 
extremities with a moderately strong current, for from 5 to 20 minutes daily, 
perhaps favorably influences the regeneration of the sensory neurons by 
stimulating their functions; Eumpf has successfully used this method with 
a simultaneous inunction treatment, and I also can recommend it; especially 
in cases in which there are marked hypesthesia and extreme and lancinating 
pain. 

Static electricity, high tension currents, in the magnetic field, and other 
modern methods which are much employed and praised, have as yet no war- 
rantable scientific foundation. 

The application of derivatives to the slcin is still advised, and in some cases 
is undoubtedly useful. I employ these in different ways, especially when there 
is pain in the back, a girdle sensation with localized lancinating pains, gastric 
and anovesical crises. Occasionally I use them in a more permanent way by 
keeping up a counter-irritation on the back, and thus favorably influence the 
entire process. This action, although of very mysterious nature, is not to be 
denied. 

" Pointes de feu" (a small thermo-cautery applied over an area about the 
size of the hand, making 30 to 50 small superficial burns in an ascending row 
immediately over the vertebrae, applied every 6 to 8 days to the entire back, 
and continued for months), used and particularly recommended by French 
physicians, are most suitable for this purpose; so also are fly blisters, and 
painting with weak or strong tincture of iodin over the vertebral column. 
I have repeatedly applied mercurial plaster to the vertebral column for a 
long time, also alcoholic solution of veratrin, spirits of camphor, or similar 
rubefacients. 

Finally, we must mention the highly lauded and sometimes extreme surgi- 
cal or orthopedic curative measures which have recently been proposed in 
tabes. In spite of the exaggeration with which they have been introduced into 
practice, a slightly beneficial effect cannot be denied them. 

Massage has been much resorted to in tabes, and certainly has a beneficial 
effect upon the process as a whole; by stimulating the metabolism of the 
peripheral nerves, etc., it assists the circulation in the skin and muscles, stimu- 
lates the sensory tracts, increases the metabolism, the general nutrition, and 
the muscular activity. All the various movements of German and Swedish 
massage may be useful, provided they are not carried to an exhausting ex- 
treme. I have certainly seen great benefit from it, especially in poorly nour- 
ished, emaciated persons with weak muscles, loss of energy, sensory disturb- 



546 TABES DORSALIS 

ances, conspicuous ataxia, a dry, flaccid skin, etc., and for these cases it may 
always be recommended. 

Gymnastic exercise is also valuable as a general strengthening measure, 
assisting the muscular functions and the muscular activity, etc.; it has re- 
ceived a great impetus through Frenkel's " reeducation method," to which we 
shall revert when describing the symptomatic treatment of ataxia. That this 
treatment may also favorably affect the general process is obvious. But with 
gymnastics and massage, as with all other remedial measures, we must strictly 
individualize. 

Eecently but little has been reported of nerve stretching, formerly so much 
discussed, especially of operative nerve stretching. After a few apparently 
favorable results, we were led to perform a number of such operations, and 
during one of these both sciatics and both crural nerves were stretched. After 
a few cases had terminated fatally, and many had proved unsuccessful, the 
method was abandoned; Benedikt's opinion that it is a mistake not to per- 
form it has failed to prevent the method from falling into disuse. In the 
rare cases of very stubborn, localized, tabetic neuralgia affecting various nerve 
regions (sciatic crises, severe intercostal neuralgias, etc.) the method may 
be useful, for we cannot deny the possibility of an effect upon the spinal 
ganglia and the posterior roots. 

For these and similar cases, however, bloodless nerve stretching will suf- 
fice, and is certainly less dangerous, v. CorvaPs method of performing it is to 
have the patient lie perfectly flat with legs extended, then to flex the legs so 
strongly at the hip- joint that both feet approximate the head, this position 
to be maintained for some time; or, according to Hegar, the patient is to sit 
upon a table with the lower extremities extended, then the head and chest are 
to be strongly bent forward ("stretching of the spinal cord"). The milder 
method of Blondel is daily to approximate the flexed knee to the chin, and 
hold it firmly there for five minutes by tying a bandage around the neck of 
the patient and also around the popliteal space; Bonuzzi has the patient lie 
flat upon a bed with his head supported, fastens a towel around the ankles, 
then by flexing the vertebral column forward as far as possible the legs are 
drawn so high above the head that the knees are brought to the frontal region 
and even further. This movement is very painful, and not devoid of danger ; 
only the hypotonia of the tabetic enables him to perform it at all. 

All of these methods are occasionally productive of benefit, especially for 
the lancinating pains; they relieve, partly by stretching the nerves, partly 
by their effect upon the lower half of the vertebral column, perhaps also by 
stretching the posterior roots of the nerves and spinal ganglia. Hence, when- 
ever suitable, they may be cautiously used. 

The method introduced by Motschutkowsky, and eagerly adopted by Char- 
cot's disciples, that of suspension of the patient, also depends upon its effect 
on the vertebral column. Sayre's apparatus is used, the patient being sus- 
pended by the chin and nape of the neck while supported at the axilla, and is 
held in this position for from one to five minutes; this is done once daily or 
less often. The weight of the body stretches it, and elongates the vertebral 
column; perhaps it also produces tension of the meninges and nerve roots, 
and frees the nerve trunks. 

Sprimon's suspension apparatus is more simple and gives better results. 



TREATMENT 547 

The patient is seated, and weights of varying size (50 to 150 pounds) are 
attached to his elbows, his chin, and the nape of his neck; he is then slowly 
lifted. This method is susceptible of various modifications, is never injuri- 
ous, and may be continued for a longer time — from 10 to 20 minutes. Simple 
surgical extension of the vertebral column upon the oblique bed (as in caries 
of the vertebrae) may also be resorted to. 

No satisfactory explanation has yet been offered of the undoubtedly favor- 
able results of this process; certainly, in spite of suspension, some decided 
successes in tabes must be admitted, for the disease as a whole, as well as 
many of its individual symptoms — the lancinating pains, the girdle and armor 
sensations, the disturbance of muscular sensation and the ataxia, the bladder 
and sexual weakness, etc. — have shown decided amelioration. I can report 
some favorable results with this method, but, in many cases, it has been unsuc- 
cessful ; carried out according to the original method I do not consider it free 
from danger; very heavy patients, or those with arteriosclerosis, disease of 
the heart, or cerebral disorders, etc., should not be subjected to it. 

For slight but permanent suspension, extension of the vertebral column 
by a well made Hessing brace corset is useful. This furnishes a support and 
removes weight from the vertebral column; in fact appears to give a certain 
relief to many a patient. Bladder disturbances, pain, and paresthesia are 
lessened, and patients acquire a firmer station and gait. But the permanent 
results are by no means so brilliant as Hessing assumes; his versatility, as 
shown by the invention of such an apparatus, probably depends upon his 
ignorance of the pathology of the spinal cord, and is exaggerated by the 
naivete of his prognosis; actual cure is impossible, and I have seen a number 
of cases in which the patients soon objected to the corset, and refused to 
wear it. Nevertheless for many a corpulent and nervous tabetic it forms a 
good palliative remedy. 

An important part of the treatment of tabes remains to be discussed. This 
includes the almost endless symptomatic indications, which, as well as the 
treatment of the fundamental disease, call for the attention of the physician; 
when the malady is of long duration, and even after all hope of improve- 
ment is gone, these become more and more prominent. Above all, the treat- 
ment of the most typical and specially important symptom of tabes — ataxia — 
has lately been accurately studied and developed to a high degree, and we 
will now thoroughly discuss this as well as less noteworthy conditions. 

One of the earliest indications is given when the lancinating pains become 
at all severe, and — as in "tabes dolorosa" — these soon may become unbear- 
able. In the milder forms the patient frequently regards them as ordinary 
" rheumatic pains," he scarcely notices them, and does not seek professional 
aid, so that the affection is often recognized too late. 

For these pains numerous remedies are at our command; only a few of 
these have a certain effect, and the reaction in individual cases is very differ- 
ent, hence many remedies must be resorted to. In the most severe cases the 
patients suffer greatly, and only the injection of large quantities of morphin 
affords relief. 

At first, mild, external remedies should be tried; among these are warm 
or cold compresses, the application of cotton saturated with chloroform or 
ether to the painful and hyperesthetic areas, ethyl chlorid or ether sprays, 



548 TABES DORSALIS 

sinapisms, inunctions with oil of chloroform, or with veratrin dissolved in 
alcohol or in an ointment, the application of various blisters, of emplastrum 
opiatum or emplastrum belladonna?, etc., also counter-irritation with strong 
solutions of iodin, and the use of vesicators or pointes de feu on the vertebral 
column or over specially affected root regions. Electricity often has a favor- 
able effect, either the stabile application of the anode or cathode to the pain- 
ful area and to the affected root regions of the cord, or strong faradization 
of the skin (moist, or with the faradic brush). Friction and brushing of the 
skin, strong pressure (the tying on of leaden plates, etc.), massage, suspen- 
sion, bloodless nerve stretching, may also be very beneficial. 

Various internal remedies are, however, much more active; among these 
are quinin, the salts of iodin and bromin, gelsemium, and especially the 
numerous anodynes which have almost all been recently employed for the 
pain of tabes, and are very active. Chief among these is antifebrin (0.3-0.5 
per dose), then antipyrin (0.5-1.0), phenacetin (0.5-1.0), salipyrin, lacto- 
phenin, aspirin (0.5-1.0), exalgin, migrainin, pyramidon (0.25-0.75), etc. 
As in migraine, the effect of these remedies varies in different persons; hence 
they must be carefully tried in each individual case. Sometimes different ones 
may be combined with small doses of narcotics (codein, atropin, morphin, 
heroin) and thus a more powerful effect be secured. Lately I have often seen 
excellent results from a so-called "mixed powder" (phenacetin 0.6, antipy- 
rin 0.3, codein 0.04), and this may be used in different combinations. In 
severe cases, the doses must be large, and rapidly increased. In the worst 
forms, which are accompanied by severe paroxysms of lancinating pain, last- 
ing for days, only morphin will relieve the sufferer; yet this calls up the 
threatening specter of morphinism, therefore great care is necessary ! In each 
individual case we must decide how far it is safe to go. For myself, an exist- 
ence made tolerable by the use of morphin is infinitely to be preferred to 
unmitigated torture. 1 

Much more difficult, however, is the treatment of the various tabetic crises, 
especially those of the stomach and intestine, which are usually intractable 
and cause the patient great misery. These are best treated by complete rest 
and a strict diet (almost complete abstention from food, nourishment being 
by means of enemata and salt water infusions). The lancinating pain must 
be relieved by anodyne remedies, including morphin; warmth, cold, deriva- 
tives, pointes de feu, and vibration massage may also be of use. Sometimes 
electricity is effective (the galvanic current, a large electrode being applied 
to the epigastrium and abdomen, to the celiac plexus and the mesenteric plexus, 
to the splanchnic nerves, etc.; or faradization and the faradic brush) ; last of 
all we resort to the hypodermic syringe. Eecently some results have been 
noted from lumbar puncture. During the periods free from attacks, forced 
feeding should be employed as far as possible. For the laryngeal crises, inha- 
lations of chloroform or ether, spraying with cocain, galvanization of the cer- 
vical cord and of the pneumogastric and sympathetic nerves, narcotics, bromin, 

i The remarkable observation of Stembo {Neurol. Centralbl., 1904, p. 303), that 
after 28 injections of antirabic medulla emulsion in a case of tabes dolorosa the pains 
entirely disappeared, must be mentioned as a curiosity! French authors, Oberthur and 
Bousquet, have lately recommended subcutaneous injections of sodium nitrite (T-10 
cgm.) for the pains as well as for other tabetic symptoms. 



TREATMENT 549 

etc., may all be tried. For the anovesical and clitordl crises, local applications, 
sedatives, the salts of bromin, codein, heroin, and morphin are recommended. 

In the cases with paresthesia and anesthesia electricity by means of the 
faradic brush, or the labile application of the galvanic cathode, etc., forms 
the best remedy. Friction, massage, irritative inunctions (veratrin with spir- 
itus formicarum), may also be beneficial. 

We will rarely succeed in aborting the progressive blindness caused by 
atrophy of the optic nerve, although in a few instances energetic treatment 
with mercury and potassium iodid has been known to arrest the affection for 
some years. Many authors, as well as I, have seen such cases. The state- 
ment that this treatment increases the visual disturbance is unwarranted; 
nevertheless successes are rare. Besides prolonged galvanic treatment of the 
eyes, subcutaneous injections of strychnin, and the application of derivatives 
to the nape of the neck, etc., are advisable. 

In so far as they do not yield to the treatment of the underlying affec- 
tion, the paralyses which occur in the course of tabes, the various paralyses of 
the ocular muscles, the atrophic paralyses of the extremities, the paralyses 
of the muscles of the tongue, of the muscles of mastication, of the larynx, the 
bulbar paralyses, etc., should be treated according to the usual methods — either 
with the electric current, injections of strychnin, baths, massage, or exercises. 

More difficult is the treatment of the chief symptom of the second stage 
of tabes — the ataxia; this merits a detailed description, particularly because 
the results recently attained have in so many cases been exceedingly gratify- 
ing. We must not forget that we are here chiefly treating merely a symptom, 
although one of the most important and distressing, and that its removal 
conduces but little to the improvement or cure of the underlying affection. 
In this respect many authorities greatly exaggerate the treatment of ataxia, 
which they make too prominent. We might almost believe that there is abso- 
lutely no other rational treatment of tabes than the treatment of the ataxia; 
i. e., the treatment of a symptom which sometimes appears only 5, 10, or even 
20 years, after the onset of the affection ! 

Sometimes ataxia appears early, and in a few weeks or months rapidly 
develops to an extreme degree, perhaps in consequence of over-exertion, sexual 
excess, trauma, or the like. In such cases I advise complete and prolonged 
rest; the patients should remain in bed for weeks, certainly should be almost 
always in the recumbent posture, and should avoid all use of their legs. Under 
this treatment the ataxia may rapidly recede, and when this has occurred and 
the condition has become stationary the true treatment of ataxia should begin. 
Of course, we must never allow the debility and hypotonia of the muscles, 
the loss of energy of the patient, etc., to reach an extreme degree. 

The conditions are different in chronic cases of slowly progressive ataxia, 
or when this has already developed. In these cases particularly, a decided 
advance in treatment has been brought about by means of Frenkel's " reedu- 
cation method.*' It is greatly to Frenkel's credit to have formulated this 
" gymnastic treatment " of ataxia, and to have established it on such a sound 
theoretic and practical basis. 

In 1876 v. Leyden expressed the opinion that ataxia might in part be 
compensated for by developing and strengthening the muscles; here, it seems 
to me, he had especially in mind the increase of power in the muscles. 



550 TABES DORSALIS 

Frenkel's method, on the contrary, consists in gradually restoring by practice 
the use of the coordinating muscles, especially to lessen the immoderate mus- 
cular contractions which form such a prominent feature of ataxia. But to 
Leyden belongs the honor of having correctly appreciated 'Frenkel's method 
when first reported in 1889-90, for having combined it with his own idea 
of a possible " compensation for ataxia/ 7 and for having called the attention 
of the scientific world to the method. He recognized that Frenkel had brought 
a new factor — exercise — into the therapy of this disease. 

Certainly Leyden and his followers, Goldscheider, Gutmann, Jacob, and 
others, paved the way for the general recognition in Germany of Frenkel' s 
practice therapy; while in France, following Hirschberg's comprehensive re- 
searches, it was the School of the Salpetriere which, under Raymond's direc- 
tion, developed and broadened the method. I cannot here detail the history 
of this method. 

" Practice therapy " consists in learning oy systematic practice coordi- 
nated and proper movements in place of ataxic ones ; as this is really the prac- 
tice of coordinated movements which have been lost, the designation chosen 
by Raymond, " reeducation des mouvements," appears to be most fitting. 

The tabetics can to some extent control their ataxia, and are able to com- 
pensate for it, by the energetic exercise of their muscles, by concentrating 
their attention on their movements, and by using their eyes to control them, 
etc. ; we know this from the mere observation of any ataxic. As a rule, how- 
ever, the patient succeeds only imperfectly, and here practice in gymnastics 
is valuable since it enables him to attain better results, for he is trained to 
perform coordinated movements with the least possible muscular exertion. 

Theories regarding the results of practice therapy are naturally based on 
our views of the nature of the tabetic coordinative disturbance. We have 
previously discussed these, and have shown them to be very complicated, and 
by no means thoroughly understood ; but no matter what our opinions regard- 
ing ataxia, the benefits from exercises are readily apparent. We note daily 
that the fineness of our cutaneous and muscular sensations, as well as those 
of the special senses, may be greatly increased by practice, and also that by 
practice the fineness and precision of our most complicated movements may 
be extraordinarily increased. Therefore this depends either upon practice or 
an increase of the susceptibility to centripetal stimulations, so that " the 
regulating central apparatus contents itself with a minimum of sensory im- 
pressions" (Frenkel), or upon a vicarious sensory stimulation which is still 
retained (that is, a greater implication of the sense of feeling, and especially 
of the sense of sight) ; and, in regard to a new and changed exercise of the 
coordinative centers and conduction tracts, of the practice of a more rigid 
control, of a proper distribution, and the more accurately gauged strength of 
the individual centrifugal (motor) stimulations. The principle is this; that 
by frequently repeated, perfectly understood, and well controlled movements, 
the patient's mind being concentrated upon them, both centripetal and cen- 
trifugal stimulations are reawakened, refined, rearranged, or conducted into 
new tracts, and this reestablishes coordination. Therefore neither the strength 
of the muscles nor the activity induced by practice is the main condition 
(although these are not unimportant) ; but, by the production of sensory 
control and a compensatory sense of sight, a greater sensitiveness and pre- 



TREATMENT 551 

cision, and especially the proper distribution and strength of individual mus- 
cular activities in the production of complicated movements, is attained; here 
too slight, as well as too great, exertion is referred to its proper source. Hence, 
the essential point is practice for the acquirement of an easier and improved 
coordination of movements. 

That this presupposes a fine method, a strictly systematic process, which 
should be constantly under the direction and control of the physician, has 
been shown by the development of practice therapy. After determining the 
exact nature of the motor disturbance, we plan all the details of the individual 
movements. Naturally this plan will var}^ according to the severity of the 
case and the degree of ataxia; we must proceed gradually from the milder 
to the more severe exercises, from the use of individual muscles to that of 
a larger number of muscle groups; in the severer cases, exercises in the 
recumbent posture and while sitting should precede those while standing and 
walking; exercises in maintaining the equilibrium and in balancing the body 
require special attention. Leg exercises are more important, and usually more 
necessary, than those of the arms; both are based on the same principles, 
although the methods and aids are different. It is well to utilize the intelli- 
gence of the patients, as well as their understanding of the processes in co- 
ordination of movements ; this enables them to comprehend what the reeduca- 
tion method requires of them, and often proves of great service to the physician. 

Above all, the patient must avoid over-fatigue, chiefly for the reason that 
in tabetics "the sensation of fatigue" is usually lacking; therefore the} r are 
specially liable to over-exertion, and this is most injurious. This necessitates 
the supervision of a physician or of a well-trained nurse; the practice of 
these exercises can rarely be left to the patient. Certainly from time to 
time it should be superintended by the physician. Since this treatment must 
be carried out very energetically as well as carefully, and since, as a rule, 
no decided benefit can be seen until weeks or months have elapsed, it calls 
for great patience on the part of both the physician and patient. For the 
latter constant watchfulness, and for the physician a periodic supervision are 
necessary if good results are to be secured. 

The scope of this article does not permit me to detail individual methods. 
I can give merely an outline of the process, and must refer the reader to excel- 
lent manuals in regard to these gymnastic exercises; above all, to the explicit 
work of Frenkel, 1 to the monographs of Leyden, 2 of Goldscheider, 3 of Jacob,* 
of Hirschberg, 5 of Graupner, 6 of Stintzing, 7 and especially to that of 0. Fors- 

1 " Die Behandlung der tabischen Ataxie mit Hilfe der Uebung." F. C. W. Vogel, 
Leipzig, 1900. 

2 " Die Tabes dorsalis." Berlin, Urban & Schwarzenberg, 1901. 

3 " Anleitung zue Uebungsbehandlung der Ataxie." 2. Aufl., Leipzig, Gg. Thieme, 
1904. 

* " Physikalische Tberapie der spinalen Erkrankungen." " Handb. d. physik. 
Therapie," Leipzig, Gg. Thieme. 

5 " Traitement mecan. de l'ataxie locomotrice." Bull, gener. de Therap., 1893, 
30. Janv. 

6 " Die Bebandlung der Gangstorung bei der Tabes mittels der Uebungstherapie." 
Allg. med. Centralztg.. 1898, Nr. 38. 

7 " Behandl. der Erkrankungen des Rlickenmarkes," etc. ; " Handb. d. Therapie 
innerer Krankheiten von Pentzoldt und Stintzing." 3. Aufl., V, 1903. 



552 TABES DORSALIS 

ter 1 which gives all the details. These contain accurate descriptions and 
plans, and various more or less ingenious apparatus are described, so that any 
physician who desires to study the subject will here find the necessary direc- 
tions. There is nothing to prevent him from carrying out this treatment, but 
he cannot do it without carefully studying the method. If unable to do this, 
he should send his patients to a specialist, or to one of the institutions which 
are now everywhere established for the carrying out of exercise therapy. 

In the legs only the simplest exercises performed in a slow, measured way 
on command are at first possible. These consist of the flexion and extension, 
the abduction and adduction, the elevation and lowering of all the joints, the 
touching of definite points or of articles such as ten-pins with the tip of the 
foot, the performing of lateral and oblique movements, describing circles and 
other figures in the air, drawing figures upon pasteboard or tracing them upon 
the floor (circles, spirals, squares or other angular figures, numerals, stars, 
etc.), catching with the tip of the foot balls or coins which are moved to 
and fro, and the like. Then follow exercises in standing, rising and sitting 
down, in knee flexion, first with, then without, support and the aid of the 
eyes. Finally there is practice in standing and walking, sometimes by means 
of a walking frame or other support made for this purpose, sometimes with 
the aid of figures drawn upon the floor, or especially constructed linoleum, 
etc. Then follow exercises in standing with the feet widely separated, after- 
ward more closely approximated, finally with the feet close together; then 
standing upon one foot, upon the heels, upon the toes, at first with support 
and open eyes, then with the eyes closed; lastly in walking with the support 
of apparatus, the help of the physician, a stick, etc., taking carefully meas- 
ured steps forward and backward, walking in a straight line, then so as to 
describe certain figures, placing one foot immediately in front of the other, etc. 

All of these exercises necessitate the closest attention on the part of the 
patient, and, therefore, the supervision of a physician; at first they are very 
exhausting, and should be practised only one to three times daily, beginning 
with a few of them which are practised five or ten times, later twenty to forty 
times, then gradually extending the exercises to manifold combinations with 
intervals of rest; these periods of practice should not be longer than from 
15 to 30 minutes, and at the conclusion of the exercise mild massage of the 
muscles should be given to relieve the sensation of fatigue (Hirschberg). 

When there is ataxia of the arms, finer and more complicated exercises 
are necessary, and for these Frenkel has proposed ingenious apparatus and 
methods. These comprise at first flexion, extension, and spreading move- 
ments of all the joints of the hands and fingers, and touching the tips of 
the fingers with the thumb; then more complicated movements, such as the 
tracing of lines, figures, and circles upon paper, using a pencil in a groove 
or upon an edge, touching objects held before the patient, catching balls 
which are in pendulous motion, playing ball ; upon command touching a series 
of grooves in a board, sticking corks in holes, assorting coins, counting money, 
shuffling cards, playing the piano; finally, writing and drawing. These 
patients must also be taught the finer movements of the hand necessary in 
daily life, such as those in dressing and at table, with attention and control. 

i " Die Physiologie und Pathologie der Coordination," Jena, 1902. 



TREATMENT 553 

This treatment by exercise is very beneficial to many tabetics; we might 
say to nearly all who are ataxic. The earlier it is begun the better, and the 
more energetic, self-reliant, and hopeful the tabetics are, the greater the chances 
of success; but there are certain contraindications. When the ataxia is very 
recent and acute, and the patients are debilitated, weak in muscle, and have 
lost energy, when arthropathies, muscular atrophy, gastric or other crises are 
present, when the affection has reached the third stage, when there is atrophy 
of the optic nerve, advanced arteriosclerosis, valvular lesion, or cerebral dis- 
ease, this treatment is never in place. 

There can be no doubt that in many cases of ataxia this practice therapy 
brings about distinct improvement; the results are sometimes astonishing. 
Evidently suggestion and the removal of a purely psychical inhibition to co- 
ordination play a certain role. This, however, does not lessen the beneficial 
effect of the treatment. The general condition of the patient may be decidedly 
improved, as well as the hypotonia of the muscles and the naccidity of the 
joints. But I am under the impression that in our enthusiasm regarding 
exercise treatment the importance of our results has been exaggerated, as is 
the case with many therapeutic innovations. In not a few instances the bene- 
fits are slight or not at all apparent, in many cases they are only temporary, 
the ataxic loses patience and courage, he discontinues the exercises, loses 
control of the individual movements, and, finally, improvement ceases. The 
next ten years will show what permanent results may be expected from the 
reeducation method in tabes. 

Frenkel and v. Ley den have also advised exercise treatment as the best 
prophylaxis for ataxia. It should be instituted in the pre-ataxic stage, at all 
events as soon as ataxia appears, so as to conserve for the patient as long as 
possible his natural coordination, to enable him better to control and more 
accurately to perform movements, to guarantee to him a greater precision, and 
to show the effect of these movements. In the first stage, when there is but 
little disturbance of movement, it is difficult to induce the patient to under- 
take this tedious treatment. 

The question in how far the exercises can be combined with other curative 
methods — for instance, with baths, hydrotherapy, galvanism, massage, or in- 
ternal remedies — must be decided according to the circumstances of each indi- 
vidual case. As a rule, there is no objection to the use of tonics, change of 
climate, or a mild water treatment, and electricity is in most cases well borne. 
Massage properly given is beneficial for the exhausted muscles after exercise. 
We should be very cautious about recommending a simultaneous spa treat- 
ment. Certainly treatment at Nauheim and Eehme, especially the more pow- 
erful methods (Sprudel baths and Sprudel needle baths), are contraindicated. 
They exhaust the patient, and, moreover, it is unwise to combine them with 
exercise treatment; in my experience this is frequently injurious to the patient. 
The same is true of the use of Hg and iodin. I must, therefore, issue a 
warning against attempting too much in this condition. For practice therapy, 
fresh and recuperative powers are necessary. 

Bladder and sexual disturbances frequently demand treatment; except for 
a possible catarrh the former consist chiefly of deranged innervation and 
debility of the detrusor and sphincter muscles; they are frequently benefited 
by the use of electric currents applied according to well-known methods (but 



554 TABES DORSALIS 

not intravesical!); ergot and strychnin are also advised for weakness of the 
detrusor, and the latter is a valuable remedy for lack of power in the sphincter. 

With extreme weakness of the detrusor and partial retention of the urine, 
we should first attempt to relieve the bladder by manual compression from 
the abdomen, which is usually successful ; this should be followed by the early 
employment of the catheter, but with the greatest care in order to guard 
against cystitis. By this process we may often prevent or stop the very un- 
pleasant dribbling of urine. Massage of the bladder (bimanually from the 
rectum and abdomen) is sometimes beneficial, as well as treatment by sus- 
pension. 

Sexual disturbance in those rare cases which, in the initial stages, show 
increased sexual irritability and libido or frequent pollutions must be ener- 
getically treated with large doses of bromin, camphor, lupulin, or heroin (0.01 
or 0.02 given at evening), cool sitz baths, a regulated diet, and the avoidance 
of all sexual excitement. 

In the much more common cases of diminished or lost potency it is usu- 
ally advisable not to restore the sexual activity of the patient; nevertheless 
in some cases this may be attempted — and, as I have often noted, with success ; 
by suitable hydriatic applications and electricity, by the careful use of nux 
vomica, of tonic pills, of preparations of arsenic, and by suspension, which, 
in this class of cases, gives astonishing results. I must urge caution in the 
use of yohimbin by tabetics. 

For the obstinate constipation almost always present, the usual dietetic 
and physical methods, also drugs, are available. It is better to withhold 
the last as long as possible, and at first to employ only physical remedies (mas- 
sage, hydrotherapy, faradism, enemata) with a regulated diet. The later 
stages always give abundant opportunity for the use of purgatives. 

Muscular atrophy is a trophic disturbance which must usually be com- 
bated, and electricity, massage, injections of strychnin, friction, and all kinds 
of gymnastic exercises, are useful. The arthropathies so commonly noted 
are not amenable to treatment. They are progressive ; but in their first stages 
may sometimes be favorably influenced or arrested. The most successful treat- 
ment is by complete rest of the joint, mild compression, counter-irritation 
with iodin, inunctions of iodin vasogen, or ichthyol ointment, and, occasion- 
ally, various surgical measures (puncture of the hydrarthrosis, resection, etc.). 
As a rule, such surgical treatment is unsatisfactory; then nothing remains 
but orthopedic relief by suitable apparatus. These often help the patient for 
a long time. 

Bed-sores are to be treated according to the well-known surgical principles 
(cleanliness, avoidance of pressure, antisepsis, etc.) ; the same applies to the 
perforating ulcer of the foot, mal perforant, which is occasionally observed, 
and in which, according to my experience, treatment by mercury and iodin 
combined with suitable antiseptic dressings, etc., is often very beneficial. Chi- 
pault has recently treated most successfully several cases of this complication 
by stretching the nerves of the leg. 

In conclusion, a word concerning the psychical treatment of tabes. In a 
severe disease of such prolonged duration, this is naturally of the highest im- 
portance. It is only wise and humane to conceal from the patient as long 
as possible the gravity of his affection, but we must impress him with the 



TREATMENT 555 

importance of submitting to the necessary treatment, and of carrying it out 
persistently. We need not mention locomotor ataxia or tabes. I usually tell 
the patient that he is suffering from an irritation of the nerve roots of the 
back, or of the membranes of the spinal cord, and that by suitable treatment 
this may be greatly relieved, but I let him understand — particularly if he is 
easy-going — that, under some circumstances, the affection becomes very much 
aggravated. 

We must always seek to inspire him with the hope of improvement and 
recovery, with confidence in his physician, and in the mode of treatment pur- 
sued. Variation in the treatment, regular but light exercise, harmless enjoy- 
ment, change of residence and scene, the stimulation of varied interests, driv- 
ing, pleasant company, the theatre, music, etc., often serve to keep up the 
courage of the patient. In tabes little is to be expected of true suggestion, 
especially of hypnotic suggestion. But, without this, a skilful, tactful physi- 
cian will be able to exercise a certain authority over the unfortunate sufferer. 

Following this almost endless enumeration of remedies and methods in 
tabes, a few words are permissible concerning the general plan of treatment. 
This to some extent is shown by the foregoing presentation. But it must be 
modified according to the individual circumstances, external conditions, the 
position, mode of life, and the social, pecuniary, and family relations of the 
patient. 

Therefore the individual indications are to be considered carefully, and 
no means necessary to the desired end should be neglected. The long dura- 
tion of the affection and its many complications make it our duty carefully to 
choose the method of treatment, bearing in mind the change of seasons ; a vari- 
ation in the methods may serve to reawaken hope in the patient. The psychi- 
cal elements of the case demand close consideration. The uncertainty of suc- 
cess, the difference in the reaction of individual patients, justifies us in trying 
various methods of treatment. Their results will shape the further process; 
treatment productive of benefit should, naturally, be continued; that ineffec- 
tual should be discarded. 

Under any circumstances, we must caution the patient against over-exer- 
tion. Different methods are not to be resorted to indiscriminately or employed 
simultaneously, although all combinations are permissible. 

The treatment, at least in patients of the better classes, can often be car- 
ried out in spas or other institutions for the treatment of nervous diseases; 
here much depends upon the choice of the place and the physician. For the 
lower classes, treatment in clinics and in hospitals is preferable to that in 
their own homes. 

In the first stage of the disease, and especially at the onset, we must seek 
a causal indication; should this necessitate anti syphilitic treatment, it must 
be carefully but energetically undertaken, and, under some circumstances, must 
be repeated after intervals; these pauses can be utilized for treatment by 
tonics, a change of air, a bath cure, or electricity. A choice between hydro- 
therapy and thermal salt or other baths will depend upon the circumstances 
and the season of the year. In winter galvanism, the administration of vari- 
ous drugs, the application of derivatives, suspension, perhaps also slight fric- 
tion or artificial C0 2 baths, may be resorted to. For wealthy patients we must 
consider the advantages of change of climate. In any case the patient's mode 



556 TABES DORSALIS 

of life should be strictly regulated, and every attention should be paid to 
the symptomatic treatment of the individual case. 

In the second or ataxic stage specific and tonic treatment may alternate. 
But, as a rule, the practice of exercises should supplement these, and form 
the chief treatment. In addition, we may try any other curative measures: 
in a warm climate treatment by water and baths, in winter electricity, massage, 
suspension, and drugs. Careful regulation of the diet, so as to improve the 
general nutrition, and the consideration of the psychical condition of the 
patient are necessary, besides meeting the symptomatic indications which now 
fill a wide field. 

In the last stage, the hopeless patient who seeks relief should be spared 
exhausting treatment, journeys to spas, and expensive special treatment, and 
we should advise only the most necessary change of scene. Massage, elec- 
tricity; and tonics may here be of benefit. Our chief reliance must now be 
upon symptomatic therapy and the psychical treatment of the sufferer. In 
the latter sense it may occasionally be desirable to institute a new mode of 
treatment, so as to arouse hope in the patient and divert his mind. This may 
be more or less effective, and even in the last stages the tactful and humane 
physician may be able to give such relief that to his patient he appears to be 
a helpful and sympathetic friend. 



MULTIPLE SCLEROSIS 
By E. REDLICH, Vienna 

Multiple sclerosis is in many respects of especial interest. In the first 
place it is one of the most common affections of the central nervous system; 
but the cases of multiple sclerosis in which, considering all the circumstances, 
the diagnosis can be made with certainty are far outnumbered by those in 
which the differential diagnosis may be perplexing. And, moreover, the clin- 
ical aspect of multiple sclerosis may vary considerably; this variation, which 
often furnishes surprising clinical pictures and impresses its stamp upon the 
course of the disease, necessitates an explanation; hence, from clinical obser- 
vation and the pathological anatomy of multiple sclerosis interesting questions 
may arise which involve the general pathology of the nervous system. 

Cruveilhier, in his celebrated Atlas, was probably the first to describe ana- 
tomical preparations in cases of this disease and to furnish illustrations. 
Frerichs, Leyden, Eindfleisch, Schiile, then Vulpian and others, reported addi- 
tional cases. Charcot, with his peculiar intuitive faculty, must be credited 
with having separated the mass of symptoms presented in the peculiar symp- 
tom-complex, and clearly defined the typical clinical picture of multiple scle- 
rosis. Above all, it became possible by this means to diagnosticate multiple 
sclerosis from paralysis agitans with which it had been commonly confounded ; 
Charcot's contributions to our knowledge of multiple sclerosis naturally extend 
much further ; for, at the same time, he gave us an insight into the pathology 
of this disease and its definite histological landmarks ; for example, the reten- 
tion in the sclerotic foci of the axis cylinder, this being fundamentally neces- 
sary for our understanding of the affection. Since Charcot described multi- 
ple sclerosis we have acquired additional knowledge of it, details of which 
will not now be given. But one point is of special significance. The clinical 
picture, delineated by Charcot, is certainly typical of many cases but not of 
all, and certainly not of all stages of multiple sclerosis, the course of which 
may be protracted for many years. Subsequently other authors, among whom 
were Oppenheim and Bruns, showed that other groups of symptoms might 
frequently be found in multiple sclerosis, perhaps as often as Charcot's pic- 
ture, that the so-called " formes frustes " of Charcot have an important place 
in the clinical description of multiple sclerosis, and that among these pictures, 
which often vary so decidedly, there are many very different from the Charcot 
type, yet which must be regarded as typical cases of multiple sclerosis. But 
of this later. 

557 



558 MULTIPLE SCLEROSIS 

ETIOLOGY 

On considering the distribution of the disease it may be stated that the 
sexes are almost equally affected. Charcot reported that the female sex showed 
a special predilection, and this was confirmed by Berlin (among 39 of the 
latter's cases 26 were women). More recent statistics, however, do not coin- 
cide. For instance, Lent had, among 51 cases, 37 men and 14 women; Krafft- 
Ebing had, in 100 cases, 58 men and 42 women; 12 of my 23 cases were men. 
Frankl had, among 206 cases, 140 men and 66 women; Probst, in .58 cases, 
34 men and 24 women; Bruns-Stolting, among 38 cases, 13 men and 25 
women. This does not show a preponderance of the female sex, but, on the 
contrary, of the male. I must emphasize that multiple sclerosis, as a rule, 
occurs somewhat more frequently among the poorer classes than the well-to-do. 

The question of its age incidence is more interesting. Charcot stated 
that the disease is more common in the young, which succeeding authors have 
confirmed. This is especially significant from the circumstance that in multi- 
ple sclerosis which has persisted for years the history often shows that its 
onset dated back many years. 

Since, therefore, the majority of cases of multiple sclerosis are between 
20 and 30 years of age when they come under observation, we may certainly 
assume that in many cases the onset was much earlier, even in childhood. In 
spite of the difficulty in diagnosticating multiple sclerosis in childhood, it 
may be positively maintained that typical multiple sclerosis does occur in 
children. 

Marie, Nolda, Unger, Frankl-Hochwart (8 cases with onset between the 
first and tenth years of life), and others have reported such cases; Zenker, 
Schiile, Humphrey, and Eichhorst have confirmed this by anatomical findings. 
But it must not be forgotten that multiple sclerosis is also observed in later 
life, hence the statement of Charcot that the affection rarely occurs after the 
fortieth year is erroneous, but was apparently due to accidental circumstances. 
Cases at fifty years of age, and often after sixty, have been described, among 
them an entire series which were proven by autopsy to be typical multiple 
sclerosis, cases in which the history removed all doubt that the disease had 
developed during later life. 

After determining the general etiologic limit, we should ascertain the 
actual cause of the disease ; and here we encounter difficulty. First, an impor- 
tant question must be decided. Are we dealing in multiple sclerosis with an 
exogenous or an endogenous disease, i. e., is multiple sclerosis due to external 
cause in persons previously entirely healthy, in whom there might be at most 
a certain predisposition, or are such factors unnecessary? Is it a faulty pre- 
disposition which produces a pathologic degeneration of the central nervous 
system, and does this, without external lesion, perhaps from internal cause 
or merely functional use, cause multiple sclerosis? The majority of authors 
incline to the first opinion, but the theory of the endogenous nature of multi- 
ple sclerosis has also found prominent supporters who base it upon the circum- 
stance that numerous cases of multiple sclerosis are observed in which the 
etiologic factors known to us, even allowing the greatest latitude, are inop- 
erative. 

First, from the anatomical standpoint of Ziegler and Jutzler, congenital 



ETIOLOGY 559 

anomalies with areas of unusually close neuroglia have been thought to be 
responsible for the development of sclerotic foci. Striimpell is decidedly of 
the opinion that multiple sclerosis is an endogenous disease. He observed the 
concurrence of hydromyelia, a congenital anomaly of the spinal cord, with 
multiple sclerosis, and this led him to suspect its endogenous origin, a view 
which was further confirmed by the appearance of the disease in childhood 
and by the persistence of the axis cylinders, which in his opinion is not the 
case in exogenous diseases. Although Hoffmann also recently inclines to this 
theory, I must confess that I do not consider it well founded. 

The factors mentioned by Striimpell are soon discussed. The coincidence 
of hydromyelia and multiple sclerosis is exceptional, therefore of no signifi- 
cance. Hydromyelia is found in every other disease, even in persons with 
sound nerves, just as frequently as in multiple sclerosis; furthermore, it is 
not true that axis cylinders do not occur in exogenous diseases. It is sufficient 
to call attention to the periaxial neuritis of Gombault in experimental lead 
poisoning, in which affection the axis cylinders remain perfectly intact and 
the medullary sheath alone is diseased. Multiple sclerosis in childhood may 
readily be due to other factors (see later). 

The assumption of an endogenous cause of a disease, a reference of it 
to congenital factors, must be based upon very weighty reasons, especially 
clinical, if it is to constitute an etiologic explanation ; for the anatomical view 
of a " predisposition " to the affection under discussion has little foundation. 
Clinical experience, therefore, in the great majority of cases, is opposed to 
the view of an endogenous origin of multiple sclerosis. 

Those affections in which, considering all that is known, we feel justified 
in assuming an endogenous origin often prove, in the absence of all other 
causes, to be of hereditary, family nature, or. at least, we recognize a strong 
hereditary predisposition in the affected individuals. This is not the case 
in multiple sclerosis. We have an example of direct heredity in an interest- 
ing report by Eichhorst of a mother and child in whom the existence of mul- 
tiple sclerosis was verified by anatomical investigation. Without wishing to 
detract from the value of this observation, it seems proper to state that in the 
mother the puerperium played a role in the causation and development of the 
disease, a factor which we also encounter in the etiology of multiple sclerosis. 
In the mother, therefore, multiple sclerosis was probably of exogenous origin. 
In a second report of hereditary multiple sclerosis affecting a mother and 
child, and described by Ella, the cases were only clinically observed; the cases 
of Cestan and Guillain, in which two children in one family showed the 
symptoms of multiple sclerosis, were not confirmed by the necropsy. 

The same is true of other isolated reports of the family appearance of mul- 
tiple sclerosis. Other authors unanimously admit that hereditary predispo- 
sition plays no special role in the etiology of multiple sclerosis, i. e., as a rule, 
persons affected by the disease show no marked predisposition, and these alone 
come seriously into question. 

In reviewing the other factors considered with more or less justice to 
be the causes of multiple sclerosis, it is evident that in the course of time 
views have greatly altered, that much which played a prominent role in earlier 
literature has to-day been relegated to the background, and much has been 
emphasized without this change having shaped our views. The psychical fac- 



560 MULTIPLE SCLEROSIS 

tors formerly enumerated, fright, excitement, etc., may be disregarded. We 
must admit that multiple sclerosis, heretofore latent, may appear after psy- 
chical emotion or psychical trauma, but we can hardly ascribe to these factors 
an actual causative role. The influence of exposure to cold is different. In 
earlier literature this played a significant, and even the main, role in the 
etiology of multiple sclerosis, as will appear to any one who studies the works 
of the early writers ; with them contracting a cold was one of the most impor- 
tant etiologic factors. All this is changed to-day; refrigeration has more and 
more been replaced by causes which could be better substantiated. But al- 
though this is true, and it is to-day impossible to determine the precise impor- 
tance of exposure to cold and dampness, and thus make these a clear patho- 
logical formula, yet it must be conceded that these factors have an undoubted 
etiologic significance in multiple sclerosis. Among recent authors, Krafft- 
Ebing strongly maintains this. Other authors are more conservative, and 
believe it to be true in a small number of cases; the majority are unanimous 
in admitting this factor. Every author of experience has known cases of 
previously healthy persons in whom, after a more or less severe chilling or 
wetting, for example, by falling into water or into snow, or being thoroughly 
drenched by a heavy rain, etc., the symptoms of multiple sclerosis appeared, 
or they subsequently developed after indefinite symptoms. In other cases it 
is the same factor working in a more chronic way, for example, by living in 
damp, draughty rooms, etc. ; this is apparently the only cause that can be 
determined. 

Under the stimulation of a report by Marie — Leyden, Kahler and Pick 
had even earlier reported the same — another etiological factor assumed promi- 
nence, that is, the influence of preceding infectious disease. The fact that 
multiple sclerosis occurred more frequently in youth than in later life was 
utilized to show the etiologic dependence of the malady on infectious diseases, 
for the number of infections occurring in youth or infancy is much greater 
than in adult life. Thus the argument used by Striimpell to prove the endog- 
enous nature of multiple sclerosis, probably has another more correct appli- 
cation. Recently the etiologic importance of preceding infectious diseases 
received a certain setback because of the skepticism of some prominent authors, 
among whom we must mention Krafft-Ebing, Striimpell and Hoffmann, who 
have absolutely denied that this is a cause. 

Let us investigate the facts. We have long been familiar with the appear- 
ance of multiple sclerosis after enteric fever. It has also developed after 
variola, pneumonia, erysipelas, diphtheria, measles, scarlatina, dysentery, chol- 
era, influenza, angina tonsillaris, rheumatism, etc. There are also reports, 
especially recent ones, of the relation of multiple sclerosis to malaria. For 
example, Spiller cites the case of a sailor, aged 40, who presented the symp- 
toms of multiple sclerosis. Anatomical examination revealed the presence 
of numerous sclerotic foci in the central nervous system, and the capillaries 
of the brain were filled with the estivo-autumnal parasite of malaria. 

The number of cases which individual authors have described as occurring 
after infectious diseases varies widely. This is due to the circumstance that, 
only in the rarest cases, can we demonstrate an immediate connection between 
a preceding infectious disease and the appearance of multiple sclerosis; this 
was true of a case I described in which, after an angina tonsillaris, probably 



ETIOLOGY 561 

of diphtheritic nature, the typical symptoms of post-diphtheritic polyneuritis 
first developed, and, after some years, multiple sclerosis became manifest. 
This case finds its confirmation in one on which Henschen held an autopsy, 
and in which multiple sclerosis and polyneuritis could be demonstrated as 
following diphtheria. In other cases, relatively soon after the disappearance 
of the acute infectious disease, an indefinite, pathologic, nervous stage sets in, 
the persons convalescing from the infectious disease are not restored to perfect 
health, and subsequently the picture of multiple sclerosis develops. In other 
cases there is an intervening period, lasting even years, of apparently perfect 
health between the infectious disease through which they have passed and the 
appearance of multiple sclerosis, so that we are forced to doubt their etiologic 
connection. Still, the last mentioned circumstance is not a positive proof of 
the contrary. It may be stated that multiple sclerosis has a long period of 
latency. Nothing prevents us from assuming that this is the case because the 
onset of multiple sclerosis with its vague symptoms is masked by the symp- 
toms pertaining to convalescence from acute infections. 

If, on the other hand, we take the view that multiple sclerosis is not the 
direct consequence of an immigration of microorganisms into the central 
nervous system, and their local propagation, many factors make it difficult 
to estimate the influence of infectious disease in the etiology. We may adhere 
to the importance of this factor in multiple sclerosis although unable to state 
in how many cases it becomes operative ; the number is probably by no means 
small. In the first sense, we may utilize the fact that an already existing 
multiple sclerosis may, after an intercurrent infectious disease, sometimes show 
distinct aggravation and an increase of symptoms. 

It is universally admitted that, in contrast with other diseases of the cen- 
tral nervous system, syphilis plays a slight, if any, role in the etiology of mul- 
tiple sclerosis. Aside from the fact that the picture of syphilis resembles 
multiple sclerosis, there are but few cases in which, with any degree of proba- 
bility, hereditary or acquired syphilis may be considered an etiologic factor 
in its development. 

A number of cases have been observed in which multiple sclerosis occurred 
after pregnancy and the puerperal state, and even in afebrile conditions with- 
out infection. We have alread}^ mentioned a case of Eichhorst's as belonging 
to this category. Oppenheim, Balint, and others mention the same etiologic 
factor; and I have also observed such cases. The fact, several times noted, 
that symptoms of multiple sclerosis become more intense after the puerperal 
period must be here mentioned. How this influence is to be explained, 
whether by the foregoing alterations in metabolism, by the return of the geni- 
talia, etc., to their normal condition, or whether toxic products are the cause, 
cannot be determined ; analogous conditions, in a certain sense proofs, are 
found in the appearance of polyneuritis and various psychoses during preg- 
nancy and the puerperium. This fact leads us to propound another question : 
Are intoxications an etiologic factor in multiple sclerosis? Of the acute poi- 
sons we can, in this respect, mention only isolated cases of carbonic oxid gas 
poisoning which, as we know to-day, under some circumstances promotes the 
development of other serious changes in the central nervous system (softening, 
etc.). Chronic alcoholism, certainly, has no special influence. But this is not 
the case with other chronic intoxications, particularly the metallic poisons. 
37 



562 MULTIPLE SCLEROSIS 

Oppenheim called especial attention to chronic metallic poisoning contracted 
in an occupation. Here he mentioned lead, copper, verdigris, zinc, and anilin 
dyes, and he recently reported an interesting case of multiple sclerosis (which 
I also had an opportunity of seeing) in which the poisoning due to zinc in 
dyed stockings (traces of zinc were found in the urine) had produced nephritis, 
and gradually multiple sclerosis. 

Recently Embden, and subsequently Jaksch, described a peculiar clinical 
picture appearing among workers in manganese (Embden calls it a manganese 
dioxid, grinder's disease) which symptomatologically closely resembled multi- 
ple sclerosis. Embden, as well as Jaksch, differentiates this affection from 
multiple sclerosis, although both of these authors assume multiple changes 
in the central nervous system to be the anatomical foundation. As no 
autopsy reports are } r et available, this point cannot be decided. Neverthe- 
less, from our present standpoint, this affection is not without importance, 
and may be said to favor Oppenheim's view, for we cannot deny that his opin- 
ion has been seriously opposed. In the majority of cases there is certainly 
no question of chronic intoxication due to occupation for the disease occurs 
in the young who have not yet been compelled to work or in persons in walks 
in life which exclude all possibility of such chronic intoxication. Therefore, 
the number of cases in which this etiology may be considered is certainly not 
large, but its possibility must be admitted. 

We must now mention a deleterious factor which, as was shown by recent 
authors, among them Kiewlicz, Jutzler, and Mendel, may not rarely produce 
multiple sclerosis, and this fact is trauma. But we must be very cautious in 
assuming trauma to be the etiologic factor. It is evidently such if a previ- 
ously healthy individual after severe trauma, for example, a fall from a great 
height, is not restored to perfect health, but gradually develops all the symp- 
toms of multiple sclerosis. I remember a case that was very characteristic. A 
previously healthy girl, while on a raft and in consequence of the raft's col- 
liding with another vessel, sustained a fall upon the buttocks. Certain sequels 
remained, and years later the unmistakable picture of multiple sclerosis devel- 
oped. As an index of this genesis of the affection, symptoms referable to the 
lower portion of the spinal cord, such as paralysis of the bladder and rectum 
and paresis and ataxia of the lower extremities, were very conspicuous. Cases 
of trauma to the vertebral column in consequence of a fall or blow upon the 
back or the head should be considered in a similar light. On the other hand, 
there are cases in which the relation of the symptoms is less clear, either because 
the trauma was slight or because multiple sclerosis developed too long after. 
Here decision is very difficult ; even after a careful weighing of all the circum- 
stances it can be made only with the greatest reserve. But the etiologic influ- 
ence of trauma upon the development of multiple sclerosis in a previously 
healthy individual is in a certain number of cases undoubted, and this is very 
important in considering accident cases. In differentiating these supposedly 
traumatic cases we must bear in mind the fact that trauma may greatly aggra- 
vate already existing symptoms of multiple sclerosis. 

I have now stated the chief and positive points upon which we may base 
a more or less well-founded assumption of the etiology of multiple sclerosis. 
In the main these are the same factors to which we attribute many other 
organic diseases of the nervous system. Why, in one case, their effect is so 



SYMPTOMATOLOGY 563 

severe as to produce an organic, progressive, nervous affection, while in many 
other cases there are no serious consequences, why multiple sclerosis arises 
and not another form of organic disease, we are unable to say. But this is 
not true of multiple sclerosis alone. As a rule, our etiologic diagnosis does 
not actually correspond with the real condition; an unknown quantity, per- 
haps several such, frequently remains. 

As I stated in the introduction, in a number of cases of multiple sclerosis 
we are unable to find any etiologic factor, anything which we may with some 
degree of probability assume to be the cause of the disease. It is to be hoped 
that the researches of the future will bridge this gap in our knowledge of 
the malady. 

SYMPTOMATOLOGY 

We shall now discuss the uncommonly rich symptomatology of multiple 
sclerosis, and shall first describe the symptoms in regard to their frequency 
and importance, subsequently their grouping, and the course which charac- 
terizes its typical forms. 

Among the symptoms emphasized by Charcot because of their frequency 
we first find tremor and nystagmus. It is at once evident that these belong 
together. Charcot aptly described the tremor of multiple sclerosis as an inten- 
tion tremor, i. e., it does not appear during rest but with intended movements. 
The tremor is most distinct in the upper extremity, particularly in the hands ; 
for example, the arms hang down and are completely at rest, but as soon as 
the patient attempts to grasp any object a tremor occurs which during the time 
the motion is being made increases decidedly and is sometimes an actual shak- 
ing. The tremor is even more distinct when tbe patient tries to hold any 
heavy object, for example, a vessel full of water, when the tremor finally 
becomes so marked that the patient spills some of the water. It is particu- 
larly noticeable in the hands while writing. Specimens of handwriting reveal 
distinctly this course, gradually increasing tremor, and here it must be men- 
tioned that because of a simultaneous ataxic disturbance the writing is very 
irregular. The lower extremities not infrequently show this tremor, as well 
as the musculature of the trunk and the head. Here also its intentional char- 
acter is generally marked ; i. e., when the patient, who has been lying quietly 
upon his back, rises or walks a coarse, usually increasing, tremor develops, 
and Oppenheim has characteristically designated this as waddling. Since 
even standing still requires the continuous innervation of many muscles which 
serve to maintain the erect posture, here also there is usually a decided tremor 
or waddling. Oppenheim has reported that reflex movements, for example, 
the extension of the knee-joint after percussion of the tendon of the quadriceps 
tendon, sometimes produce tremor. It must be added that an intention tremor 
is often more fully developed upon one side than upon the other, and may even 
be unilateral, an observation which is further borne out by the hemiplegic 
mode of development of multiple sclerosis (which will later be explained) as 
well as by the fact that in rare cases the intentional type of the tremor is not 
well marked, but there are certain transitional stages which lead to a more 
continuous type of tremor. 

Intention tremor is a common symptom of multiple sclerosis. Of course, 



564 MULTIPLE SCLEROSIS 

it is not observed in all stages of the disease ; it may be safely said that it is 
found, at least periodically, in from 50 to 60 per cent, of the cases. 

Quite analogous to intention tremor is nystagmus. This similarity is in 
many cases at once apparent, for the oscillation of the eyes, designated nystag- 
mus, does not appear when the eyes are quiet, but occurs only upon turning 
them laterally or upward. If the patient rolls the eyes to the extreme left, 
there is sometimes only a to and fro motion while in this position, and it is 
soon arrested; in other cases, however, this motion ceases only when the eyes 
have returned to their former position. The same oscillation is seen when the 
patient looks up or down. In other cases rotatory nystagmus occurs, the 
rapid oscillations succeeding each other in a varying excursion. Here we may 
mention a movement described by Kunn, in which two or three lightning-like, 
horizontal twitchings appear when the patient attempts to gaze steadily at any 
object. The same author refers, to tremor of the ciliary muscle — certain dis- 
turbances of sight when looking at close objects. 

In the frequent cases in which horizontal or rotatory nystagmus occurs 
when the eyes are directed forward, we have a condition analogous to tremor 
on standing still, for even when the glance is directed straight forward a cer- 
tain continuous muscular innervation is necessary. 

The majority of authors estimate that nystagmus, as a conspicuous but 
by no means typical symptom of multiple sclerosis, occurs in about one-half 
of all cases. 

To the motor disturbances we must now add others, first, those which occur 
in the extremities. Charcot called attention to the frequency of spastic pareses 
in the lower extremities; and subsequent investigations have confirmed their 
importance. In multiple sclerosis, at least in some stages, more frequently in 
the later but sometimes rather early, there are more or less well developed 
pareses, the nature and appearance of which of course vary; when the lower 
extremities are attacked there may be all degrees, from slight implication of 
motility to the most profound paralysis. Often the sides are not uniformly 
affected; one leg may be more involved than the other, or the affection may 
be confined to one leg alone. While, therefore, in the milder cases, the patients 
note only a weakness of the affected leg, an increased lassitude and fatigue so 
that the legs become stiff on walking for a short time, in severe cases the 
power of locomotion decreases more and more, the gait becomes remarkably 
spastic-paretic in character, and the footsteps are short and dragging; finally 
the paralysis becomes complete and the patient is bedridden. The paretic legs, 
as a rule, exhibit distinct, very often conspicuous, spasms and contractures, 
and even passive movements meet with resistance; the tendon reflexes are 
greatly increased ; where the implication is not uniform they are usually most 
increased upon the side most markedly affected ; we also find patellar and foot 
clonus. With severe spasm we frequently note Babinski's toe-reflex. Well 
developed flaccid paralysis is seldom seen. Loss of the tendon reflexes is very 
rare. Marked atrophy is scarcely ever observed and moderate atrophy only 
exceptionally. 

Paralysis of the lower extremities often develops gradually, and increases 
in the course of years. If the disease is not steadily progressive, well devel- 
oped pareses may disappear or, at least, their intensity is lessened. But a more 
acute onset of paralysis in the lower extremities has been noted, even in rela- 



SYMPTOM AT()L<>< i V 565 

tively early stages, and in this complication improvement or complete disap- 
pearance of the paralysis is sometimes observed. 

To the motor disturbances due to spastic paresis of the legs, in quite a 
number of cases ataxic symptoms are added. I must call especial attention to 
this combination of spastic paresis and increased ataxia as a common symptom 
in multiple sclerosis. As a rule the ataxia resembles the cerebellar form. 
There is a swaying, particularly upon walking, a deviation from a straight line 
which may increase to actual waddling, but is less unsteady than in tabes 
dorsalis. In these cases, the patient while standing feels very insecure, espe- 
cially when the space on which he stands is reduced by his placing his feet in 
juxtaposition when, without closing his eyes (unlike the case in tabes dorsalis), 
there is a marked increase in the swaying. With unilateral paresis we occa- 
sionally note that the ataxia also is chiefly one-sided. In some cases ataxic 
phenomena are observed without especially marked paresis. It must be added 
that in isolated cases v. Frankl-Hochwart noted a gait which is usually typical 
of paralysis agitans, namely, retropulsion and propulsion, i. e., a backward and 
forward movement of the patient which, after a corresponding intention or 
after a slight push, causes him to break into a run either backward or forward. 

Paresis of the upper extremity is less prominent among the symptoms of 
multiple sclerosis, although it is by no means rare, and is more often uni- 
lateral than bilateral. In the cases of common hemiplegia type there are 
pareses in the amis and legs of the same side. 1 must emphasize a not unusual 
distribution of the paralysis — paraparesis, or paraplegia, of the legs and paresis 
of one arm; sometimes this develops from the hemiplegia type in such a way 
that the more marked affection of the leg corresponds to a paresis of the arm 
upon the same side. In severe cases there are usually spastic symptoms in 
the upper extremities; contractures develop and the tendon reflexes are in- 
creased. What has been stated of the appearance and course of paralysis of 
the lower extremities is also true of that of the upper extremities. 

We have already mentioned that the addition of ataxia to the usual inten- 
tion tremor causes an impairment in the use of the arms; hut it is not always 
easy here to discriminate between tremor and ataxia. Some authors, for exam- 
ple, Strtimpell — in my opinion erroneously — have gone so far as to consider 
intention tremor and ataxia identical. Oppenheim has occasionally observed 
an acute hemiataxia of the upper and lower extremities. 

Marked atrophy of the upper extremity is also rare. It is most often found 
in the small muscles of the hand, and was described by Charcot. Some cases 
have been reported which resembled the picture of amyotrophic lateral scle- 
rosis; Charcot mentions a case which simulated spinal muscular atrophy. 

Among the spinal paralytic phenomena, disturbances of the bladder must 
be mentioned. These were formerly declared to be rare, but we know to-day 
that they may be very common in multiple sclerosis. Frankl-Hochwart and 
Zuckerkandl noted them in four-fifths of the cases, especially with paraplegia 
of the legs. As in other pareses, these may be temporary paralytic conditions 
which appear and rapidly disappear, or, especially in the later stages, such 
disturbances may persist. As a rule, there is dysuria, difficult and prolonged 
urination, sometimes with strangury and intact sensibility of the bladder; 
in other cases there is incontinence of urine with a constant dribbling, particu- 
larly in the terminal stages of the disease. Impairment of the activity of the 



566 MULTIPLE SCLEROSIS 

rectum is not very common, being usually seen only in the form of chronic 
constipation. 

In comparison with the motor disturbances, sensory disturbances are less 
important. Charcot's opinion that these are usually absent has not been 
confirmed, as we know, by the investigations of Oppenheim, Freund and 
others; but these sensory disturbances are rarely intense or conspicuous, and 
they can sometimes be discovered only after thorough and repeated investiga- 
tions ; often they are only transitory, existing for weeks and months, and then 
disappearing. Sensory disturbances may appear either with the late or the 
early symptoms. The patients complain of paresthesia, burning, formication, 
numbness in one or the other leg (the periphery being less frequently at- 
tacked), more rarely there is pain which, however, is not lancinating. 

Examination not infrequently discloses objective disturbances of sensibil- 
ity, such as a uniformly diminished sensation to all stimuli. In the later 
stages, particularly when the paraplegia is severe, there is in some cases total 
anesthesia of the legs. In isolated cases disturbances of thermal sensibility 
preponderate. 

Here and there the sensory and motor disturbances are distributed accord- 
ing to the type of Brown-Sequard's paralysis. We sometimes find a combi- 
nation of multiple sclerosis and hysteria which may naturally produce exten- 
sive sensory disturbances, a point to which we will revert when discussing the 
differential diagnosis. 

The cutaneous reflexes are rarely altered ; the occurrence of Babinski's sign 
has been previously mentioned. Vasomotor and trophic disturbances of the 
skin are rare or may be wholly absent. Bed-sores occur only in the terminal 
stages of the disease in patients long bedridden. 

Disseminated foci distributed over the spinal cord and brain are character- 
istic of multiple sclerosis. It, therefore, becomes necessary to describe the cere- 
bral disturbances, especially those prominent in the symptom-complex of the 
affection under discussion. 

On account of their frequency and diagnostic importance, symptoms refer- 
able to the optic nerve must primarily be considered. 

In his reports Charcot called attention to the importance of changes in 
the optic nerve and their clinical characteristics ; Uhthoff , with his pupil, Lub- 
bers, has lately investigated this subject, and given us very comprehensive 
reports. Among other authors who have done much to increase our knowl- 
edge of these disturbances I must mention Oppenheim, Frank, Bruns, and 
Stolting. Statistics reveal their importance. Uhthoff demonstrated ophthal- 
moscopic changes in the optic nerve in 45 per cent, of all his cases; Bruns 
and Stolting report disorders of vision as the first symptom of multiple scle- 
rosis in 30 per cent, of their cases. 

In this description we shall differentiate between demonstrable ophthalmo- 
scopic changes in the optic nerve and disorders of vision; these by no means 
always correspond. 

First, in regard to the ophthalmoscopic appearance of the papilla, in well 
marked cases there is sometimes a slight general atrophy of the optic nerve. 
Frequently this is not symmetrically distributed, but the temporal half is pale 
and distinctly atrophied. This temporal pallor of the papilla is the most char- 
acteristic of the ophthalmoscopic findings in multiple sclerosis. Total atrophy. 






SYMPTOMATOLOGY 567 

on the other hand, is only exceptionally demonstrated. But in some cases 
described by Uhthoff, Lubbers, Oppenheim, and lately by Bruns and Stolting, 
there was typical optic neuritis, even true choked disc. 

We must now mention a point of special significance. The ophthalmo- 
scopic changes may take place very early; for example, an atrophy of the 
optic nerve or neuritis may wholly or almost entirely disappear, and some time 
subsequently, in isolated cases even after years, the other symptoms of multi- 
ple sclerosis may develop. Between the appearance of changes in the optic 
nerve, which sometimes may be most acute, and the actual onset of the disease 
there may be a stage of latency of varying duration, even years. In other cases, 
the optic nerve becomes affected at a relatively late stage and shows a slow but 
progressive advance. These alterations are more frequenth r unilateral than 
bilateral, that is, they are more conspicuous on one side than on the other. 

What is true of the ophthalmoscopic changes is also true of the disturbances 
in sight of which the patients complain; these may come on very early and 
acutely and lead to total blindness, and then gradually improve, or they may 
form a late symptom that sometimes, although rarely, shows steady progres- 
sion. The disturbances in sight are not always due to distinct ophthalmo- 
scopic alterations, and, vice versa, notwithstanding evident pallor, even atrophy 
of the papilla, vision may be very clear. As a rule, however, disturbances in 
sight correspond with the ophthalmoscopic picture. In the early stages cen- 
tral scotoma, such as we observe in retrobulbar toxic neuritis, is relatively 
common. The scotomata are rarely total, more frequently they relate only to 
colors, particularly green and red. In other cases, on the contrary, there is a 
limitation of the field of vision, rarely concentric, usually irregular. Some- 
times a diminution of central acuity of vision is combined with anomalous 
extension of the visual field. Permanent total blindness is very rare. 

Not quite so common as disordered vision but, nevertheless, frequent 
enough, are disturbances in the innervation of the muscles of the eye. Paral- 
ysis of the muscles of the eye is sometimes an early but transitory symptom, 
so that we not infrequently hear the patient with multiple sclerosis state that 
he long ago had double vision. Often there is partial paralysis of the oculo- 
motor nerve, as a rule unilateral, a paralysis of one abducens, etc. Some- 
times there is so-called associated paralysis of the muscles of the eye, for exam- 
ple, paresis of the oblique muscles or the muscles of convergence. In isolated 
cases extensive permanent ophthalmoplegia has been observed. Disturbed 
innervation of the pupils, above all, inequality of the pupils, anisocoria, may be 
transient or permanent; myosis or mydriasis is sometimes observed. On the 
other hand, disordered reflex activity of the pupil is very rare; occasionally 
there is a sluggish reaction to light. Uhthoff once found reflex rigidity of the 
pupil, twice the reaction of convergence was absent. Probst and Pini have 
noted an absence of pupillary reaction. As this finding is very rare, it is ques- 
tionable whether this was not due to complications. 

"We have previously referred to the frequency of nystagmus, which is due 
to a disturbance of the innervation of the muscles of the eye. 

Isolated paralysis of other cranial nerves than the facial is rare; this is 
prone to occur in the hemiplegic forms of multiple sclerosis, and has all the 
characteristics of cerebral facial paralysis (predominant implication of the 
middle and lower branches) and is relatively common. Facial paralysis impli- 



568 MULTIPLE SCLEROSIS 

eating all the branches, sometimes combined with simultaneous paralysis of 
the abducens and crossed with paresis of the extremities (pons!), has occa- 
sionally been observed. Spasms in the course of the facial nerve have been 
described. Oppenheim reported a case with symptoms referable to the fifth 
nerve (as the first evidence of a developing multiple sclerosis there was per- 
sistent neuralgia of the fifth nerve and a sclerotic focus at its point of exit). 
Affections of the auditory nerve, for example, nervous deafness, are very rare. 

We must more minutely consider the disturbances of speech in multiple 
sclerosis. In Charcot's classic description they occupy a prominent position; 
above all " scanning speech," i. e., a slow, measured wa}^ of speaking in which 
the different syllables are quite distinct from each other, as in the so-called 
scanning of verses is significant. This form of speech is very frequent, but 
not invariably present, nor at every stage. In some cases the speech is exceed- 
ingly slow, bradyphasia, and at the same time it is hoarse, low, monotonous, 
without modulation, or it may be stuttering or explosive. In addition to 
these speech disturbances there are in some cases rare but significant symp- 
toms of a dysarthritic nature; words are not clearly articulated, some par- 
ticularly difficult sounds are indistinct ; the speech can scarcely be understood ; 
it resembles that observed in progressive bulbar or pseudobulbar paralysis. 
Sometimes the movements of the tongue are impeded, and there are cases 
which to a certain degree resemble bulbar paralysis. In these, speech is very 
indistinct and almost incomprehensible, the lips move slowly, the expression 
of the face is flaccid, the tongue is markedly implicated, deglutition is diffi- 
cult (fluids regurgitate through the nose), and the retching reflex is dimin- 
ished or absent. Paresis of the tensors of the vocal cords was observed by 
Oppenheim. Increased salivation is noted as well as disturbance of the action 
of the pneumogastric nerve, such as irregular and accelerated action of the 
heart, oppressed and jerky respiration, etc. A conspicuous feature of pro- 
gressive bulbar paralysis is absent in these rare cases of multiple sclerosis, 
namely, atrophy of the implicated muscles; slight emaciation, for example, 
of the tongue, has been observed. 

We must now discuss some general cerebral disturbances which are quite 
common in multiple sclerosis, for example, headache. This, as a rule, is 
neither persistent nor particularly severe. There are, however, cases in which, 
in the early stages or even as the first symptom, uncommonly severe headache, 
such as we find, for instance, in tumor of the brain; this, however, may sub- 
sequently cease. This headache may be accompanied by severe vertigo, and 
vertigo alone is often experienced in multiple sclerosis. There is also syncope, 
but loss of consciousness with epileptiform convidsions is more rare. 

Apoplectiform attacks are specially interesting. Sometimes they are early 
symptoms, or it may happen that they introduce the disease. They may dis- 
appear very rapidly, or for a few days cause paralysis of a monoplegic, hemi- 
plegic, or paraplegic character. In some of these cases — for example, when 
of hemiplegic type — these cerebral paralyses are somewhat more intense and 
may be persistent. Such apoplectic attacks with paralysis may occur in the 
further course, even in the late stages of the disease. 

Finally, we must mention the psychical disturbances; rarely well devel- 
oped, they are more common than was formerly believed; and, as has lately 
been proven, even severe psychoses may be due to multiple sclerosis. A very 



SYMPTOMATOLOGY 569 

frequent symptom — not of psychic nature but, nevertheless, conveying a strange 
impression — has been designated as spasmodic laughter or crying, for, on the 
slightest cause, sometimes even without any (for example, when stimulated 
by the laughter of another person, echo laughter), the patient laughs immod- 
erately. Perhaps the opposite symptom, spasmodic crying, is more often 
noted. When the patient describes his symptoms to the physician, and, finally, 
when he is addressed in any way, he cries, and the tears roll down his cheeks. 
He cries like a child. It takes some time to pacify him and on the slightest 
cause he begins anew. This curious spasmodic laughter and crying is also 
seen in other affections, in hemiplegia, in bulbar paralysis, etc. We shall not 
attempt to explain it, because, in my opinion, this is impossible. We may 
regard these symptoms as expressive of a loss in inhibition, of a lack of self- 
control, hence a certain psychical influence cannot be denied. In some cases, 
perhaps even in the majority, the psychical state remains intact to the end. 
In others, there is a slight impairment of intellection and memory, a certain 
veiling and obtuseness of the psychical personality, a predominant childish- 
ness, occasionally an unmistakable euphoria, or, at least, the patient takes 
an optimistic view of his own condition; sometimes he is apathetic, or, 
as Oppenheim states, shows a mania for joking. Sometimes the psychic 
disturbances are severe; there may be a marked impairment of intelligence, 
even to dementia or confusion, excitement with hallucinations, etc. These are 
relatively rare and their explanation must be sought in the occurrence of mul- 
tiple foci in the cortex of the cerebrum. 

In isolated cases, the coincidence of multiple sclerosis with true progressive 
paralysis has been described, and the difficulty of this diagnosis will be revealed 
when we discuss the differential diagnosis of multiple sclerosis from progressive 
paralysis. 

We have discussed the various symptoms of multiple sclerosis, and will 
now study their grouping in individual cases. 

The symptom-complex described by Charcot is the typical form ; those 
running another course are the formes frustes, but, as we have already men- 
tioned, the latter are actually the most common, Charcot's complex being 
noted only in a minority of eases. I repeat, a combination of intention tremor 
and nystagmus with disturbances of speech, not necessarily always of scan- 
ning character, in which there are quite invariably or at least in the later 
stages, spastic paretic symptoms on the part of the lower extremity, must 
be regarded as the classical type. To this are frequently added affections of 
the optic nerve with disordered vision and transitory, but rarely permanent, 
paralysis of the muscles of the eye. Paresis, especially of the lower extremities, 
not rarely accompanied by ataxic symptoms, may be observed. 

In other cases there is neither nvstasjmus nor intention tremor, and we 

n JO 

find merely paralytic symptoms in the lower extremities combined with spastic 
phenomena, disturbances of the bladder, occasionally also sensory disturbances; 
in a word, the picture of transverse interruption of the spinal cord. In rare 
instances, the symptoms of spastic paresis combined with ataxic disturbances 
are limited for years to only one of the lower extremities. These have led 
to the differentiation of a purely spinal form of multiple sclerosis, which is 
especially favored by Leyden. In some of these cases symptoms of a cerebral 
nature for a certain time supervene or precede; this is particularly true of 
38 



570 MULTIPLE SCLEROSIS 

changes in the optic nerve. Oppenheim and Frank have shown that multiple 
sclerosis may run its course under the picture of spastic spinal paralysis, a 
circumstance which will be more minutely discussed under spastic spinal paral- 
ysis, but in these cases atrophy of the optic nerve or typical temporal pallor 
of the papilla may point to the existence of multiple sclerosis. 

It has been mentioned that in isolated cases atrophy may be added to these 
spastic symptoms in the upper and lower extremities, and the picture of amy- 
otrophic lateral sclerosis thus be simulated. In my experience the so-called 
hemiplegic type of multiple sclerosis, hemiplegia of spinal origin with unilat- 
eral paralysis of the extremity, or of cerebral nature involving the facial 
nerve, and in which the upper and lower extremity upon the same side are 
involved and the symptoms may appear acutely, apoplectiform, are quite fre- 
quent. Occasionally we observe a crossed paralysis of the extremity and of 
the cranial nerve (facial, abducens), and here for a long time, perhaps even 
permanently, there may be neither intention tremor nor nystagmus, or at most 
but slight tremor of the paretic upper extremity. Subsequently disturbances 
of the bladder are added, or paresis of the other side, or bilateral hemi-paresis 
with predominant implication of the legs, or changes in the optic nerve may 
supervene, and finally, nystagmus, typical disturbances of speech, or dysarthria. 
Cases with bulbar paralytic disturbance, with or without other typical symp- 
toms of multiple sclerosis, have been described. 

We would digress too far were we to enumerate all the aspects which mul- 
tiple sclerosis, particularly in the stage of onset, may assume. The foregoing 
should warn us again schematism. It should be borne in mind that the symp- 
toms of multiple sclerosis may vary greatly, and in all doubtful cases its possi- 
bility must be considered. 

COURSE 

We will consider briefly the course of multiple sclerosis. As a rule, it 
is an extremely chronic disease, under some circumstances lasting for years, 
even ten to twenty ; but we have several times stated that in the great major- 
ity of cases it is by no means invariably and steadily progressive, as are other 
chronic diseases, in the sense that the symptoms appear gradually and grad- 
ually become aggravated. On the contrary, acute periods of aggravation fre- 
quently alternate with periods of improvement. This variation in the inten- 
sity of the symptoms, these remissions, sometimes lasting for one or two 
years and longer, which may even simulate perfect recovery, are among the 
typical features of multiple sclerosis. An acute onset, apparently when in 
perfect health, severe symptoms followed by periods of aggravation and im- 
provement, and a stage with relapses, are typical of multiple sclerosis. Under 
the consideration of the etiology we mentioned that external agencies, such as 
cold, trauma, infections and the puerperium, may aggravate the malady. In 
other cases the cause cannot be determined. A more profound paralysis finally 
ensues, and the patient may be bedridden for years. 

In opposition to this exceedingly chronic course we have recently seen 
subacute cases which are, on the whole, more rapid; if the symptoms begin 
acutely, slight amelioration is rapidly succeeded by aggravation, so that the 
disease is fatal in a relatively short time, about two years. 



PATHOLOGY 571 

Death is often the result of intercurrent diseases ; more rarely, but particu- 
larly when the paralysis is severe and the patients are bedridden, death is due 
to bed-sores, phlegmons, cystitis, etc. 

PATHOLOGY 

We must now investigate the pathological anatomy in multiple sclerosis, 
and try to explain the symptoms by these pathological findings. The presence 
of numerous disseminated sclerotic foci in the brain and spinal cord is char- 
acteristic — the existence of a pure spinal form or a pure cerebral form must 
be considered doubtful — and they vary in magnitude from the size of a pin's 
head or lentil to that of a filbert. By the confluence of several foci large scle- 
rotic plaques may develop, the form of which is rarely circular but usually 
irregular. In comparison with normal tissue the foci of multiple sclerosis 
are elevated so that in fresh sections they usually appear to be slightly elevated 
if recent, but contracted if of long standing. As a rule they show a gray or 
grayish red color. The number, seat and distribution of the foci in individual 
cases is subject to great variation, although even here there is a certain stand- 
ard. In the optic nerve and chiasm the foci are frequently of varying extent 
but implication of the optic nerve is common; the cortex of the cerebrum is 
rarely attacked although, in opposition to former views, in many cases the 
presence of numerous foci has been demonstrated. Foci are occasionally noted 
also in the cortex of the cerebellum. The centrum semiovale, especially the 
area surrounding the lateral ventricles, is often the seat of extensive foci, 
while they are less common and less extensive in the trunk ganglia. The pons 
and medulla oblongata are the preferred seats, and here the foci may fill 
the greater part of the transverse section, covering extensive areas, and equally 
implicating the white and gray substance. In such cases the sections are often 
smaller and contracted, they are coarse in texture, and sometimes appear 
slightly transparent. In the spinal cord there are usually numerous foci, the 
form and extent of which vary greatly ; some are the size of a pin-head or a 
millet-seed, some the size of a pea or even larger. Sometimes the entire 
transverse section or the greater part of it is implicated, in which case the 
circumference of the spinal cord is usually decreased in lesions of long stand- 
ing. The longitudinal extent of the foci also varies. Large areas of the spinal 
cord may show diffuse changes. Occasionally there is a special implication 
of the cortical regions. Foci may be found in the anterior and posterior roots 
of the cord, while they are quite rare in the peripheral nerves; it has even 
been doubted whether they are ever found there. 

While macroscopically and in preparations the foci are, as a rule, sharply 
defined, under minute microscopic investigation the normal tissue appears 
to be only slightly involved in the sclerotic focus. 

I shall not minutely discuss the histologic examination, especially as the 
findings are not always the same. Some prominent points should be empha- 
sized. A characteristic of ordinary foci is a marked increase of glia tissue, 
especially of the glia fibers, which appear as a dense film composed of parallel 
or interwoven fibrillse. We usually find within this dense glia tissue — in suit- 
able preparations also in longitudinal sections — naked axis cylinders or axis 
cylinders whose medullary sheath has been greatly diminished. The axis cyl- 



572 MULTIPLE SCLEROSIS 

inders themselves may appear unchanged ; in other cases they reveal slight his- 
tologic alterations. Some authors maintain a regeneration of axis cylinders 
in multiple sclerosis, but these have probably been confounded with glia fibers. 

This retention of the axis cylinder — described by Charcot and Schultze — 
is very characteristic of multiple sclerosis. Although we find naked axis cylin- 
ders in other processes, in these they form an exceptional occurrence, and not 
the rule as in multiple sclerosis. As the axis cylinder represents the most 
important constituent of the nerve fiber, this explains the circumstance that 
even extensive foci of multiple sclerosis, for example, in the pons, the spinal 
cord, etc., may show no secondary degenerations. Nevertheless, there are 
exceptions to this rule. For instance, we may maintain that with intense 
sclerotic foci a certain number of axis c}dinders perish; this is most apparent 
in the cases running a subacute course, where the secondary degeneration may 
even be marked. 

Let us first consider the ordinary picture of multiple sclerosis in which we 
know that even extensive foci usually show histologically less damage than 
might be expected. This is apparent also when we examine foci of the gray 
substance; here, upon minute examination, we are often astonished to find 
absolutely or comparatively intact ganglion cells in the foci of apparently 
severe sclerosis. It is true that some have suffered considerable damage. It 
must be remembered that, among the symptoms, we mentioned the relative 
rarity of muscular atrophy; this finds its anatomical correlation in the above 
circumstances. 

The vessels in the foci of multiple sclerosis frequently show change. They 
are often dilated, their walls infiltrated or much thickened, the tissue around 
them somewhat indurated. But this finding in the vessels is by no means 
invariable, and the occurrence of such changed vessels in the center of foci 
cannot be considered characteristic of anatomical changes in multiple sclerosis, 
as is maintained by some authors. 

The histologic picture of cases with subacute course differs somewhat from 
this description; here histologically we usually perceive the signs of acute 
degeneration, such as medullary granules and fatty granular cells; glia pro- 
liferation, as a rule, is but slight ; swollen axis cylinders are numerous ; in fact, 
the true sclerotic character of the process is but slightly if at all apparent. 
To this we must add that the nerve fibers are sometimes completely destroyed 
in circumscribed areas so that empty spaces develop, or spaces filled with fatty 
granular cells, and give to the tissue a so-called vesicular appearance. Almost 
invariably we find changes in the vessels, which are dilated, their walls often 
infiltrated, the adventitious sheaths filled with fatty granule cells. The same, 
or a similar histological picture, is commonly seen in the periphery of old foci, 
and forms the anatomical sign of subacute and acute aggravations of the 
process with which we are already familiar from the clinical picture. Corre- 
sponding to acute foci in those areas in which nerve fibers have to a large 
extent actually disappeared, we sometimes find secondary degeneration, either 
ascending or descending. The meninges, as a rule, remain uninvolved, and 
only occasionally have slight inflammatory processes been described therein. 

According to the valuable researches of Uhthoff and Lubbers, focal anatom- 
ical changes also occur in the optic nerve. Histologically they resemble those 
found in multiple sclerosis; they consist of marked proliferation of the inter- 



PATHOLOGY 573 

stitial tissue of the internal optic nerve sheath as well as of the finer elements ; 
in some areas there are marked contraction, disappearance of the medullary 
sheaths, and the persistence of axis cylinders; sometimes, however, there is 
complete atrophy of the nerve fibers with severe vascular changes and, as a 
cause of dilatation and increase of the same, small celled infiltration, thick- 
ening of the walls, etc. The focal character of the changes, the persistence 
of the axis cylinders, and the consequent absence of extensive secondary degen- 
eration, differentiates this process from the atrophy of the optic nerve which 
is common in tabes dorsalis. 

In the general pathology of multiple sclerosis much might be said of the 
nature of the pathologic process, but since the views of eminent authorities are 
by no means unanimous and are still under warm discussion, we shall be brief. 
First is the question, What is the nature of the fundamental process and where 
does it arise, or, differently expressed, are we dealing with an inflammatory or 
non-inflammatory process, and is it of parenchymatous or interstitial form? 
Are the changes in the nerve fibers of primary, and the proliferation of the 
supporting tissue of secondary nature, or vice versa? In answer to the first 
question, multiple sclerosis has been included among the inflammatory proc- 
esses; Leyden, for example, regards it as a form of chronic myelitis. Based 
upon clinical factors the acute appearance of some cases, the paroxysmal 
progress of others, the transition of various clinical pictures into multiple 
sclerosis, etc., transitions from acute disseminated myelitis or from dissemi- 
nated encephalomyelitis, have been regarded as multiple sclerosis, and this 
has led us to adopt the view of its inflammatory genesis. Additional argu- 
ments have been found in the histologic picture of multiple sclerosis, especially 
in the not infrequent and severe vascular changes and infiltrations surround- 
ing these. Others, however, have pointed out that the positive inflammatory 
processes of the central nervous system in many points resemble other pictures 
than those of multiple sclerosis, that vascular changes in themselves prove 
nothing, that anatomical chronic inflammatory processes of the central nervous 
• system are exceedingly difficult to define. This question, however, involves a 
second, namely, Whence does the process arise? Charcot and many others 
assumed the primary condition to be an inflammatory process in the supporting 
structure which leads to proliferation, and that from this, therefore indirectly, 
the parenchymatous portion of the central nervous system is damaged. 
Opposed to this is the view of a primary damage to the nervous parenchyma, 
especially to the medullary sheath and by the consequent diminution of the 
resistance of the tissue, the glia is stimulated to proliferation. I cannot dis- 
cuss the pros and cons of these views nor shall I consider, for example, the 
disturbance of the lymphatic circulation which, according to Borst, consti- 
tutes the primary pathologic factor. 

Let us attempt, nevertheless, to gain some insight into the pathologic 
condition. Evidently the circulation plays a role in the production of the 
changes. Infectious toxic products, which we have learned to recognize as 
among the etiologic factors of multiple sclerosis, which to some extent may 
also be the consequence of exposure to cold, may damage the circulation after 
trauma — that trauma may damage the vessels and the circulation we know 
from other experiences — and are probably the cause of the pathologic changes. 
The nervous parenchyma, being the most sensitive tissue, first shows reac- 



574 MULTIPLE SCLEROSIS 

tion by the disappearance of the medullary sheath; irritated by this, but per- 
haps also because the same irritation which caused the degenerative processes 
in the parenchyma acts as a more powerful irritant to the supporting tissue, 
proliferation of the glia follows. Since, as we have seen, the circulation plays 
a causative role in these disturbances, it does not surprise us to find histological 
changes in this structure also. The varying pictures then depend upon the 
greater or less intensity of the deleterious factor, upon its especially injurious 
nature or its especially rapid entrance into the central nervous system, and 
enable us to understand why in one case there is merely slight parenchymatous 
damage with scarcely noticeable glia proliferation, and in another severe degen- 
eration of the nerve fibers with histologic pictures which otherwise would be 
designated as inflammatory processes. Possibly there may be transitions of 
multiple sclerosis into inflammatory processes, for purely degenerative and 
inflammatory processes are exceedingly difficult to distinguish from each 
other — much more so than is commonly supposed. In the last mentioned form 
of multiple sclerosis there is apparently an extreme effect, whereas usually 
slight damage of the parenchyma is characteristic — hence the persistence of 
the axis cylinders and ganglion cells. 

Let us briefly review the symptoms of multiple sclerosis from the stand- 
point of pathological anatomy. Much that we have learned to recognize loses 
its conspicuous character. We can readily understand that numerous foci 
may be present without generating special symptoms and that there may be 
a prolonged period of latency if the foci of multiple sclerosis leave intact the 
chief constituents of the functionating substance, the ganglion cells and nerve 
fibers. Hence the disproportion so often observed between the clinical and 
anatomical findings is to a certain degree only an apparent one. The acute 
exacerbations of the disease are explained by the more rapid advance of the 
anatomical process, due apparently to disturbances in the circulation of the 
blood and lymph which produce a greater derangement of function than actual 
degeneration. With the cessation of these initial phenomena and the persist- 
ence of but slight anatomical changes, the clinical symptoms also markedly 
decline. The remissions of the more chronic cases may be similarly explained, 
while the regeneration of axis cylinders which have perished need not be 
considered, this process having by no means been proven although some authors 
use it in explanation. 

The frequency of spastic paretic symptoms, of disturbances of the bladder, 
etc., briefly, of symptoms which belong to the ordinary picture of a transverse 
interruption of the spinal cord, is accounted for by the remarkable changes 
which the extensive foci in the spinal cord undergo. Ataxic symptoms are 
not always referable to the same cause; they are seldom due to spinal foci 
which extend to the posterior columns; much more often they are produced 
by foci in the medulla oblongata which reach the cerebellar peduncles, etc. 
The hemiplegic cases, in particular, have a spinal seat, and when the cranial 
nerves are implicated they are of cerebral or bulbar nature ; the latter locality 
must be borne in mind for the common disturbances in speech, foci in the 
pons and cerebral peduncles causing paralysis of the muscles of the eye, and 
disturbances in sight being due to foci situated in the optic nerve and optic 
chiasm. 

But I cannot discuss in detail the various possibilities which may explain 



DIFFERENTIAL DIAGNOSIS 575 

the symptoms of multiple sclerosis. On account of the numerous aspects of 
the clinical and anatomical picture, in the majority of cases a satisfactory 
explanation can only be arrived at after a minute comparison of the anatom- 
ical findings with the clinical picture of the individual case. At the same 
time, it is extremely difficult to elaborate an anatomical foundation for the 
classical symptoms of multiple sclerosis, especially for nystagmus and the 
intention tremor. Every attempt at localization, i. e., the reference of these 
symptoms to definite localities, and their implication, is beset with difficulties, 
since the symptoms may be absent in spite of the undoubted affection of the 
localities. Is the damage in such cases not sufficiently severe? We do not 
know. Perhaps, as in nystagmus, we are dealing with a symptom which is 
susceptible to many explanations, and this enhances the perplexity. Prob- 
ably there is also an indirect damage to the motor and coordinating tracts in 
question, and these symptoms are, to a certain extent, merely the expression 
of a general weakness of the systems which come under consideration. 

DIFFERENTIAL DIAGNOSIS 

We now come to one of the most important and interesting chapters, 
namely, the differential diagnosis of multiple sclerosis. Many cerebral and 
spinal processes must be considered in a differentio-diagnostic respect, as well 
as a number of so-called neuroses. In some cases all our diagnostic power 
must be brought to bear upon the case and yet it may be impossible to come 
to a decision. On the contrary, this must be based ujdou the subsequent course 
of the affection. 

To be emphasized as especialty characteristic of multiple sclerosis are the 
following: The coincidence of cerebral and spinal symptoms, occasionally a 
distribution which directly points to numerous foci in which there have been 
for years remissions and acute exacerbations. We will now consider in detail 
the differential diagnosis. 

If we find the picture portrayed by Charcot : nystagmus, intention tremor, 
disturbances of speech, spastic paretic symptoms in the extremities, perhaps 
combined also with ataxia, disturbances of the bladder, characteristic affec- 
tions of the optic nerve and a typical course, the diagnosis will generally not 
be difficult. But even here we must be cautious. Westphal, subsequently 
Strumpell, v. Frankl-Hochwart and others have shown that in a small number 
of cases there may be a condition analogous to Charcot's picture, while the 
autopsy reveals no characteristic changes nor any anatomical alterations which 
indicate multiple sclerosis. These cases, described as pseudo-sclerosis, as a 
rule begin early, about the tenth year or a little later; heredity appears to 
have some influence, and even a family predisposition has been observed. 
Strumpell believes that hereditary syphilis may perhaps play a role. There 
are invariably disturbances of motility, less frequently paralysis, at most only 
in the latest stages, while spastic phenomena, even marked contractures and 
spastic gait, are common; a remarkable slowness in movement is also some- 
times noted. The tendon reflexes are exaggerated. Usually there is tremor, 
particularly of the upper extremities, which does not, however, closely resem- 
ble intention tremor but is slower, much coarser, and may terminate in pro- 
found jactitation, Disturbances of speech are prone to appear, above all 



576 MULTIPLE SCLEROSIS 

slowness of speech, and also dysarthric affections. On the other hand, nystag- 
mus has rarely been observed; there are neither changes in the optic nerve 
nor sensory disturbances. Pain or slight paresthesia is occasionally mentioned. 
Bladder affections are rare. A common, perhaps an invariable symptom, is 
psychical disturbance, which sometimes increases to actual dementia. Spas- 
modic laughing and crying have also been observed. Attacks of syncope and 
apoplexy are relatively frequent, and sometimes leave paralysis which lasts 
for several days; epileptiform attacks have several times been noted. As a 
rule, these cases of pseudo-sclerosis are of long duration, ten years or, under 
some circumstances, much longer. Remissions may occur. 

Thus a clinical picture is presented which bears a close resemblance to 
multiple sclerosis, but certain differences may be discerned; for instance, the 
paretic symptoms are less developed, there is usually no actual paralysis, the 
tremor is not absolutely typical, bladder disturbances are rare, there is no 
change in the optic nerve or paralysis of other cerebral nerves; on the other 
hand, psychical disturbances, etc., are more prominent. Moreover, we must 
admit that if the cases described as pseudo-sclerosis be compared we see no 
uniform picture ; some of these force upon us the suspicion voiced *by Strum- 
pell that we are dealing with juvenile progressive paralysis. We must not 
forget that some of these cases originated at a time when the clinical picture 
of juvenile paralysis was less clearly portrayed than it is to-day. 

I have stated that the anatomical findings in these cases apparently indi- 
cate normal conditions, and histological investigation reveals little of impor- 
tance. But it would be a great mistake if, for this reason, we were to con- 
sider these cases hysterical, as French authors have done. Striimpell, Ger- 
hardt, Frankl-Hochwart are probably correct in assuming that these cases 
are in a state of transition to diffuse sclerosis, which we must now discuss, 
and also consider it differentio-diagnostically as compared with multiple scle- 
rosis. In this affection large sclerotic areas develop in the cerebrum, even 
covering entire lobes, the brain substance shrinks and becomes tough; similar 
changes occur in relatively wide areas of the spinal cord. But here histologic 
investigation also sometimes leaves us in the dark by revealing little that is 
pathologic. Since there is also a slightly increased consistence in pseudo- 
sclerosis, we are, in a certain sense, justified in including these "cases of so-called 
pseudo-sclerosis with diffuse sclerosis. 

Cases of diffuse sclerosis occur in childhood and in youth; sometimes, 
however, only after the fortieth year of life. According to Frankl-Hochwart 
trauma plays a certain role in the etiology. Among the prominent symptoms 
of diffuse sclerosis are the following: Spastic paretic symptoms in the ex- 
tremities with contractures and increased tendon reflexes, tremor which does 
not closely resemble intention tremor but appears in peculiar muscular con- 
tractions, disturbances of speech, usually dysarthric, but sometimes resembling 
paralytic speech disturbances, lalling speech, etc. (Gerhardt is inclined to 
classify some of these cases with progressive paralysis.) Actual aphasic symp- 
toms have several times been observed. Moreover, there are changes in the 
optic nerve and paralysis of the muscles of the eyes, but, as a rule, no nystag- 
mus ; on the other hand, facial paralysis, disorders of the bladder and rectum, 
and sensory disturbances appear. Apoplectic and epileptiform attacks are 
common, spasmodic laughing and crying are noted, and psychical disturbances 



DIFFERENTIAL DIAGNOSIS 577 

which lead to actual dementia, The course is protracted throughout years, 
usually, however, without remissions, and steadily progressive. 

Here there is an unmistakable resemblance to the symptom-complex of 
multiple sclerosis, and this, according to Gerhardt, is particularly marked in 
children. In fact, the differential diagnosis is extremely difficult. The ab- 
sence of nystagmus, the prominent psychical disturbances, the severe dis- 
orders of speech, etc., may in some cases, but not in all, form a contrast to 
multiple sclerosis. 

In so-called pseudo-sclerosis and diffuse sclerosis we are dealing with rare 
affections which are therefore perhaps of less practical importance. We must 
now, however, differentiate multiple sclerosis from a number of common dis- 
eases. Starting from the fully developed picture of multiple sclerosis, we 
first consider progressive paralysis. In progressive paralysis there is a con- 
spicuous tremor which does not always resemble intention tremor, very com- 
monly there are also spastic paretic and ataxic symptoms, particularly in com- 
bination with tabes. Disturbances of the bladder, alterations in speech, changes 
in the optic nerve, paralysis of the muscles of the eye. apoplectic attacks with 
consequent paralytic phenomena, etc., are noted. On the other hand, dementia 
is usually and early prominent in progressive paralysis and increases to an 
extreme degree: as a rule, even in the early stages, other psychical disturb- 
ances are added (maniacal state or depression), while psychical disturbances 
are relatively rare in multiple sclerosis, and, if at all, usually occur late. The 
disturbances of speech in progressive paralysis are not of the same character 
as those occurring in multiple sclerosis. Optic atrophy, if present, resembles 
the tabetic form. There is reflex pupillary rigidity, a symptom not occurring 
in multiple sclerosis, but exceedingly common in progressive paralysis. The 
course of progressive paralysis is more rapid than that of multiple sclerosis. 
Frequently there is a history of syphilis so that, as a rule, a positive differ- 
ential diagnosis is possible. 

In childhood cases of apparent multiple sclerosis must be distinguished 
from the infinitely more numerous cases of cerebral diplegia, In the latter 
disease the symptoms are often congenital, or they appear very early. Epi- 
leptic attacks are frequent at the onset; as a rule, we find no nystagmus, no 
disturbances of speech, certainly no typical ones, no changes in the optic nerve, 
nor affections of the bladder, etc. The symptoms are usually stationary, or 
regressive rather than progressive. 

Acute disseminated encephalitis or encephalo-mi/rlitis. which must be com- 
pared with sub-acute cases of multiple sclerosis, develops acutely, usually with 
febrile symptoms, and subsequently shows remissions which, contrary to the 
condition in multiple sclerosis, are not. as a rule, succeeded by exacerbations. 
Xystagmus and affections of the optic nerve, certainly atrophy, are absent. 
Transitional stages in the clinical picture have already been mentioned. As 
multiple sclerosis may be apoplectiform in type, and leave more or less per- 
manent hemiplegia, we must also exclude the ordinary causes of such condi- 
tion, softening and hemorrhage. The latter, as a rule, occur in elderly persons 
or in the young who suffer from a disease of the heart, from nephritis or 
syphilis. As a rule, after the first symptoms have disappeared, the disease 
is stationary, except when renewed attacks occur. Tremor is usually on the 
hemi-paretic side. There is neither nystagmus, bladder disturbance, nor 



578 MULTIPLE SCLEROSIS 

change in the optic nerve, etc. Gerhardt calls attention to the fact that, 
under some circumstances, multiple softening may produce a clinical picture 
resembling that of multiple sclerosis in which advanced age, demonstrable 
arteriosclerosis or nephritis, a moderate degree of nystagmus, intention tremor, 
and marked prominence of bulbar symptoms (?) favor softening. 

Westphal and others have described cases in which multiple sclerosis was 
simulated by brain tumors; tumors of the cerebellum or the posterior cranial 
fossa may, under some circumstances, cause spastic paretic and ataxic symp- 
toms, as well as apoplectiform attacks, affections of the sight, changes in the 
optic nerve, nystagmus, and disturbances of speech. As a rule, however, the 
differential diagnosis is not difficult. But it must be borne in mind that there 
are cases, such as Bruns and Stolting described, in which multiple sclerosis 
appeared acutely with severe headache, vomiting, loss of consciousness, optic 
neuritis, and even choked disc, and we can readily understand that in this 
stage no differential diagnosis can be made. Only the subsequent course, the 
regression of the threatening symptoms, and the later appearance of other 
typical features, will disclose the nature of the case. 

The differential diagnosis of multiple sclerosis from cerebrospinal syphilis 
is of great importance particularly in regard to treatment. There may be a 
clinical similarity because in both maladies numerous disseminated foci may 
form in the brain and spinal cord. Among the common symptoms relating 
to the brain are disturbances in the optic nerve, apoplectiform attacks with 
ensuing paralysis, occasional disturbances of speech, spastic paretic phenomena 
in the extremities, derangements of the bladder and sensation. On the other 
hand, in multiple sclerosis pains in the back and vertebral column are usu- 
ally absent, and in syphilis there is neither nystagmus, intention tremor nor 
scanning speech. Spontaneous remissions occur in syphilis, but these are 
rarely of such long duration as in the case of multiple sclerosis. In doubtful 
cases the results of an antisyphilitic treatment will frequently, although not 
invariably, decide the case — for syphilis of the central nervous system may 
prove refractory under this form of treatment. 

Among the so-called neuroses we must first consider paralysis agitans; 
a certain similarity is at once evident; Charcot was the first to differentiate 
these affections positively by their clinical course. As a rule, their differences 
are greater than their points of resemblance, for paralysis agitans usually 
occurs in elderly persons, multiple sclerosis in the more youthful. The tremor 
of multiple sclerosis is intentional, that of paralysis agitans continuous. In 
the latter affection there are peculiar states of tension and contraction, but 
no true paretic and ataxic symptoms, no marked disturbances of speech and 
of the bladder, no intense sensory disturbances, above all, no changes in the 
optic nerve, no nystagmus, and no paralysis of the muscles of the eye. The 
course is also different : In paralysis agitans it is steadily progressive, in mul- 
tiple sclerosis there are acute exacerbations and succeeding remissions. Apo- 
plectic insults with succeeding paralysis are common in multiple sclerosis. In 
paralysis agitans, as a rule, the numerous attacks of vertigo and syncope leave 
no well marked paralysis. Of course, these distinguishing features may not 
always be so well developed. Tremor may be absent in multiple sclerosis, and 
in the early stages of paralysis agitans intention may distinctly increase the 
tremor. Paralysis agitans may occur in relatively young persons, multiple 



DIFFERENTIAL DIAGNOSIS 579 

sclerosis in the aged. Hence, in imperfectly developed cases of multiple scle- 
rosis there are certain, although by no means insurmountable, obstacles to the 
diagnosis. 

The differential diagnosis from hysteria may be very perplexing, and here 
grave errors have occasionally been made. Oppenheim warns us that begin- 
ning multiple sclerosis may readily be mistaken for hysteria. Vice versa, 
Irysteria may, under some circumstances, produce a picture resembling multi- 
ple sclerosis. In such cases there is tremor (often resembling intention 
tremor), spasm and paresis — of course, I refer here only to the so-called 
pseudo-spastic tremor after trauma — increase of tendon reflexes, ataxia or 
pseudo-ataxia with pseudo-Romberg sjinptoms, disturbances of sight and of 
sensation, diplopia, and even bladder disturbances. As a rule, a differen- 
tiation can be made; sensory disturbances are usually much more conspicu- 
ous in hysteria than in multiple sclerosis, often are unilateral, and the 
sensory functions are implicated. In hysterical disturbances of sight the 
concentric limitation of the field of vision is characteristic (in multiple 
sclerosis central scotomata or irregular contractions of the field of vision 
are predominant) ; diplopia is monocular, bladder disturbances are tran- 
sitory, etc. ; above all, in hysteria the demonstrable ophthalmic changes of 
the optic nerve as well as actual paralysis of the muscles of the eye, the 
nystagmus, the severe disturbances of speech, the bulbar symptoms and spastic 
phenomena are, as a rule, of different nature. Babinski's sign is usually 
absent. The appearance of typical, hysterical, spasmodic attacks or the peculiar 
psychical condition of many hysterical patients may clear up the case; but 
the possibility must always be borne in mind that multiple sclerosis may 
be combined with hysteria, in which ease the difficulty of diagnosis is 
multiplied. 

The formes frustes of multiple sclerosis which have been described may 
for other reasons give rise to differentio-dia,v;]i<»t ic problems. Without dis- 
cussing all the possibilities, a few important points may be emphasized. We 
have seen that multiple sclerosis may produce symptoms which are usually 
attributed to a transverse interruption of the spinal cord; therefore, spastic 
paresis of the lower extremities with sensory <li>turbances, affections of the 
bladder, etc. In such instances we must consider all of the affections which 
lead to transverse interruptions of the spinal cord, namely, caries of the ver- 
tebral column with compression of the spinal cord, syphilis of the spinal 
cord, chronic myelitis, tumors of the spinal cord, even syringomyelia. Mi- 
nute examination to determine whether other symptoms of multiple sclero- 
sis are present — for example, those of cerebral nature such as nystagmus, 
above all, an implication of the optic nerve or phenomena in the upper extrem- 
ities (intention tremor), or ataxia — will furnish data for the differential diag- 
nosis. The same is true when the symptoms of spastic spinal paralysis re- 
semble the clinical picture under discussion. Aside from the fact that the 
latter affection is rare, the diagnosis, as was shown by Oppenheim, Frank and 
others, can only be made by exclusion and the ophthalmoscopic finding, and 
sometimes the subsequent course of the disease will alone permit a decision. 
The same is true when multiple sclerosis appears under the guise of amyo- 
trophic lateral sclerosis. In spastic ataxic paresis, some forms of combined 
system disease will be considered; the presence or appearance of severe cere- 



580 MULTIPLE SCLEROSIS 

bral symptoms will be decisive. Other spinal processes, such as tabes, Fried- 
reich's disease, etc., need only be mentioned. They may, under some circum- 
stances, be considered, but, as a rule, they are readily excluded. 

PROGNOSIS 

Little remains to be said of the prognosis. Multiple sclerosis is a chronic, 
incurable malady. In isolated cases cures have been reported, for example, 
by Charcot and Oppenheim, but without doubting the reports of these experts, 
such cures are certainly rare. A cessation of the process, remarkable remis- 
sions lasting even for years certainly occur, and this not even infrequently, 
but, finally, new phenomena appear. 

TREATMENT 

In contrast with our perhaps too lengthy discussion of the pathology and 
diagnosis of multiple sclerosis, in the treatment of the disease we may be 
brief. So far as the disappearance of the symptoms or the complete arrest 
of the process is concerned, there is unfortunately no actual treatment. Nor 
can we speak, except to a very limited degree, of the prophylaxis of multiple 
sclerosis, especially in those cases in which it follows injuries or these favor 
its advance, for example, exposure to cold, trauma, excessive exertion, the 
puerperal state, intoxications, etc., all of which are preventable. 

Patients with severe paralytic sjanptoms, particularly acute exacerbations, 
should have prolonged rest in bed. In the latter case, mild antiphlogistic rem- 
edies (ice coils to the back, and preparations of the salicylates, etc., internally) 
may be tried. Among other remedies specially indicated in the early stages 
are mild hydriatic procedures, for example, lukewarm baths (32° C), per- 
haps with the addition of salt, or artificial carbonic acid baths; in the initial 
stage half baths (at a temperature of 31° C. and reduced to 29° or 28° C.) or 
moist packs. Indifferent baths, or salt and carbonic acid baths, are not in- 
jurious, but the temperature should never exceed 32° to 34° C. In addition 
to these electricity, galvanization of the spinal cord and the extremities, is 
to be emploj^ed and light massage may be carefully tried. In stationary 
conditions in which ataxic phenomena are predominant, exercises such as 
Frenkel employed in tabes may be practised. As we are dealing with a chronic 
affection lasting for years, these curative measures are not to be used continu- 
ously, but should be employed from time to time according to the urgency 
and intensity of the symptoms, at one time this remedy, at another that, 
or their combination and then cessation. Some drugs may be given inter- 
nally for a few weeks, for example, sodium iodid in moderate doses (1.5-2.0), 
which is given in all chronic spinal processes with appreciable or positive 
results; silver nitrate and ergotin, formerly much in vogue for the treat- 
ment of spinal processes, may be used but it must be with care; their effect 
is more than doubtful. Roborants may be tried, preparations of arsenic and 
quinin, nux vomica, etc., which sometimes favorably influence the general 
condition and, perhaps indirectly, the spinal process. In all of these methods 
of treatment we must bear in mind the very frequent spontaneous remissions. 
If one of these occurs during the progress of such treatment, it will naturally 



TREATMENT 581 

appear that the therapy is effective, but a new advance of the process will 
show the treatment to have been completely without result. In the terminal 
stages of the disease, when the patients are permanently bedridden and there 
is paralysis of the bladder, there is danger of cystitis and bed-sores, and the 
most careful nursing is necessary as well as all the precautions with which 
we are familiar in the treatment of other severe cerebral or spinal processes. 



SYRINGOMYELIA 
By FR. SCHULTZE, Bonn 

A patient, a young man, presented upon inspection a peculiar anomaly, marked 
emaciation of the muscles of the hand. All of the interossei muscles of the left hand 
and the hypothenar surface were flattened and there was also thenar atrophy. The 
same changes had taken place in the right hand, but to a less extent. 

There was a claw position of the fingers, this being more marked in the left than 
in the right hand. The terminal phalanges were abnormal, and were laterally dis- 
placed toward the mid-phalanges ; the nails were somewhat defective. In yarious areas 
of the skin were elevated cicatrices and indurations left by previous injuries. 

While the color of the hands was, in the main, normal or only slightly bluish 
( livid ) , the index finger of the right hand, in spite of the warmth of the lecture room, 
was ghastly pale. 

Upon examining the arms of the patient, a decrease in the musculature of the 
forearm, upper arm, and shoulders was noted. 

On testing the muscular power of the hand and fingers a more decided loss was 
observed. The fingers could not be fully extended, although some of them more so 
than others. Adduction and abduction were abnormal; perfect apposition of the thumb 
and little finger was impossible and more marked in the left than in the right hand. 

In this case a condition of muscle atrophy existed. According to the report of 
the patient, this had come on gradually, first attacking the left and then the right 
hand, and then involving the forearm. We have to consider that form of progressive 
muscular wasting which we designate progressive muscular atrophy. 

This euphonious title sounds diagnostic, yet it is not. Just as little as jaundice, 
dropsy, or fever prefigure a satisfactory diagnosis, so little does the designation " pro- 
gressive muscular atrophy " define the condition. 

In each individual case we must investigate the cause of the disease. It may be a 
primary affection of only the muscular fibers, a so-called primary myopathy, a primary 
muscular dystrophy, or a secondary change. 

These disturbances might at first be assumed to have been the result of chronic 
arthritic changes ; but in such conditions certain muscles pertaining to the joint affected 
are invariably diseased; for example, the muscles of the hands and fingers after disease 
of the knuckles. Secondly, they might have been the consequences of primary disease 
of the nervous apparatus. In this case we must consider the peripheral nervous sys- 
tem and the medulla spinalis including the root bundle. After changes in the brain 
this double atrophy of the muscles of the hand and arm is not observed. 

Under all circumstances, therefore, the entire nervous system must be investigated. 

In regard to sensation, we note that tactile sensation in the hands was by no means 
obliterated, but was decreased to such an extent that the touch of dull and sharp 
pointed instruments was no longer distinguished. This was most marked in the course 
of the left ulnar nerve. 

Much more conspicuous is the fact that in the hands sensibility to pain was nearly 
or wholly lost, as well as in the arms and the upper part of the trunk, and that it 
was markedly decreased in the neck. 

This was, as a rule, true also of the temperature sense, especially in regard to heat, 
which was almost absent in the skin of the hands, and decreased in the upper extremity, 
582 



SYRINGOMYELIA 



583 



in the trunk, and in the neck. Behind the ears and near the angle of the jaw cold was 
frequently felt as heat, and lower down thermic variations could not be differentiated. 

On palpation of the peripheral nerves which were susceptible to this method, and 
the brachial plexus, no thickening nor tumor could be discovered nor was there any 
abnormal sensation of pressure. 

The vertebral column was found to deviate markedly to the right. In the main 
there were no recognizable sensations upon inspection or palpation. The spinous proc- 
esses and their surroundings showed no abnormal sensitiveness to pressure. 




Fig. 174. — A Man, Aged 25, Suffering from Syringomyelia. 

Examination of the functions of the brain revealed no marked change. The sensory, 
the central, and the peripheral apparatus belonging to the individual cranial nerves 
functioned normally. 

The palpebral fissures were equally wide, the eyeball neither protruding nor too 
much sunken. Both pupils were somewhat contracted, the right less so than the 
left, but in refraction, accommodation and convergence they were normal. 

Except a mild, not distinctly pathologic nystagmus when the glance was directed 
to the right, no abnormal change was discovered in the eye-muscles. 

The motor portion of the trigeminal and facial nerves was intact. 

Sensatio)i in the fifth nerve, to delicate tests as well as upon mere contact, was not 
everywhere normal. Dull and pointed objects were commonly confounded; sharpness 



584 



SYRINGOMYELIA 



was distinctly appreciated only in the upper part of the forehead, being less acute in 

the lower trifacial branches. 

Deep pricking with a needle was described as painful, but not slighter pricks. 

Pricking through a fold of the skin below the chin was not felt at all. 

If a test-tube containing boiling water was held against the skin, pain was at once 

experienced above the orbital border, but not below this region; the patient felt it to 

be warm, and reported that the sensation of 
" heat " decreased in distinctness from 
above downward. 

Heat upon the mucous membrane of the 
lips, the mouth, and the tongue, also on 
the skin of the nose and on the eyelids was 
distinctly felt. This area of distinct sensa- 
tions was bounded by a line drawn from the 
external commissure of the eyelid to the 
alae of the nose. Upon the hairy scalp cold 
and heat were distinctly appreciated. 

In the distribution of the hypoglossal 
nerve no disturbance could be demonstrated, 
which is also true in general of the course 
of the pneumogastric. The right vocal cord 
could be adducted, but no longer abducted, 
hence there was a right-sided paralysis of 
the recurrent laryngeal, especially in the 
region of the posterior cricoarytenoid mus- 
cle. No abnormality was detected in the 
course of the spinal accessory nerve. 

Besides the changes in sensation men- 
tioned, examination of the spinal nerves 
showed that the muscular power of the 
lower arm as well as of the upper arm, the 
shoulders, the trunk, and lower extremities 
was almost normal. Electric examination 
of the muscles of the hand revealed com- 
plete DeR in both primary external inter- 
ossei. In the remaining interossei, and in 
the hypothenar eminences, there was a par- 
tial DeR. 

The reflexes were as follows: While the 
tendon and periosteal reflexes were absent 
in the arm and hand, they were active in 
the lower extremities but not pathologically 
increased. 

There was neither foot nor patellar 
clonus; on tapping the tibia there were no 
contractions of the quadriceps. The plantar 
reflexes were normal ; the reflexes of the 
abdominal walls were produced only by 
stroking the skin over the lower part of the 
abdomen. 

There were no disturbances of coordina- 
tion, tremor, or other motor irritative phe- 
nomena. The gait was unchanged. The 
bladder, the rectum, and the genital appa- 
ratus acted normally. 

In addition, a careful testing of sensa- 
tion in the trunk and extremities showed 
that the anterior line of thermo-anesthesia 

extended to the seventh rib in the mammillary line, while sensibility to heat increased 

to the navel, that is, hot water was felt as warm. 




Fig. 



175. — Syringomyelia 
(A man, aged 25). 



SYRINGOMYELIA 585 

Severe irritative pain from firm pinching was not felt above the eighth rib, but was 
evoked lower down. Still lower down sharpness and dulness could not be distinguished. 

There was complete analgesia in the hack from the middle of the neck down to the 
eleventh rib on the right, and to the tenth rib on the left side. 

Examination of the other organs of the body elicited no recognizable anomaly. 
With the exception of the finger- joints which were stiff, the joints as well as the bones, 
tendons, and ligaments were normal. 

This patient was of medium size, of strong bony frame, the muscles and fatty tissue 
showing a moderate degree of nutrition. The skin and mucous membranes were pale. 

There were no cicatrices upon the penis; a few cubital and axillary glands were 
somewhat larger than normal. 

The history showed that the parents of the patient died early, of unknown diseases, 
and that two of his brothers were healthy. Whether his birth was normal, he did not 
know. As a boy he was always well. In 1892, during an adventurous life in Dahomey 
with the French mercenaries, he suffered severely from fever with jaundice. In 1894, 
in Tongking, he had tearing pains in both arms with distinct muscular twitching, so 
that not infrequently his gun fell out of his hand. This peculiar twitching usually 
came on after he had been resting for some time. It lasted from two to three minutes, 
often awakened him from sleep, and was not accompanied by loss of consciousness. 
Tioo or three months later deformity of the fingers gradually appeared, and he noted 
that he could touch hot substances without experiencing inconvenience, but that blisters 
followed. He had to resign from the service, and returned to Europe in October, 1895. 

The deformity of the fingers steadily increased; and for nine months he was fre- 
quently affected by whitlow. 

The legs remained normal ; the patient could walk for hours without fatigue. No 
other disturbances than those mentioned were ever observed. 

In this patient we were not dealing with a progressive muscular atrophy 
alone, but there were simultaneously peculiar sensory and trophic disturb- 
ances, so that the nervous system was unquestionably affected. The stiffness 
in the joints was only to be regarded as an accompanying symptom. 

What was the nature of this nervous disturbance, and where its seat? 

Any one familiar with the symptomatology and etiology of multiple neu- 
ritis or polyneuritis, which this case resembles, would say that in this patient 
none of its known causes was operative. Neither alcoholism, lead poisoning, 
nor the effects of arsenic could be demonstrated. And it would be extremely 
arbitrary to assume refrigeration as a cause, or a rheumatic origin in the 
same sense as though an acute or chronic arthritis or muscular rheumatism had 
preceded or accompanied the disease. There had been no trauma, no syphilitic 
infection could be determined, nor had their been excessive use of the arm*. 

Above all, in -ordinary polyneuritis, no matter of what nature, the local- 
ization of the paralysis is different. In most cases the nerves of the lower 
extremities are simultaneously implicated, or even earlier and more markedly ; 
in lead paralysis the region of the radial nerve suffers most, but was here 
exempt. Notwithstanding the sensory disturbance in our case, there was no 
sensitiveness to pressure upon the peripheral nerve trunks, while in polyneu- 
ritis and other forms of neuritis there is none of the peculiar sensory disturb- 
ance which was here present. In polyneuritis the sensations conveyed by the 
individual cutaneous nerves are distributed much more uniformly. Finally, 
the limitation of the analgesic area and the zone of temperature sensation of 
the skin in peripheral neuritis is very different from that in the case described; 

Only one form of neuritis could be thought of, the variety occurring in 
leprosy, i. e., in that form of leprosy designated by the term nervous leprosy, 
because it particularly implicates the nerves and not the skin. 



586 SYRINGOMYELIA 

As our patient had lived in countries where this disease occurs he might 
easily have acquired it. 

Cutaneous leprosy was out of the question. This affection, as a rule, is 
first localized in the face, which in this patient was entirely devoid of the 
characteristic nodules and abnormal pigmentation. The eyebrows were per- 
fect, and there were none of those white and dark areas upon the skin of the 
trunk and extremities which are so typical of leprosy, as well as circumscribed 
anesthesia in certain areas. 

Perhaps the state of the hands resembled leprosy, since in leprous neuritis 
there is an atrophy of the muscles, and trophic disturbances and vasomotor 
changes appear, even the dissociated sensory paralyses which we detected in 
the patient under consideration and which were formerly called partial sen- 
sory paralyses may occur. 

But there was no swelling of individual portions of the peripheral nerves 
such as occurs in leprosy, particularly of the ulnar nerve in the vicinity of the 
olecranon which may be felt in this area. Sometimes, I must admit, the 
diagnosis of such a thickening is very perplexing; it may, perhaps, be the 
result of chronic inflammation and ulceration in the hands and forearms with- 
out leprosy. In this case, however, no such change was present. 

Concerning other symptoms it must be stated that unilateral paralysis of 
the recurrent laryngeal nerve without implication of the skin of the face and 
of the other nerves of the head does not occur in leprosy except when other 
causes accidentally produce paralysis of the larynx. Moreover, contractures 
of the muscles of the arm such as occurred in our patient is rarely observed 
in leprosy. Finally, the bacillus of leprosy was not found in the nasal mucus, 
and, according to recent investigations, this microorganism is frequently, if 
not invariably, present in leprosy. 

Taken all in all, no diagnosis of leprosy could be made in this ease, and 
as there were no reasons for assuming a bilateral plexus paralysis, the question 
arose, What form of spinal disease is here present? 

The peripheral nerve fibers and nerve bundles extending to the spinal cord 
pass through the intervertebral spaces and the membranes of the spinal cord 
before they reach the medulla spinalis. Therefore, diseases of the cervical 
vertebrae and the ligamentous apparatus belonging thereto, as well as diseases 
of the membranes, may exert pressure upon the passing roots or cause inflam- 
mation therein. 

In this patient there were none of the symptoms which appear in chronic 
and progressive diseases of this nature. 

Inflammation and neoplasms of the vertebrae and the membranes of the 
spinal cord, even in the beginning, usually produce local and radiating pain, 
and stiffness of the neck, with difficulty in moving it. Although the pain is 
not always severe it is invariably present in those cases in which anesthesia 
subsequently appears. In our patient, there was no pain, nor was there rigidity 
and inhibition of motion in the cervical vertebral column, therefore we were 
forced to consider whether the white substance of the spinal cord was diseased, 
and whether chronic myelitis of the cervical enlargement might be present, 
since, according to experience, in these affections the white substance usually 
is implicated. 

iSince, however, these are accompanied by changes in the lower extremities, 



SYRINGOMYELIA 587 

particularly spastic paresis as well as changes in the function of the bladder, 
and as these were absent in our patient, no alternative was left us but to 
regard the central gray substance as the seat of the disease. Isolated chronic 
inflammation never occurs in the anterior and posterior portions of this struc- 
ture without the formation of cavities — that is, it has never yet been demon- 
strated; and since even tumors rarely form in this substance without pro- 
ducing cavities, we were certainly dealing with a chronic, progressive, and 
extensive cavity formation with destructive processes ; briefly, with progressive 
syringomyelia, or with a central tumor formation which did not produce pres- 
sure upon the white substance from within. 

"While refraining from an immediate differentiation of these two pathologic 
conditions, I wish to state that syringomyelia is by no means a rare affection 
of the spinal cord. 

We must now discuss the nature of this peculiar spinal change, and we 
will most readily understand the clinical symptoms if we begin with the 
pathological anatomy, and then utilize our knowledge of physiology in con- 
sidering the functions of the spinal cord. 

The name "syringomyelia " ( i. e., a flute-like spinal cord, from fj avptvy^, 
the flute) was applied to this affection by a French physician, Ollivier, who 
wrote a monograph upon diseases of the spinal cord in the early part of the 
preceding century. In fact we not rarely find throughout the entire extent 
of the medulla spinalis a space in which a penholder or lead-pencil might 
easily be introduced, and which contains a watery fluid. 

Such a curious and conspicuous change was of course observed long before 
the time of Ollivier, apparently first by Etienne at the end of the sixteenth 
century, later by Morgagni, Portal, Andral, Xonat and others. But prior to 
about the seventh decade of the last century, these eases were regarded as mere 
curiosities. 

In explaining its occurrence, writers of about the year 1800 assumed the 
condition to be due to an abnormal development of the central canal which 
in children, as is well known, extends the entire length of the spinal cord 
from above downward, while in later life it is partially or wholly occluded 
or even obliterated. 

It is quite conceivable that this canal might, under some circumstances, 
remain open; if, for instance, abnormal dilatation of the cerebral ventricles 
and the consequent pressure of the fluid they contain dilates the central canal 
in the early stages of its development. This canal communicates directly 
with the fourth ventricle. 

Undoubtedly there is such a hydromyelia, but it is usually a stationary 
condition which does not produce progressive clinical symptoms. It is uncer- 
tain whether or not, from this abnormal condition, increasingly destructive 
processes may at the same time develop and, like ordinary progressive syringo- 
myelia, destroy the anterior and posterior gray substance and also the white. 

If we examine transverse sections of a syringomyelic spinal cord we at 
once observe that, particularly in the cervical portion, there is a great but 
varying loss of substance, chiefly of the gray. Throughout the whole of the 
posterior horns and the commissures there are empty spaces which separate 
the entire anterior and lateral portions of the spinal cord substance from the 
posterior columns. Minute microscopic investigation sometimes reveals around 



588 



SYRINGOMYELIA 



these spaces a thin layer of glia fibers, in other cases simultaneously such a 
great accumulation of glia cells are produced that they cause displacement 
phenomena (so-called gliosis or primary gliosis) or an extensive tumor forma- 
tion (gliomatosis, gliomata). 

The central canal is often situated in front of the cavities and spaces 
although commonly filled with those ependymal cells which are quite fre- 
quently found in the normal cord of adults. 

Therefore, in such cases we can exclude simple dilatation of a normally 
situated central canal in the walls and surroundings of which destructive and 
proliferative processes have been generated, and other morbid states must be 
considered. 

In the first place minute anatomical investigations have demonstrated that 
several central canals may develop and be situated behind each other, and that 
consequently an extensive diverticulum of the central canal may be formed 
similar to that observed in the esophagus. 

A number of cases make it seem extremely likely that syringomyelitic 
processes may readily develop from such diverticula and auxiliary cavities. In 




Fig. 176.— Syringomyelia of the Cervical Enlargement. The white substance is stained 
black by Weigert's stain. The entire gray substance is changed, destroyed, or permeated 
with cavities. Even in the posterior horn, only a part of its contour is normally retained. 

other instances no such anomalies can be found, and we must assume that 
syringomyelia may develop also in a normal spinal cord. Nevertheless, we 
must finally inquire how these anomalies of development arise, and whether 
in the growing cord, at birth or later, other disturbances may not naturally 
develop which finally lead to progressive syringomyelia. The cavities and 
spaces are not necessarily the primary condition but may arise secondarily. 
Thus it is possible that tumors of a gliomatous nature may develop from the 
ependyma or from other parts of the glia substance and gradually break up 
in the center, thus causing these secondary cavities sooner or later to com- 



SYRINGOMYELIA 



589 



municate with the normal or abnormally situated central canal. Moreover, 
it has been proven that chronic pachymeningitic and Jeptumeningitic processes 
may here and there occur, and be combined with the formation of an elongated 
central cavity. We know, too, that hemorrhages into the spinal cord, such as 
appear after trauma, are most often found in those areas in which the cavities 






<%< 





Fig. 177. — Transverse Section throi'gh the 
Dorsal Portion of the Spinal Cord. Tu- 
mor-like accumulations of glia around a cen- 
tral cavity (gliosis). 



Fig. 178. — Transverse Section through the 
Dorsal Portion of the Spinal Cord (from 
the same case as Fig. 177). There is no cen- 
tral cavity, but only rarefaction of the great- 
ly proliferated central glia masses. 



of syringomyelia are localized. The interesting fact has been observed that 
in infants whose bodies had been subjected to marked pressure and com- 
pression because of hard labor circumscribed hemorrhages were found in 
the spinal cord and the medulla oblongata in the same areas in which cavities 
form. 

It is noteworthy, too, that the blood-vessels in the surroundings of the 
cavities are often greatly thickened, and obliteration as well as thrombosis may 
appear. 

We may, therefore, conclude 
that in some cases elongated tu- 
mors first appear followed by cen- 
tral destruction, that in others 
hemorrhages first occur and sub- 
sequently, as in ordinary cerebral 
hemorrhage, cavities and cysts 
form. We must still explain how 
progressive syringomyelia develops 
from these cavities. Here we en- 
counter the same difficulty as in 
the case of hydromyelia and the 
other anomalies of development we 
have named. 

In other cases, acute or chronic 
inflammatory processes may be 

combined with necrosis and loss of substance so that a " myelite cavitaire" as 
some French authors designate it, develops. These cavities are not found in the 
usual acute and chronic forms of myelitis even when progressive processes are 
present, as in the case of multiple sclerosis. Except in the pachymeningitic 




Fig. 179. — Transverse Section of a Dorsal Por- 
tion of the Lumbar Cord (from the same case 
as Fig. 177). A larger central cavity with a 
marked glia ring. 



590 SYRINGOMYELIA 

processes just mentioned, elongated cavities do not form in syphilitic affec- 
tions, although I observed a case of this kind in which a moderately extensive 
syringomyelia was present as well as chronic myelitic changes in the lumbar 
cord without pachymeningitis. 

If in such cases we assume thrombotic changes or vascular obliteration 
from a non-inflammatory cause, the increased length of the canals and spaces 
is difficult to explain, since the arteries and veins which penetrate the gray 
substance have only a short course and we must therefore assume that numer- 
ous adjacent areas, without exempting isolated diseased ones, have been suc- 
cessively implicated. Acute poliomyelitis is often widely distributed so that 
here also the involvement of many areas takes place; but, notwithstanding 
great vascular changes no cavities are formed. 

When we state, in addition, that in certain cases of brain tumor or com- 
pression of the spinal cord pressure from the central canal was regarded as 
the cause of cavity formation, it is evident that every effort has been made 
to explain the development of these processes. 

It is, however, apparent from the very complete and accurate description 
of these conditions that we are not at present justified in assuming for syringo- 
myelia a uniform mode of development. Congenital anomalies of develop- 
ment, either disturbances occurring at birth or in the course of extra-uterine 
life, here play a role. Tumors, hemorrhage, and necrosis, inflammatory and 
degenerative processes, as well as peculiar, localized, vascular changes, must be 
considered. 

All of these have the common property of localization in the central por- 
tion of the spinal cord and a tendency to progression. 

The latter condition is most difficult to understand. In the case of tumors 
of great or less extent, therefore in gliomatosis, in formation of glioma, or in 
primary central gliosis, advancing growth is at this time as impossible to 
explain as that of tumors in general. If it be due to inflammatory or degen- 
erative processes, the understanding of its tendency to progress as well as 
many other advancing pathologic conditions in the central nervous system and 
in other organs is lacking. 

It is remarkable that these by no means invariably accompany arterioscle- 
rotic processes in other organs, or even in the brain. Therefore, they are not 
typical of a disease of advancing age, as is arteriosclerosis, but they usually 
develop in youth; hence early disturbances in the formation of the spinal 
cord, and injuries occurring prior to this, must also be considered. 

The conclusion is obvious that in many cases in which the first signs 
of syringomyelia appear during the course of infectious disease, for example, 
enteric fever, the microorganisms or toxins which are generated in such affec- 
tions accumulate in abnormal diverticula of the central canal, and thence set 
up pathologic processes. 

Whether this be true or not, the condition and localization of the syringo- 
myelitic changes which have been described may be deduced from other well 
known physiologic facts and many of the symptoms which necessarily arise 
in this affection. 

Before attempting this, I must state that, besides affecting the gray sub- 
stance and the posterior columns, the proliferative and destructive processes 
going on around the spaces and cavities may also implicate the lateral columns, 



SYRINGOMYELIA 



591 



Accumulation of glia 




Pyramids 



Olives 



Fig. ISO. — Transverse Section through the Medulla 
Oblongata in a Case of Syringobulbia. (After A. 
Westphal.) 



for they are either exposed to a marked lateral pressure from the proliferated 
glia masses or are directly implicated in the chronic degeneration. 

Frequently the entire spinal cord is permeated by cavities and spaces; 
sometimes, however, the cervical and dorsal portions alone may be implicated, 
while syringomyelia limited 
solely to the lumbar por- 
tion is very rare. Usually 
the segments of the cer- 
vical enlargement, particu- 
larly the lower ones, are 
most markedly affected. 

It is significant that 
not rarely the medulla ob- 
longata also is implicated 
in this peculiar cavity for- 
mation, that, therefore, a 
syringobulbia develops. 
This anomaly is usually 
unilateral at the height of 
the fourth ventricle in the 
region of the nucleus of the 
pneumogastric, and thence 
passes anteriorly and lat- 
erally. In other cases the 
destruction is bilateral but asymmetric. The destruction terminates on the 
floor of the fourth ventricle above the nucleus of the vagus and the striae 
acoustics; its localization may be clearly understood from the illustrations 

appended to this article. 
GHa streak It is remarkable that 

in syringobulbia the 
spaces and cavities within 
the bulbus of the medulla 
oblongata are usually 
surrounded only by scant 
accumulations of glia, 
and the vessels show, as 
a rule, unusually thick- 
ened walls. 

If we recall our phys- 
iologic knowledge of the 
function of the spinal 
cord, these anatomical 
findings furnish a sim- 
ple explanation of many 
of the symptoms of 
syringomyelia. 

The gradual disappearance of the large motor ganglion cells in the anterior 
gray substance causes the disintegration and, finally, the destruction of their 
nerve fibers and muscle bundles. As the lower portion of the cervical en- 




Pyramids Olives 

Fig. 181. — Syringobulbia. (After A. Westphal.) 



592 SYRINGOMYELIA 

largement is frequently first attacked, the muscles of the hand atrophy first, 
since the ganglion cells which innervate these are situated in this region. 

As, moreover, so far as we at present know, the motor ganglion cells quite 
irregularly, each for itself but by no means symmetrically, atrophy in groups 
which belong functionally together, it is to be expected that in the larger mus- 
cles isolated bundles may be alone diseased, and in fact this is not rarely the 
case. 

The destruction of the posterior gray substance inhibits the conduction of 
the sense of pain, as was shown by the well known experiments of Moritz Schiff 
in animals. The conduction of the sensation of touch, which often remains 
intact for a comparatively long time, is explained by the circumstance that 
the majority of the fibers of the posterior column frequently remain exempt, 
and to these fibers the conduction of tactile sense is chiefly ascribed. 

If these are markedly implicated from the onset, the consequent disturb- 
ances keep pace with the limitation of other sensory functions; partial sen- 
sory paralysis is absent. 

We are still ignorant as to the areas of the transverse section in which the 
nerve tracts which conduct temperature sensations are situated. 

Trophic disturbances are usually attributed to destruction of definite areas 
of the transverse portion of the spinal cord. 

Diseases of the gray substance must be considered the cause of these, 
although perhaps not a direct one. At all events, we know that such sequelae 
do not occur, as a rule, in chronic myelitic processes which chiefly affect the 
white substance. Neither is the gray substance of the posterior horns normal 
in tabes dorsalis, in which similar changes occur. 

If the disease in any way attacks the lateral columns, symptoms referable 
to the lateral column appear; above all, spastic paresis or paralysis of the 
extremities, especially the legs. 

If the nuclei of the pneumogastric or hypoglossal nerves, or the fibers 
belonging to them in the bulb of the medulla oblongata, are attacked, derange- 
ments of deglutition, of the innervation of the larynx, and of cardiac and respi- 
ratory functions are produced. If the cavity formation in the gray substance 
extends as far as the ascending trigeminal roots, the same sensory disturbances 
occur in the area of distribution of this nerve as in those of the extremities. 

If there is an extensive tumor formation, a gliomatosis, of course more or 
less decided pressure symptoms may result. 

SYMPTOMATOLOGY 

The foregoing enables us to understand that, owing to the irregular and 
varying distribution of the syringomyelitic processes, the grouping of the 
symptoms of this disease must necessarily vary. 

Some typical forms may be differentiated. First, the most common, the 
so-called classical form, a case of which I have described. 

In this variety, the symptoms of gradually increasing muscular atrophy, 
paralysis, and sensory disturbances, usually with partial sensory paralysis, are 
combined with trophic disturbances. 

As subdivisions and special types we observe first a cervical form, as in 
the patient whose history I quoted, next, if syringobulbia develop, a bulbo- 



SYMPTOMATOLOGY 593 

medullary type, finally, the much more rare " dorso-lumbar" and "sacrolum- 
bal* " types. 

In some cases the motor symptoms may be so prominent as to form the 
picture of chronic progressive muscular atrophy or of so-called amyotrophic 
lateral sclerosis. 

In other cases sensory disturbances predominate, and there is either exten- 
sive analgesia, even resembling hysteria, or, at the same time, irritative symp- 
toms develop like those of tabes dorsalis. 

Finally, there are forms in which marked trophic disturbances appear. 
If whitlows and phlegmons develop, particularly upon the upper extremities, 
with analgesia, we have before us the so-called " Morvan's disease." 

Deformation, if prominent, produces the mutilating form which bears 
a great resemblance to lepra mutilans. 

Osseous and arthritic changes lead to the variety which Schlesinger calls 
the osteo-arthritic form ; this sometimes causes a change in the hands and 
forearms similar to that in acromegalia. But the most common form is that 
in which progressive muscular atrophy of the hands and arms is combined 
with sensory disturbances and bulbar symptoms, as in the case which was 
detailedi 

In all of these forms we usually find a variety of symptoms, and these we 
will now consider. 

Beginning with motor disturbances, we note that the irritative symptoms 
of this disease are usually not at all prominent; certainly they more readily 
escape observation than debility and paralysis. Fibrillary muscular contrac- 
tions are frequent, even with a sufficiently warm external temperature and 
during rest ; contractions which also occur in other slowly progressive dis- 
eases of the great motor ganglion cells. 

Tremor may occur, particularly in the hands, and. if increased by voluntary 
movement, as is usually the case, it resembles the tremor in multiple sclerosis. 

More rare are choreic contractions which also appear during rest, and are 
chiefly confined to the upper extremities. Sometimes, although rarely, they 
are observed in muscles supplied by the bulbar nerves. I shall never forget 
a patient with this symptom whom I first saw in Friedreich's Clinic in Heidel- 
berg. He held a handkerchief in his mouth continually, and only with the 
greatest difficulty could be persuaded to remove it. When he did so, against 
his will his tongue protruded, it trembled violently, and painful jerkings 
of the lower jaw pressed it against the upper teeth so that it was bitten. In 
order to prevent this, the patient had had all his teeth extracted. There were 
also irregular clonic and tonic contractions in the trapezius, and subsequently 
also in other muscles of the shoulder. 

Since such contractions are only occasionally present we are forced to 
assume an especial predisposition of the patient to irritative conditions of this 
kind, but a certain relation to syringomyelia and syringobulbia present can 
scarcely be denied. 

Tonic spasms may be persistent or occur in acute attacks. Contrary to 
the clonic spasms, they are much more common in the lower extremities: in 
rare cases they are distributed throughout the body, so that general rigidity 
and opisthotonos may appear. Larvngospasms also occur and mav threaten 
life. 

39 



594 SYRINGOMYELIA 

It is self-evident that with spastic paresis in the legs we frequently find 
also a permanent rigidity of the corresponding muscles. 

Attention has only recently been called to peculiar myotonic disturbances 
occasionally found in syringomyelia. These fail to present all of the charac- 
teristics of true Thomsen's disease, myotonia congenita, particularly the 
simultaneous disturbance of almost all of the muscles of the body. But in 
some cases we note the same long-continued rigidity of individual muscles to 
the effect of cold as in the last named disease ; also the same inability to relax 
the muscles which have been voluntarily contracted, particularly after a pre- 
ceding long rest. We find the same peculiar and persistent pitting of the 
muscles after palpation which occurs in Thomsen's disease, and to some extent 
even the peculiar myotonic electrical reaction, which I cannot here describe. 
But these disturbances are found only in those muscles whose activity has 
been altered by the syringomyelitic process. 

The motor symptoms of weakness and paralysis, which occur with and 
without muscular atrophy, are of much more significance in the clinical picture. 

They usually develop slowly, particularly in the hands, and the appearance 
of weakness and atrophy is coincident. Sometimes, however, particularly in 
the legs, and when there is softening of the spinal cord substance following 
thrombosis, vascular constrictions, or hemorrhages, paralysis may come on 
very suddenly. This paresis of the arms is usually flaccid, of the legs spastic, 
except when associated with lumbar syringomyelia and destruction of the gray 
anterior horns in the lumbar enlargement. Spastic contractures of the mus- 
cles of the arm, the leg and the trunk rarely exist simultaneously. 

The localization of the atrophy and paralysis varies greatly with the differ- 
ing localization of the spinal changes. Muscular atrophy with debility is usu- 
ally first noted in the small muscles of the hand, and shows a special predi- 
lection for the interossei primi externi. This, however, is generally by no 
means symmetrical. 

If the ulnar region is first implicated, the claw-hand develops; if the 
median tract, which is more rarely the case, the ape's hand. When the vari- 
ous muscles are simultaneously implicated the disturbances are, as a rule, 
simultaneous. If, subsequently, the flexor muscles of the forearm become atro- 
phic, the so-called preacher s hand may develop, and the claw-hand, on account 
of the preponderance of the extensors, is at the same time flexed dorsally. 
Usually, however, because of the atrophic weakness of the forearm, the ex- 
tensor muscles are also implicated, or the atrophy, passing by the muscles of 
the arm, leaps from the hands to the muscles of the shoulder. 

In other cases the disease is from the onset confined to the muscles of the 
shoulder, thence it passes to the muscles of the upper extremities and to the 
trunk. Here the muscle in its entirety is not always attacked, but it is often 
only partially involved, and most frequently the extensive area of the trapezius. 
This leaping of the disease from one muscle to another and its asymmetry are 
almost characteristic. 

Atrophy of the muscles of the leg is rarer, and there is no definite rule for 
the development and progress of the disease. Pes equino-varus and pes cal- 
caneus have been observed, but here the localization of the atrophy was very 
different and it involved also the muscles of the hip. 

Hence it is evident that the most varied disturbances in the function of 



SYMPTOMATOLOGY 595 

individual members may arise, but these cannot here be detailed. It can only 
be stated that as spastic paresis is most common in the leg, so the gait typical 
of this condition is most frequently observed, while the ataxic, even the stag- 
gering, gait is more rare. Distinct ataxia has rarely been observed in the arms 
and hands. The disordered gait usually develops gradually; often very early 
on one side. Sometimes, however, disturbances in gait are sudden or quickly 
increase in severity. 

The atrophies and paralyses in the course of the bulbar nerves are very 
interesting. Earely is there paralysis of the motor branch of the trigeminal 
nerve on one side only. Paralysis of the muscles of the eyes, especially of the 
abducens, is uncommon, but more frequent is paralysis of the facial nerve, 
which is almost always unilateral, and generally affects only the branches 
supplying the mouth, although the upper branches also may be implicated. 
Like paralysis of the legs this usually develops slowly, yet may sometimes 
come on rapidly; the electric contractility of the muscles supplied by the 
nerve shows the same changes as that of the muscles of the hand. 

Hemiatrophy of the tongue, in which one-half of the tongue becomes so 
thin that grooves develop, is more common. The fibrillary contractions are 
usually well developed. The absence of the power of motion, as a rule, causes 
the patient no great inconvenience. 

Often there is unilateral paralysis of the muscles of the palate and larynx. 
Deglutition then becomes so difficult that it is almost impossible to swallow 
a bolus, and ingested foods are regurgitated through the nose. Examination 
of the velum of the palate discloses the defective elevation of one-half upon 
phonation, also the retraction of the uvula to the healthy side and fibrillary 
contractions. 

In the larynx we can determine only unilateral paralysis of the recurrent 
laryngeal nerve, which at first involves merely the abductors of the vocal cord, 
but finally becomes complete. Sometimes the paralyzed vocal cord is decidedly 
atrophic, and the voice becomes rougher and hoarser. Paralysis of the vocal 
cord may come on very gradually, or it may occur suddenly and rapidly 
become worse. 

In rare cases the paralysis is bilateral. Unilateral paralysis is often com- 
bined with paralysis of the palate and esophagus of the same side; if there 
is simultaneously hemiatrophy of the tongue, this is also found upon the same 
side; therefore we have the picture of a unilateral lower bulbar paralysis. In 
such cases the voice is not only hoarse but has a nasal tone, and speech may 
be difficult; now and then it is slow and scanning as in multiple sclerosis, a 
symptom the cause of which is still unknown. 

It is not remarkable that, under such circumstances, cardiac activity 
should sometimes be disturbed by the changes in the nucleus of the pneumo- 
gastric nerve. Tachycardia has not infrequently been observed, more rarely 
bradycardia. Dyspnea may follow these disturbances, which in different cases 
must be ascribed to various causes. 

Dyspnea may arise from paralysis of the posterior cricoarytenoid muscles 
and may readily be recognized by the accompanying stridor; it may be due 
to increasing atrophy of the muscles of inspiration and expiration, and is 
then steadily progressive and increased by the slightest exertion. It may occur 
paroxysmally without any changes of this kind, owing to sudden paralysis of 



596 SYRINGOMYELIA 

the respiratory muscles. In this case, if syringobulbia soon becomes marked, 
an early and fatal termination may be looked for. 

The results of electric examination of the nerves and muscles vary. With 
a slow progress and prolonged duration of atrophy in the muscles, direct and 
indirect irritability is more or less diminished, and DeE rarely occurs, most 
rarely that complete form associated with rapid atrophy. This is partly due 
to the fact that the atrophied muscle masses frequently contain some muscle 
fibers which still react well. 

Mechanical irritability corresponds with this ; upon tapping the muscles 
we rarely note distinctly sluggish contractions. 

The sensory disturbances are of especial significance. 

Here irritative phenomena are less important than paralytic symptoms; 
nevertheless, they are very prominent. Especially in regard to the sense of 
temperature, paresthesias appear in the form of burning, of hot, and of cold 
sensations. Ordinary tactile paresthesias appear to be rare, which corresponds 
with the fact that paralytic symptoms also are chiefly confined to the realm 
of temperature sensations and are less often found in the tactile. 

Pain may be of a tearing and boring as well as of a burning character. It 
is rarely so marked as to cause suffering. Usually it is so persistent that 
rheumatism is simulated. In some cases, however, pain is of the same lanci- 
nating character as in tabes. Here and there a girdle sensation is com- 
plained of. 

The seat of the paresthesia and pain corresponds with the extent of the 
spinal and bulbar substance implicated. These symptoms are most frequently 
found in the arms and the upper parts of the trunk, more rarely in the legs 
and the region of the fifth nerve, sometimes even in the occipital region. 
They may appear quite early as well as late, and they may be combined for 
a' long time with other symptoms of the disease. 

The peculiar, sensory, paralytic symptoms are much more constant; the 
discovery of these about 1880, together with the atrophy, first made the diag- 
nosis of syringomyelia possible. 

Partial sensory paralysis or dissociated " syringomyelitic " sensory paral- 
ysis, as this condition has been called is often noted, although it occurs also 
in other diseases of the nervous system. This was present in my patient. 
While tactile sense is normal or shows only slight impairment, pain and tem- 
perature sense are markedly decreased. It is erroneous, however, to assume 
that the tactile sense is normal throughout the entire course of the disease. 
Sometimes it is but slightly impaired, and it is wholly absent in at most only 
a relatively small part of the cutaneous surface. 

The sense of pain, corresponding with the anatomical seat of disturbance, 
is most frequently diminished or absent in the hands and arms, in the upper 
portion of the trunk, in the skin as well as in the deeper areas, and in the 
joints and bones, although neither simultaneously nor symmetrically. 

If syringobulbia appears concurrently, the scalp and mucous membranes 
of the head are implicated. Analgesia or hypalgesia in the legs is rare. The 
specific pressure pain in the testicle may disappear while, as Schlesinger has 
demonstrated, pain is almost invariably evoked by pressure over the eye-ball. 

The time of development of hypalgesia and analgesia conforms to no -rule. 
It usually appears in the early stages of the affection, and generally is coin- 



SYMPTOMATOLOGY 



597 



cident with the implication of the temperature sense and the development of 
muscular atrophy. 

The study of the disturbance of the sense of pain and its distribution, as 
undertaken in the last few years by Laehr, Schlesinger, Brissaud and others, 
is highly interesting. Although these investigations have not yet been con- 
cluded, it is evident that very different areas are involved. 

It might be supposed that these would correspond with the areas impli- 
cated in diseases of the peripheral nerves. But this is not the case. On the 
contrary, the analgesic areas are the same as those frequently found in dis- 
eases of the posterior nerve roots and the spinal cord segments belonging 
thereto, and consequently they are of a segmental type. 

The localization of the hypalgesic and analgesic areas is as follows : They 
appear on the trunk as a series of transverse zones, extending horizontally and 
parallel with each other to the median line, while on the extremities they are 
noted as bands which extend continuously upward and parallel with the longi- 
tudinal axis of the limbs until they reach the trunk, passing thence to the 
median line. Here and there the analgesic bands assume a spiral form. 

In the face and on the head the hypalgesic zones are of peculiar appear- 
ance. If higher segments of the cervical portion of the cord be affected the 
limitations are invariably found to be higher, usually as irregular arches with 
the concavity toward the front. As a 
rule, the upper half is parallel with the 
edges of the hair, and the lower with 
the border of the lower jaw, so that an- 
teriorly the nose and its immediate sur- 
roundings generally remain uninvolved. 

This is graphically portrayed by the 
diagrams here inserted. 

Secondly, a rare form of hypalgesic 
and analgesic disturbance is that in 
which the zones correspond with the 
affection of individual members or their 
parts, in which therefore the hands 
and arms are chiefly and uniformly 
attacked, and the zones disappear while 
passing upward spirally. On the trunk 
sensory defects may appear in " waist- 
coat-like" forms. If the whole trunk 
has become analgesic a shirt-like anal- 
gesia is produced, or, more correctly, a 
jacket-like analgesia, since the limit is 
usually not so low down. 

These zones of analgesia correspond 
with those found in hysteria, but they 
differ by their greater persistence; in 
syringomyelia the intensity of the 

hypalgesia may also vary. But other symptoms of hysteria are absent. Of 
course, in some of the cases hysteria may be associated with syringomyelia as 
with other organic diseases. 




Fig. 182. — The Various Limits of the 
Zones of Sensory Disturbance upon 
the Head. (After v. Soelder and Schles- 
inger.) 



598 



SYRINGOMYELIA 



How this member-like limited analgesia may be derived from anatomical 
changes is still not clear, in spite of ingenious hypotheses. But, inversely, it 
seems to throw some light upon hysterical analgesia and its nature, a point 
which I cannot here discuss. 

Thirdly, when there are extensive changes in the posterior gray substance 
and the long sensory tracts, a sensory paralysis of the Brown- Sequard type 
may exist, also a bilateral form involving the entire trunk and the legs, as 
well as the segmental type in the arms, perhaps also in the face. In the 
Brown- Sequard variety, besides the partial sensory paralysis which is usually 
very conspicuous upon one side of the body, there is simultaneously a marked 
motor paralysis upon the other side. 

The mucous membranes most frequently affected are those of the pharynx 
and oral cavity, especially their posterior portions; more rarely the conjunc- 
tiva as well as the bladder, urethra and rectum. Analgesia may reach its 
highest degree in any of these areas. 

It has been stated that these disturbances may vary in intensity. In com- 
plete analgesia they are, however, rarely observed and are not of a decided 
nature. 

The localization of derangement of the temperature sense resembles that 
of the pain sense, although its distribution and degree do not exactly corre- 




Fiq. 183. — Disturbance of the Pain Sense, Segmentaely Limited. (After Schlesinger 
and Hahn). The darkest areas correspond to the zones of complete analgesia, the lighter 
to varying but slighter degrees of disturbance. 



spond. Like disturbance of pain sense, this may occur early and rapidly lead 
to injuries, particularly to burns. 

The temperature sense may be diminished in any degree, and this disturb- 
ance may vary in that the sense for heat or for cold only may be impaired, 
or that the sensations may vary in degree of impairment. There may be a 



SYMPTOMATOLOGY 599 

variation in the rapidity of conduction of impressions for heat and cold as 
well as an inversion of these sensations, so that heat is mistaken for cold and 
vice versa, or cold and heat are appreciated only as coolness and warmth. A 
delayed temperature sense has been observed in rare cases. 

The disturbance of temperature sense is almost invariably combined with 
disturbance of the sense of pain; but it may occur earlier, and in some cases 
is said to be the only permanent defect in sensation. 

The mucous membranes and the deeper areas may be implicated just as in 
the disturbance of sense of pain. 

Much less important is disturbance of other kinds of sensibility; for in- 
stance, anomalies of the so-called muscular sense and sense of position. 

In the lower extremities the sensation for active and passive movements, 
for weight and resistance, may be more or less impaired, owing to the fact 
that localized changes gradually take place in the posterior columns in syringo- 
myelia just as in ordinary tabes, or because an actual combination of these 
diseases exists. Ataxia may then be observed which, unlike that in tabes, 
sometimes affects one leg only. It may, however, occasionally attack both an 
arm and leg of the same side. 

Bilateral ataxia of the arms alone has never been observed. But the cere- 
bellar gait as well as Romberg's phenomenon has been noted. 

The pressure sense may be disturbed. Schlesinger made tests with a special 
instrument (algesimctcr of Max Herz and Hermann Schlesinger) and found 
that the sensibility of the skin to pressure exerted upon it was sometimes 
diminished or absent, while the tactile sense was retained. The pressure sense 
of the skin also varies from that of the deeper tissues ; therefore in this respect 
also the pain and temperature senses may be analogous. 

Under these circumstances it does not appear remarkable that any diminu- 
tion of sensation in the deep as well as in the superficial parts, as well as the 
so-called stereognostic sense, may here and there be lost, so that the patients 
are no longer able to distinguish the differing substance of articles placed in 
their hands. 

In conclusion, the peculiar sensation produced in the bones by placing a 
vibrating tuning-fork upon them may be absent; this is neither remarkable 
nor, at present, of especial signifiance. 

Much more important is the third group of symptoms, trophic disturbances, 
which are found in great variety upon the skin as well as in the bones, the 
joints, and other parts of the body. 

Many abnormalities are observed, especially in the hands, where they re- 
semble those due to neuritic changes, but in neuritis they are never so exten- 
sive, so regular nor so varied as in syringomyelia. 

In this affection the hands may be swollen, the skin abnormally red, and 
erythromelalgia may be simulated ; in this, however, there is also severe pain. 
At other times the skin may be very red but smooth and firm, so that we call 
it " glossy skin " ; changes similar to those of scleroderma and sclerodactylia 
may appear, but the skin is firmer and more tense. Often it becomes cyanotic, 
and shows changes resembling those which take place in the beginning of 
Raynaud's disease from deficient arterial nutrition of the finger-tips. Mari- 
nescu has described a special " main succulente." In this condition the whole 
or a part of the dorsal surface of the hand is swollen yet does not pit upon 



600 SYRINGOMYELIA 

deep pressure with the finger. The fingers are likewise affected, and are 
swollen into a spindle-shape; the skin is smooth and glistening, and when 
cold is reddish blue. In other cases there is edema. 

Vesicles and eczema are observed, the latter, especially, being chronic. The 
vesicles resemble those from burns and not rarely are due to this cause. But, 
like pemphigus vesicles they usually develop from. an unknown cause, they do 
not itch, and they usually desquamate without leaving scars. 

Deep fissures, especially in the transverse folds of the skin, the so-called 
rhagades, are very conspicuous and slow to heal, as are also ulcerations, cica- 
trices and indurations of all kinds and origins. Sometimes the patients do 
not know how these have arisen. Sometimes the parts have been burned, 
crushed or bruised without any sensation of pain having developed, and con- 
sequently their treatment has been neglected. 

Whitlows are not so rare, as in my case, and a number of these may occur 
in close succession. Unlike the ordinary form in which they are very pain- 
ful, they run their course painlessly; after Morvan they have been designated 
" panaris analgesique " and when they occur with " pareso-analgesie " of the 
upper extremity they have been considered a special type of syringomyelia, 
since they have never been actually observed except in this disease. Being 
painless, they are frequently neglected, hence there are often severe sequels, 
more often than in the ordinary form; sometimes the nail and a part of the 
bone are permanently destroyed, the fingers are shortened and their tips 
clubbed. 

Deep suppuration in the hand also may generate necrotic processes and 
cause stiffening of the joints and loss of substance ; marked deformities resem- 
bling those of leprosy may occur and to these the term of " syringomyelia 
mutilans" (Hoffmann) has been applied. 

Arthritic inflammation and non-suppurative inflammation of a tendon are 
also frequent; in some cases a thickening of the entire volume of the hand 
may make the condition simulate acromegalia. I recently saw a case of this 
kind in which a different diagnosis had been made, apparently because the 
popular nomenclature for this condition had led to an erroneous conception. 
The other symptoms of Pierre Marie's disease, particularly the changes in the 
head, were absent, and the classical features of syringomyelia present. 

Other parts of the body as well as the hands may be attacked by trophic 
disturbances-, the upper arm, the upper part of the trunk, or even the face 
and the legs. Erythema may appear temporarily on the shin; not infre- 
quently we note the so-called writer's shin, sometimes spinal edema develops 
and is almost wholly confined to the arms, being unilateral, and lasts hours 
or days. Urticaria is more common, in the form artificially produced by 
scratching as well as that which is spontaneous. It differs from other vari- 
eties of urticaria in developing only upon the analgesic and anesthetic areas 
of the skin. Vesicle formation is found only in those parts altered by syringo- 
myelia; a pemphigus affecting the entire body has very rarely been observed. 
Herpes zoster also is extremely rare, but this may appear as the gangrenous 
herpes zoster described under syringomyelia. 

The changes in this area produced by altered sweat secretion are very inter- 
esting. Hyperhidrosis as well as anhidrosis occurs. Sehlesinger has abo 
described a " paradoxical sweat secretion " in which the patient perspires under 



SYMPTOMATOLOGY 601 

the influence of cold, not under that of heat. Usually these alterations in the 
secretion of sweat are circumscribed and unilateral, stopping at the median 
line, and are most common in the face, next in the trunk, and sharply circum- 
scribed in circles. Often, however, arms and hands unilaterally and bilaterally 
are affected ; this is much rarer in one leg. These anomalies sometimes develop 
early, and occasionally are said to be the first symptoms of the disease. Their 
duration varies; frequently they persist for months or years. 

Deep inflammation of the subcutaneous tissue or other soft parts, espe- 
cially that of a purulent, phlegmonous character, occurs in the hands but 
also elsewhere, most frequently in the arm, rarely in the feet and toes. This 
is painless, and may persist for a long time. Here and there it is steadily 
progressive, and may finally cause death. It is usually due to infection after 
injuries which the patient with syringomyelia has sustained; in how far it 
may be caused by a greater predisposition and vulnerability of the tissue itself 
in consquence of nervous disturbance is difficult to decide. In addition to the 
ordinary bed-sore which is so common in severe and progressive diseases of the 
spinal cord, gangrene and necrosis may also occcur. Mai perforant, perforat- 
ing ulcer of the foot, which occurs in tabes and peripheral neuritis, is also 
seen in this affection. But circumscribed acute necrosis may rapidly develop 
upon the skin of other parts of the body, either spontaneously or after an 
assignable cause. As in mal perforant, desquamation of the gangrenous tissue 
is sharply demarcated, leaving a round ulcer which usually shows little 
tendency to heal. Here we must also mention Raynaud's disease which is 
rarely observed in syringomyelia. 

It will be observed that these symptoms are referable to the domain of 
dermatology, but the frequent arthritic and osseous affections which so often 
accompany syringomyelia and which may even introduce the disease, belong 
to the surgeon. As in tabes, inflammatory and deforming diseases of the 
joints are common. Certainly the analgesia of the deeper parts in combination 
with the effect of trauma is the cause of this. To what extent nervous influ- 
ences directly effect this by an alteration of the vascular innervation and the 
chemism cannot be stated. Certainly their conjoint action must be assumed. 
It is different in tabes, particularly when the upper extremities are affected, 
hence, according to Schlesinger's statistics, 80 per cent, or more of arthritic 
affections occur in the arms. The shoulder-joints are most frequently affected, 
next the elbow-joints, and less often the wrist. Even the joints of the jaw 
and the sternoclavicular joints may be affected, although rarely; in a some- 
what larger proportion of cases the knee- and hip- joints are involved. As a 
rule, these changes take place on only one side of the body and are most 
common in the male, which is probably because males are more exposed to 
trauma. According to statistics, these changes in the joint are noted in 10 
to 25 per cent, of all cases of syringomyelia. They develop in various ways: 
Either slowly with moderate pain and with grating sounds on active or passive 
movements of the joints, or suddenly, with or without such prodromes, by the 
appearance of swelling in the joint due to a considerable effusion into its 
cavity. There is usually no pain. The swelling may persist for days or weeks 
and then disappear, but readily returns. The usual changes seen in the ana- 
tomical picture of a deforming joint inflammation now develop, the individual 
features being partially determined during the life of the patient by the aid 
40 " 



602 SYRINGOMYELIA 

of the X-rays. As a rule, there are at the same time atrophic and hyper- 
trophic processes in the membranes of the joint as well as in the cartilage and 
the neighboring bone. Spontaneous luxations may occur, particularly in the 
shoulder- joint, and these may become so habitual as to divert attention from 
the underlying syringomyelia which remains unsuspected. In other and rare 
cases there is ankylosis. The atrophic change, as well as the accumulation of 
fluid, is usually most marked in the affected shoulder. In the elbow-joints 
hypertrophy is more prominent, particularly of the bony parts. When the 
wrist is affected, we note dislocation of the hand to the volar side and swell- 
ing of the bones of the forearm, while the changes occurring in the hip- and 
knee-joints are chiefly hypertrophic. The periarticular soft parts are also 
sometimes implicated; the muscles and tendons of these areas may swell and 
sometimes show a partial ossification. Here and there purulent inflammations 
develop in the joint and its surroundings, most readily in the wrist-joint. 
In the hands and forearms, where, as has been stated, fissures, erosions and 
ulcerations are most common, infection readily takes places and may be trans- 
mitted from the skin to the deeper parts, to the bursa mucosa, the tendons, 
etc. Sometimes primary necrosis occurs in the bones and leads to arthritic 
suppuration; or the purulent infection may be traced to a fistula which has 
arisen in the inflamed joints from external rupture of the sac containing 
fluid. Such purulent arthritic inflammations like phlegmons may be rapidly 
progressive and lead to death; but they may also terminate favorably, par- 
ticularly under suitable and early treatment. At other times flstulae may 
persist for a long time. 

The changes in the bones are quite peculiar; and fractures of the bone may 
occur under singular circumstances. 

One of my patients, a baker, fractured his arm during ordinary kneading 
of dough. Although he felt no pain, yet crepitation and the thickening of the 
arm attracted his attention. A patient of Bernhardt's also sustained a fracture 
of the ulna while at work, but continued to break stones, and on the next day 
even carried water. A patient of Schlesinger's, while turning her bed-cover, 
heard a loud cracking sound, and found that she had fractured the bones 
of the left forearm. In my experience, the bones of the forearm, in particu- 
lar, are prone to fracture upon the slightest movement without producing 
pain, and the fracture is a simple transverse one. Such fractures may occur 
also in other bones, less frequently in the legs than in the arms. Curiously 
the right side seldom appears to be implicated, in spite of the fact that most 
persons are right-handed. The fractures are apt to occur only in the late 
stages of syringomyelia ; they may heal normally, quite rapidly, or may require 
a long time. The callus may be normal or may develop in superfluous quan- 
tities. Here and there pseudo-arthroses may form. What changes cause 
these bones to fracture so readily has not been sufficiently investigated. Natu- 
rally atrophic processes are thought of; but in one of my cases I was unable 
to determine any such change. A difference in the structure of the osseous 
trabecular or a decrease in calcium has been suggested. At all events, we 
cannot exclude physical or chemical changes of the bony substance due 
to nervous disturbance, although in some cases the assumption of an 
immoderate action of the muscles on the bones while at work appears 
to be sufficient. On account of the loss of muscle and pain sense, the pa- 



SYMPTOMATOLOGY 603 

tient may not be aware that he is subjecting these muscles to any excessive 
strain. 

Besides fractures, atrophic and hypertrophic changes occur in the bones, 
especially in those of the hand, of the wrist, and of the forearm. Now and 
then exostoses, even multiple ones, have been found on the bones of the upper 
extremities and the upper portion of the trunk, changes attributed to existing 
syringomyelia. That enlargement of the bones of the hands and forearms 
with a corresponding growth of the soft parts may simulate acromegalia has 
already been stated; but these are cases merely of pachyacria (Arnold). 

Spontaneous necrosis may occur in the small bones, in the long tubular 
bones, or in the scapula without preceding a periostitis which always impairs 
the nutrition of the bony substance. In the bones as well as in the skin 
necrotic processes may be the direct result of internal, but still unknown, 
changes, hence vascular spasm or a chemical action upon the nerves must be 
considered. In the hands and feet the desquamation of detached fragments 
of bone may be observed. 

In how far the numerous diseases of the vertebrae may be referred to pri- 
mary changes in the bone cannot be decided at present. At all events scoliosis 
and kyphoscoliosis are very common, occurring, according to Bernhardt and 
Schlesinger, in about 25 per cent, of the cases, and according to other authors 
much more frequently. In the last patients I have observed they were almost 
invariably present. Scoliosis usually develops first, and in the thoracic ver- 
tebral column; later compensatory curvatures of the deeper parts are formed 
and kyphosis is added. These changes usually arise in the later stages of 
syringomyelia, but sometimes they occur in the beginning. Their pathogenesis 
is by no means clear. Unilateral atrophy of the muscles of the back and 
shoulder with a unilateral limitation of muscular activity has certainly an 
influence. Xevertheless, primary bony changes are observed in cases in which 
there is no such atrophy, therefore these may be produced by nervous influ- 
ences. Sometimes there are simultaneous congenital changes, particularly 
spina bifida. 

The thorax shows a change in form corresponding with that of the verte- 
brae, a peculiar malformation to which Pierre Marie and Astie have given 
the name " thorax en bateau." The anterior and upper portion of the thorax 
present a depression; the shoulders protrude anteriorly, but neither atrophy 
of the pectoral muscles nor kyphoscoliosis necessarily accompany it. 

Other anomalies of the nervous system which may appear in syringomyelia 
must be enumerated. The reflexes, especially the cutaneous, show no pecul- 
iarities ; they are absent when the conduction arcs or their central stations 
are destroyed, which, however, is rarely the case; this applies also to the 
reflexes of the abdominal walls. Often they are increased; the Babinski toe- 
reflex may be present either where the pyramidal tracts are directly altered 
or merely irritated from pressure; the tendon reflexes of the arm are usually 
early lost or they decline in strength, especially when atrophy is prominent, 
while in the legs, even in this stage of the disease, they are either unilaterally 
or bilaterally more or less increased. Of course, they may be absent or dimin- 
ished when there is cavity formation in the spinal cord which extends to the 
lumbar portion or is chiefly seated there, or when the posterior columns and 
posterior roots are implicated, as may be the case in a complication with 



604 SYRINGOMYELIA 

chronic meningitis. Now and then when syringobulbia is combined with de- 
generation of the cervical pyramidal fibers, or with an increase in pressure 
of the fluid in the fourth ventricle, then the tendon reflexes in the arms and 
lower jaw may be increased. 

Various disturbances in the function of the bladder, rectum and genital 
organs may appear. Since the seat of motor change is usually the central 
gray of the cervical portion, in the majority of cases these disturbances either 
do not occur or only appear late. In lumbar syringomyelia bladder disturb- 
ances early become prominent. If massive gliotic or gliomatous proliferations 
from within press upon the white columns of the spinal cord, the same dis- 
turbances may be produced as in compression of these parts. 

The bladder disorders are usually of a motor nature; as a rule, there is 
at first difficulty in urination, then incontinence, sometimes even early the 
incontinence of retention, cystitis may be added. As nephrolithiasis has beeii 
somewhat frequently found, this condition also has been directly attributed 
to the spinal affection. 

Sensory disturbances of the bladder are rare, but they may exceptionally 
implicate the temperature sense of the mucous membrane. Strangury may 
appear with or without other sensory disturbance of the mucous membrane 
of the bladder, in which case there may be a decided retention of urine yet 
the patient be unaware of the condition. 

Gastrointestinal disturbances, from simple constipation to incontinence, 
are rare; but sensory disturbances may sometimes exist which render the 
patient unconscious of the evacuation of feces. 

Little is known of anomalies of the sexual functions. As the lumbar cord 
is usually not involved, these may be unimpaired; however, not rarely there 
is a disappearance of sexual desire although erection and ejaculation may be 
normal. In the case of pollutions the sensation of libido may be absent. 

Concerning disturbances of the cranial nerves, in so far as these have not 
yet been considered, especially in regard to disturbances of sight, the following 
may be mentioned: 

The olfactory nerve is not implicated; the optic nerve may be affected by 
complications, or when marked gliosis and gliomata have developed in the 
bulbus medulla? and the pressure of the cerebrospinal fluid is consequently 
increased, choked disc may then appear and gradually pass into atrophy. The 
other rare anatomical changes in the optic nerve depend upon complications. 
Of course, in severe disturbances the faculty of sight is correspondingly im- 
paired. 

Derangements of the field of vision have been frequently observed and 
studied. It has been shown that there is usually no limitation of the visual 
field, that in some cases a narrowing due to hysteria arises, but that it is more 
often observed without hysteria. A defective appreciation of colors, particu- 
larly green, is said frequently to exist. 

The auditory nerve is rarely affected, although in syringobulbia the spaces 
and glia folds may implicate the individual nuclei of the auditory; not those 
which are connected with the sense of hearing, but those which serve to main- 
tain the equilibrium. Notwithstanding this, however, it cannot be denied that 
the trunks of the auditory itself, or the acoustic portions of the central appa- 
ratus of this nerve, may suffer from increased pressure due to marked glia 



COURSE AND PROGNOSIS 605 

proliferation, from hemorrhage, etc. Certainly in some cases more or less 
defective hearing or perverted hearing can be assigned to no other cause. 

The sense of taste is often impaired. Its disturbances, like other bulbar 
symptoms in syringomyelia, are usually bilateral. The anterior as well as 
the posterior portion of the tongue may be involved with or without simul- 
taneous decrease of sensation. Xow and then only certain perceptions of taste 
are impaired or destroyed; perversions may also appear. In how far these 
disturbances are due to alterations of the nucleus and tracts of the glosso- 
pharyngeal or the fifth nerve, has not yet been determined. 

Disturbances on the part of the vagus have already been described; when 
gliosis produces marked pressure in the bulbus, vomiting may appear. Some- 
times, as Friedreich and I observed, diabetes insipidus may develop, while 
glycosuria is rare. Persistent and transitory glycosuria have been observed, 
as well as an alimentary form. 

Among the general cerebral symptoms which may accompany syringo- 
myelia, vertigo must be mentioned. It occurs in paroxysms in the form of 
rotary vertigo, and is quite independent of muscular disturbance of the eye. 
Sometimes it is so intense that the patient falls; usually there is no loss of 
consciousness. In explanation of its development affections of the vestibular 
nerve have been considered — those which contain the fibers for the equilibrium. 
Hemorrhages, changes in arterial tension, anemia, thrombosis, edema, and sud- 
den or rapid increase of the pressure of the cerebrospinal fluid may cause 
the vertigo. 

Apoplectiform attacks not rarely occur, probably from the same causes, 
and may be conjoined with vomiting, dyspnea, and changes in the pulse, as 
well as with various succeeding paralyses in the bulbar area and the course 
of the spinal nerves. Paresis of certain cranial nerves as well as weakness 
of the arms and legs or the cerebellar gait may be more or less persistent. 
Now and then sensory disturbances follow, chiefly paresthesia. 

Independent of other attacks there is often severe headache which is prob- 
ably due to increased pressure of the cerebral fluid. 

Among the complications are decided psychical disturbance* which are not 
rarely caused by a predisposition to anatomical anomalies of the brain as well 
as to an abnormal composition of the spinal cord. Of course, the sometimes 
very rapid rise in cerebral pressure noted in syringobulbia may produce a 
transitory dulness and confusion of mind. 

Generally, however, there is no implication of the psychical functions 
which produces true mental disturbance. But Schlesinger states that, in his 
experience, patients always show a peculiar behavior in regard to analgesia 
and thermo-anesthesia. Even if specially instructed that the application of 
heat will not lessen their feeling of cold, they persist in burning themselves 
in some way. I have never observed anything of this kind. 

COURSE AND PROGNOSIS 

In describing the course of syringomyelia and syringobulbia I may be 
brief. The disease is of long duration; it may be protracted through years 
or decades. The cases combined with gliomatosis are more speedily fatal, 
terminating in from one to six years, but of this we have no accurate statistics. 



606 SYRINGOMYELIA 

Syringobulbia is not likely to be so soon fatal, for instance in a few years; 
but it may last a dozen years or longer. In this affection death occurs earlier 
when the nuclei of the vagus are implicated than in the ordinary cervical and 
dorsal syringomyelia, while the rarer lumbar affections combined with cystitis 
and its consequences are even more rapidly fatal than the first mentioned. 
Variations in the course of the disease are not uncommon. I have already 
mentioned apoplectiform attacks with their sudden aggravations; these may 
occur in true syringomyelia without implication of the bulbus; usually the 
symptoms gradually increase in severity. The onset varies greatly. It may 
be marked by paresthesias with succeeding analgesia as well as by atrophy, 
various trophic disturbances, and sometimes bulbar or bladder symptoms. In- 
tercurrent diseases, particularly enteric fever, may aggravate the condition. 
Sepsis after any kind of suppuration, and a complication with tabes, pro- 
gressive paralysis or other disease of the central nervous system may hasten 
the end. Otherwise, and so far as duration of life comes into question, syringo- 
myelia is a benign affection, but it leads to early invalidism and deformity. 



OCCURRENCE AND ETIOLOGY 

In regard to the occurrence of the disease, it must be emphasized that 
syringomyelia and syringobulbia, contrary to the views long maintained, are 
comparatively frequent manifestations of disease of the central nervous sys- 
tem. True, they are not so common as ordinary tabes, and probably not so 
frequently noted as multiple sclerosis, but still they are much less rare than 
motor (spastic) tabes in its various forms and the combined system diseases 
in general. Even acute myelitis is not so common; naturally I except com- 
pression, the poliomyelitis of children, and that due to syphilis. Men are 
more frequently attacked than women; according to Schlesinger the propor- 
tion is 2 to 1. Youth, especially the second and third decades, is the most 
vulnerable period; but the disease is also found in the aged. Now and then 
heredity has been observed. 

The cause and pathogenesis of the disease were described under the patho- 
logic anatomy. I shall only repeat here that these are not uniform, that an 
inhibition of development must be considered, and that hemorrhages, inflam- 
mation, thrombosis, and perhaps also vascular obliteration, may play a role. 
The view that leprosy leads to syringomyelia is absolutely untenable from 
anatomical demonstration; moreover, it cannot be maintained that peripheral 
neuritis leads to the disease by an advancing inflammatory process and the 
formation of cavities in the central nervous system; for, in the first place, 
syringomyelia has never been observed even after most advanced and extreme 
polyneuritis with disease of the posterior columns or the ganglion cells of the 
anterior gray substance, although there may, of course, be an accidental com- 
bination of these pathological conditions. Ascending neuritis, too, is very 
rare, even if the diagnosis be based only upon the development of the clinical 
symptoms; finally, additional ad hoc hypotheses are necessary to explain why, 
in cases quite analogous clinically, like those in which an ascending neuritis 
is followed by a secondary syringomyelia, the posterior and anterior nerve 
roots which enter have not been " neuritically " altered. 



DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS 607 

DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS 

The diagnosis and differential diagnosis are difficult problems. For the 
recognition of classical cases of syringomyelia and syringobulbia they are quite 
easy, but not otherwise. In the first place the existence of the disease may be 
overlooked, particularly in the early stages, and especially when merely vague 
trophic disturbances of the skin and joints or isolated bulbar symptoms are 
present. Even in the examination of apparently simple affections we should 
invariably be thorough; as, in fiirunculosis or in certain eczemas, diabetes 
mellitus is thought of and investigated, so also in the case of whitlow, disease 
of the joints, and unilateral disturbances in the secretion of sweat, etc., the 
possibility of syringomyelia must be considered. If there are no symptoms, 
the probability of cavity formation cannot be decided. 

This disease may also be confounded with others: (1) With functional 
nervous disease, hysteria; (2) With destructive diseases of the nervous sys- 
tem; (3) With certain diseases of other origin. 

Unless it be an accompanying affection, hysteria can only seriously come 
into question when it is the rare sensory form of hysteria with analgesia. In 
both diseases the previously described analgesia limited to members may be 
present, while a form limited to segments favors syringomyelia, and the hemi- 
anesthesias of hysteria manifest a different symptomatology. Another decisive 
fact is that hysterical analgesias may be psychically influenced, they often 
appear and disappear suddenly, which is not the case in syringomyelia. 

The disease must be differentiated from many other organic diseases of 
the nervous system. When syringomyelia runs its course without sensory dis- 
turbance, which is rarely the case, it may be confounded with amyotrophic lat- 
eral sclerosis with or without amyotrophic bulbar paralysis, with atrophy of 
the nuclei of the motor spinal or the bulbar, ganglion cells, or perhaps with 
chronic poliomyelitis; but in fully developed motor tabes the entire course of 
the disease is more rapid, the atrophies and spasms are symmetrical, the paral- 
ysis is more extensive and more moderate. Paresthesia and pain are usually 
absent. Further, DeR and fibrillary contractions are generally more conspicu- 
ous and distributed. The bulbar symptoms are symmetrical, and not wholly 
or in part unilateral, as in syringobulbia. 

Atrophy of the anterior spinal ganglion cells alone is extraordinarily rare. 
This can be diagnosticated only when the slowly developing muscular atrophy 
of the hand and arm has existed for years, and when we are thoroughly con- 
vinced that after this long time there are no indications of sensory disturb- 
ance. Otherwise the affection is most likely syringomyelia. 

Chronic poliomyelitis is also quite rare, and differs by its much more rapid 
development, occurring within weeks or months, and by early and conspicuous 
DeR. Lead paralysis may produce the condition ; this can be determined from 
the history. 

Dystrophia muscularis may sometimes occasion perplexity, particularly if 
it be Erb's juvenile form with exclusive implication of the muscles of the arms 
and shoulder ; for syringomyelia, as we have seen, may sometimes begin with 
atrophy of the muscles of the shoulder. But dystrophy attacks the muscles 
much more uniformly and symmetrically, and runs its course almost without 
fibrillary contractions and reactions of degeneration. Finally, hypertrophy 



608 SYRINGOMYELIA 

of the infraspinatus muscle, which is almost always present, contraindicates 
syringomyelia, while increase of the tendon reflexes of the legs or even spastic 
symptoms are in favor of the latter disease. 

As multiple sclerosis sometimes runs its course with muscular atrophy of 
the hands or even partial sensory paralysis, and, on the other hand, intention 
tremor, even scanning speech and nystagmus may appear, in syringomyelia, 
confusion in diagnosis is possible. But in sclerosis the symptoms relate to the 
head, for instance, headache, changes in the temporal half of the papilla, and 
peculiar visual disturbances which begin as definite central color scotomata 
and frequently lead to transitory but decided amblyopia. Besides, the scoliosis 
of the vertebral column and all of the previously described trophic disturbances 
are absent. 

Ordinary chronic myelitis affects chiefly the white substance, and produces 
early spastic and spastic-paretic symptoms, frequently referable to the bladder, 
but there is neither muscular atrophy nor dissociated sensory paralysis. If 
syringomyelia is considered to be a peculiar central myelitis running its course 
with cavity formation (otherwise it does not occur without spaces and cavities), 
we are dealing with syringomyelia itself. The differential diagnosis of spastic 
paralysis may sometimes occasion great or even insurmountable difficulty if 
there is dorsal syringomyelia with implication of the lateral columns, which 
has been now and then observed. When sensory or bladder disturbances ap- 
pear, it is certain that the rare affection, Erb's spastic paralysis, is not present. 

It is sometimes impossible to differentiate chronic syphilitic meningo- 
myelitis owing to cavity formation. Generally, however, the ordinary picture 
of spinal, and sometimes of simultaneous cerebrospinal, syphilis may be dif- 
ferentiated by the more rapid development of the individual symptoms, by the 
severity of the pain in the back and in the head, by the early implication of 
the brain and the nerves supplying the muscles of the eye, and, above all, by 
the improvement frequently seen after antisyphilitic treatment. In syphilitic 
meningo-myelitis we almost invariably observe variations in the severity of 
the symptoms and relapses with new symptoms. Of course, these are not 
lacking in syringomyelia, for, as we know, apoplectiform attacks may super- 
vene ; but in syringomyelia there are trophic disturbances and curvatures of 
the vertebra?, which is not the case in syphilis. 

If pachymeningitis or chronic leptomeningitis is coexistent, there is early 
rigidity of the neck and back, and the pain upon pressure is much greater than 
in syringomyelia; but this affection may be combined with chronic pachy- 
meningitis, hence cannot be diagnosticated. If extensive gliomata are present, 
there is stiffness of the vertebrae and greater pain, but usually only in the late 
stages of the disease. 

Caries of the vertebral column may usually be excluded, since in this affec- 
tion, at least in the cervical portion of the vertebral column, deformities grad- 
ually occur with other symptoms of tuberculosis. There are no dissociated 
sensory disturbances. Extra-medullary tumors cause a prolonged initial period 
of pain which does not occur in syringomyelia, although it may be present in 
gliomatosis and gliomata. 

The differentiation from tabes dorsalis is sometimes difficult because sen- 
sory irritative phenomena, such as lancinating pain or even girdle sensation, 
may be prominent in syringomyelia; also because partial sensory paralyses 



DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS 609 

occur in both diseases, and the diagnosis causes the greatest perplexity if the 
syringomyelia be seated in the lumbar region, for this produces an early dis- 
turbance of the bladder and the loss of the patella tendon reflex. In differ- 
entiation we should bear in mind: 1. That in tabes there is often rigidity 
of the pupils with feeble reaction to light, symptoms which do not occur in 
syringomyelia; 2. That in the last named disease we never observe the peculiar, 
narrow, segmental, horizontal zones of hyperesthesia tactile irritations which 
in tabes often appear so early in the skin of the trunk. The concurrence of 
these diseases cannot be diagnosticated, but can at most only be presumed 
when both atypical clinical pictures are present. 

Hematomyelia in its entire localization produces the same symptoms as 
syringomyelia, but its onset is sudden or, at least, it develops rapidly. 

The differentiation of syringomyelia from polyneuritis, including the form 
seen in leprosy, was described under the differential diagnosis of the case whose 
history I detailed. In this much discussed differentiation from leprosy, it 
might be added that the entire facial nerve, particularly the orbicular portion, 
is usually soon implicated, but that the picture of unilateral bulbar paralysis 
of the lower nerves is not seen in this disease. On the other hand, in leprosy 
the lower extremities and the hands are usually affected much earlier than in 
syringomyelia and — except for trophic disturbances in the feet and legs — ac- 
cording to Laehr the reflexes of the sole of the foot are almost invariably 
absent, while in syringomyelia they are usually intact. In the differential 
diagnosis of obscure cases the entire clinical picture and the development of 
the disease, as well as the rinding of the bacillus of leprosy, will be absolutely 
decisive. Non-leprous polyneuritis may occasion decided differentio-diagnostic 
perplexity, particularly when it runs its course with arteriosclerotic changes, 
because in this affection also there are dissociated sensory paralyses. 

Some time ago I saw a patient, aged 68, who five and a half years previously had 
fallen from a ladder and sustained an injury of the cervical vertebral column and in 
whom six months later the following symptoms appeared: Sensations of cold with 
hyperesthesia in the hands and feet ; two years later a difficulty in lifting the feet 
appeared, subsequently a weakness of the hands and inability to perform finer move- 
ments. Gradually the muscles of the hands atrophied, they showed cyanosis, and 
vesicles formed upon them as well as upon the feet. 

Objectively the following was noted: Trophic disturbances in the skin of the hands 
and toes and changes in the nails ; motor symptoms ; weakness of the interossei, of the 
peronei and of the tibialis anticus ; sensory symptoms; dissociated sensory paralysis 
in the arms and legs. There were no bulbar symptoms; there was no deviation of the 
vertebral column, no bladder disturbance, no pain upon pressure over the muscles and 
nerves, no demonstrable alcoholism nor arteriosclerosis. On account of the dissociated 
paralysis, the possibility and the probability of a rare combination of cervical and 
lumbar syringomyelia was considered, particularly as there had been trauma in the 
region of the medulla spinalis. The autopsy, however, showed none to exist; unfortu- 
nately the peripheral nerves could not be examined, but these must unquestionably 
have been changed since DeR were observed in the muscles of the hand and foot. 

Syringomyelia is contraindicated here by the distribution of the distal 
changes, even of the partial sensory paralysis, to all of the extremities, by the 
absence of scoliosis, by the normal tonicity of the bladder, and by motor and 
sensory disease of the leg and foot. 

Now and then paralysis of the brachial plexus, particularly if bilateral, 



610 SYRINGOMYELIA 

may obscure the differential diagnosis, for this condition also produces mus- 
cular atrophy with sensory disturbances. But these disturbances usually set 
in rapidly, are not progressive but either stationary or regressive, they prob- 
ably never assume the form of distinct dissociated paralysis of segmental type, 
and they usually permit the recognition of their cause. 

Among the cerebral diseases which come into question is hemiparesis 
combined with hemiatrophy, which form a rare combination ; in this, however, 
there are no signs of a spinal semi-lesion nor of partial sensory paralysis. Be- 
sides, they usually develop suddenly and are regressive, except for the char- 
acteristic implication of consciousness, of speech, and of certain bulbar 
nerves, above all the centrally paralyzed hypoglossal and facial nerves 
which are usually simultaneously affected, while atrophy of the tongue does 
not appear. 

If, among other affections, scleroderma appears, particularly in the form 
of sclerodactylia and Raynaud's disease, the symptoms of syringomyelia and 
syringobulbia must be carefully weighed and considered. The same is true of 
arthritis deformans in the upper extremities. In this disease we are preserved 
from error by the circumstance that more or less severe pain usually appears 
upon pressure on the diseased joints as well as also in moving them. I have 
already spoken of acromegalia. This disease, with its unmistakable features, 
particularly the changes in the skull and chin, the enlargement of the ears, 
the tonsils and the hypophysis, bears no resemblance to syringomyelia. The 
latter affection leads, if such a term be permissible, only to pseudo-acromegalic 
conditions, to simple pachyacria. 

Finally, as Schlesinger quite properly emphasizes, arteriosclerotic changes 
in the arteries of the extremities may give rise to symptoms which resemble 
the sequelae of syringomyelia — paresthesia, trophic disturbances of the skin, 
necrosis of varying degree, even muscular atrophy — and the majority of these 
are probably localized in the lower extremities but may occasionally implicate 
the arms and hands. There are no dissociated sensory disturbances in the 
extremities, on the trunk, or on the face ; disturbance of the circulation in the 
atheromatous vessels is readily recognized; when the lower extremities are 
implicated, a peculiar intermittent limping sometimes develops. 

When the diagnosis of syringomyelia with or without syringobulbia has 
thus been made, either with certainty or a certain degree of probability, we 
may go a step farther and try to ascertain whether it is the ordinary anatomical 
form of the disease with predominant processes of destruction and compara- 
tively slight glia proliferation or actual tumor formation, extensive glioma- 
tosis. The latter runs a more rapid course than the former, having a dura- 
tion of about two years; in consequence of increased pressure, symptoms of 
transverse lesions, in the form of rapid paresis and paralysis of the legs appear 
early and rapidly, not rarely in the form of semi-lesions, also sudden and 
extensive muscular atrophy if the motor cells are compressed by swelling. 
There is severe pain of a radiating as well as local character, and stiffness, 
sometimes even distinct swelling, of the cervical vertebral region. The bulbar 
symptoms develop suddenly and run a rapid course. Finally, there may be 
marked variations in the symptoms as in ordinary syringomyelia. 

In contrast with other extensive and multiple extra-medullary tumors, 
distinctly limited dissociated sensory paralyses are easily differentiated, 



TREATMENT 611 



TREATMENT 



The more minutely and the earlier a positive diagnosis is made the better 
for the patient: For diagnosis is the mother of treatment. 

Although we cannot absolutely cure syringomyelia and syringobulbia with 
our present remedies, nevertheless, if the disease is recognized early the patient 
can be greatly benefited. By careful treatment at the onset, good nursing, and 
the choice of another occupation for the patient we can at least prevent early 
invalidism. And by explaining to him the nature of some of his disturbances, 
we may prevent trauma such as blows, burns, etc. 

Such lesions as occur will be treated on the basis of the underlying affec- 
tion, by operation or whatever other means seem necessary for the disease of 
the joints, the bones, or the skin. When there is paralysis and muscular atro- 
phy, we must proceed as in other spinal diseases ; in addition to much rest, we 
may try moderate exercise and the cautious use of warmth (not hot applica- 
tions) and electricity. Warmth and passive movements are beneficial for the 
tension, contractures, and stiffness in the joints. When there is edema and 
swelling of the joints, massage may be resorted to. Surgical and dermatolog- 
ical measures, above all physical therapy, are chiefly indicated. Internal rem- 
edies are in order when the patients suffer from pain; primarily the well- 
known antineuralgics, including the salicyl preparations, especially aspirin. 
\f syphilis is suspected, antisyphilitic treatment must be instituted. 

As they arise, other individual symptoms are treated with the remedies 
usually employed in spinal and central nervous affections; to these I cannot 
here refer. 

A more detailed discussion of this subject will be found in the second edi- 
tion of Hermann Schlesinger's monograph " Syringomyelia." If further in- 
formation is desired, the reader may consult 1,175 articles bearing upon this 
disease which have appeared within the last twenty years, and which form the 
basis of Schlesinger's work. 



HEREDITARY ATAXIA (FRIEDREICH'S DISEASE) 
By H. LUTHJE, Erlangen 

There are quite a number of diseases of the central nervous system in 
which we must accept a congenital hypoplasia of definite portions of the brain 
and spinal cord as the main cause, or in a part of the cases an inferiority of 
some special fiber systems. According to our present experience we must, at 
this time, here include the group of hereditary or family ataxias. 

Friedreich was the first to recognize hereditary ataxia as a special disease 
in a number of typical cases — 2 years after the typical description of tabes 
dorsal is by Duchenne — and of them he gave a classic description in 1861. Fol- 
lowing Brousse the affection is accordingly designated as Friedreich's disease. 

The fundamental symptoms of this disease are progressive ataxia, which 
proceeds from the legs to the trunk thence to the arms, but the loss of the 
patella tendon reflex, sensory and sphincter disturbances are lacking. Accord- 
ing to Friedreich's investigation, but especially from Fr. Schultze's researches, 
a constant gray degeneration of the posterior columns was found as the ana- 
tomical lesion. The casuistics of the following years furnished further clin- 
ical and anatomical researches (especially those of Friedreich and Schultze), 
so that the symptomatology and the pathological anatomy of the disease 
received further development. 

However, reports of cases appeared which closely resembled Friedreich's 
disease, from their family appearance as well as from the prominence of the 
progressive ataxia, but differing in other essential details, so that they were 
quite properly regarded as special varieties of the affection. Such were espe- 
cially the cases reported by Nonne as hereditary ataxia, in which the anatom- 
ical lesion proved to be a congenital hypoplasia of the cerebellum. These 
cases as well as others that were examined by P. Marie led the latter author 
to include them as an especial pathologic condition, as heredo-ataxie cere- 
belleuse. The chief symptoms of this disease were progressive ataxia and its 
family appearance. While in Friedreich's disease the patella tendon reflex 
was abolished in heredo-ataxie it was retained, often even actively increased. 
In addition to this there were also signs of absence of function on the part 
of the optic and oculo-motor nerves. The pathologic lesion proved to be 
changes of the cerebellum, but above all, congenital hypoplasia. 

Subsequently other cases were reported which were regarded as transi- 
tional ones; but there are also observations of maladies occurring in several 
members of the same family in which in some the character of the affection 
resembled throughout that of Friedreich's ataxia, while in others Marie's type 
was present. It was also noted that in one and the same patient the symptoms 
612 



CLINICAL COURSE 613 

varied in the course of the disease, for in some the patella tendon reflex was 
at first retained and later was abolished (Senator). Anatomically, transi- 
tional cases were also noted, and in some besides Irypoplasia of the spinal cord 
the same condition was also present in the cerebellum. 

This brief historic retrospect appears to be necessary to justify the succeed- 
ing description. Our present clinical and anatomical experience permits the 
view that the different varieties of hereditary ataxia are nosologically con- 
nected, that Friedreich's disease and Marie's heredo-ata.via cerebelleuse and 
the transitional forms, clinically and anatomically, present one form of dis- 
ease which is characterized by the family appearance, progressive ataxia, and 
in which other symptoms may present the greatest variety, according to the 
distribution of the congenital hypoplasia and the intensity and extent of the 
columnar sclerosis. The greatest number of authors incline to this opinion 
of the nosologic unity of these forms of hereditary ataxia (Schultze, Edinger, 
Londe, Seiffer, Bing and others) for only in this manner does the manifold 
and varying picture of the casuistic and anatomical observations become com- 
prehensible. 

CLINICAL COURSE 

The disease shows a family appearance, i. e., several members of the same 
family are attacked; it may also be observed in succeeding generations, or 
skipping one generation it may appear in the preceding and the succeeding 
one. Now and then sporadic cases have been reported. In the ascendence 
there is always a neuropathic predisposition or degeneracy of some sort. Both 
sexes are equally affected. 

The onset of the disease occurs during youth (between the 10th and 20th 
years). However the limits are by no means so fixed as was formerly as- 
sumed. Sometimes the symptoms appear in early childhood (between the 
3d— 4th years), while in other cases the symptoms arise after the 30th year. 

The first symptom is commonly great lassitude and a languid sensation 
in the legs. Walking becomes difficult without it being possible to discover 
the exact cause. Soon the signs of a disturbance of coordination develop in 
the muscles of the lower extremities. The character of this ataxia is static, 
i. e., the limbs cannot be fixed in the desired position. Associated with this, 
in most cases, there are signs of a locomotor ataxia with its associated peculiar- 
ities : improper choice of the muscles to produce a desired effect, immoderate 
use of power and persistence of the innervation impulse after the maximal 
contraction has been obtained. Slowly and gradually the incoordination at- 
tacks the higher portions of the musculature, namely, the muscles' of the trunk 
and the upper extremities, perhaps also those of the head and of the organs 
of speech. Now only does the cerebellar character of the ataxia become dis- 
tinct. There is a swaying, waddling gait, resembling that of a person under 
the influence of alcohol. In many, not in all, cases the disturbance in coordi- 
nation is increased b}' the absence of control by the eyes. Walking finally 
becomes so helpless and dangerous that the patient cannot get along unassisted ; 
even this ceases and the patient is either compelled to seek the rolling chair 
or is confined to bed. While at first it is still possible for him to sit up, this 
power also is gradually lost on account of the ataxia of the muscles of the 



614 HEREDITARY ATAXIA 

trunk. Thus these patients, in the final stage of the disease, present the pic- 
ture of complete helplessness: they are not even able to take food without 
assistance; they must be fed like children. It takes years until this pitiable 
stage is reached, but the condition is all the more unbearable on account of the 
complete absence of mental disturbance for disturbance of the intellect is 
exceedingly rare. 

The degree of ataxia, the sequence and the rapidity with which the differ- 
ent members are implicated, are in the main as stated. But variations are 
noted corresponding to the lack of uniformity in the propagation of the ana- 
tomical process. 

In comparison with the disturbance in coordination all objective and even 
subjective symptoms are decidedly secondary. The patella tendon reflexes are 
abolished or they disappear in the course of the disease. Only in some few 
cases are the reflexes retained or even increased and these are especially the 
cases which were formerly designated as Marie's disease. This variation in 
the behavior of the reflexes is explained from the progressive extension and 
from the topographical arrangement of the anatomical lesion; the Achilles 
tendon reflexes, in the majority of cases, are lost. In some patients chorei- 
form movements have been observed but this is by no means frequent. Often 
the peculiar form of ataxia may have led to the belief that choreiform move- 
ments were present. 

Very often there are alterations of speech ; it becomes lalling, slow (brady- 
lalia), uncertain and now and then shows an explosive character. There can 
hardly be a doubt that this disturbance of speech is due to incoordination of 
the muscles of articulation and accordingly this symptom is a part of the 
general ataxia. The same is true of nystagmus which is a common symptom. 
It is not present in every case and usually presents a horizontal character, 
but at times a circular nystagmus has been reported. The same importance 
must not be attached to this symptom as formerly, for movements resembling 
nystagmus are also noted in normal persons with a rapid fixation end-position 
of the bulbi (Schultze). 

As a rule Babinski's sole reflex is present, and according to the opinion of 
some authors (Cestan) the permanent dorsal flexion of the great toe, so fre- 
quently present, is due to this. Another anomaly of position, which is very 
common, is the gradual formation of pes equinus; this condition, as well as 
kyphoscoliosis of the vertebral column which is often noted, are not quite 
clear as to pathogenesis. 

True pareses are rare; they do occur and as a rule are complicated by 
antagonistic contractures. Absence of sensory phenomena is the rule. There 
are, however, exceptions especially in the late stages of the disease. Then there 
are phenomena relating to deep as well as to superficial sensation. 

The absence of sphincter implication, trophic and vasomotor symptoms is 
the rule; but there are occasional exceptions. 

On the part of the cranial nerves the optic and oculomotor are the ones 
that are principally involved embracing optic atrophy, limitation of the field 
of vision, especially of the color field, sometimes rigidity to light, paralyses 
of the ocular muscles. The symptoms relating to the optic and oculomotor 
nerves particularly belong to the affection named by Marie heredo-ataxie 
cerebelleuse. It has been shown in this particular also how limits are oblit- 



PATHOLOGIC ANATOMY AND HISTOLOGY 615 

erated, for cases of Friedreich's disease are reported with abolished patella 
reflex and symptoms on the part of. the optic and oculomotor nerves and on 
the other hand cases of Marie's disease with increased patella reflex in which 
symptoms on the part of the nerves of special senses are lacking. 

The mental faculties are intact but occasionally anomalies are observed 
particularly in the later stages of the disease. Often a conspicuous euphoria 
has been noted. 

This then, in the main, represents the essential features and the course 
of hereditary ataxia. In a clinical article it is impossible to describe all the 
varieties and complications minutely; besides this would serve no useful pur- 
pose. The casuistics of the last decades show an increasing number of symp- 
toms which complicate and obliterate the actual picture of the disease. This 
great number of secondary symptoms is by no means so remarkable as we 
shall see later on. As an anatomical starting-point we have a congenital defi- 
ciency of some areas of the central nervous system in which secondarily scle- 
rotic processes develop. These congenital defects are not always confined 
to the same fiber systems and, therefore, in the special case it must depend 
upon the duration of the malady, upon the greater or lesser tendency to sec- 
ondary sclerosis, and upon its topographical arrangement as to what clinical 
pictures the symptoms will present. We have reason to assume that in some 
cases there is complete hypoplasia or at least a functional lack of develop- 
ment of the central nervous system, and that in such instances the clinical 
picture broadens and would increase in complexity, depending upon the dura- 
tion of the disease; provided intercurrent diseases would not terminate the 
affection by causing an early death. Only in this manner is it possible to 
comprehend the continuous series of reports of cases of the so-called pure 
types and those complicated by numerous and manifold symptoms. This is 
also true of the necropsy reports in which there have been found variations 
from an almost exclusive degeneration of the posterior columns to cases with 
extensive degeneration of the entire transverse section of the cord, lesions in 
the cerebellum, in the mid-brain and even in the cerebrum. 

Nevertheless we must adhere to the clinical picture first described by Fried- 
reich x and consider all varieties and differences from the standpoint of this 
sharply circumscribed symptom-complex. 

PATHOLOGIC ANATOMY AND HISTOLOGY 

It is advisable first to review the autopsy findings. The most common 
and constant lesion is a gray degeneration of the posterior columns. This fact 
was sharply emphasized by Friedreich and is prominent in the explicit report 
of the year 1862 " Degenerative atrophy of the posterior spinal columns." 
But Friedreich also recognized an occasional involvement of the lateral col- 
umns, beside atrophy of the posterior roots and a more or less well developed 
chronic leptomeningitis; he regarded the latter as the starting-point of the 
" chronic inflammatory " process. 

1 It appears quite proper to retain the name " Friedreich's disease " for hereditary 
ataxia. The forms later described by Marie also belong to this affection; they present 
only a variety of Friedreich's disease. If desirable we may refer to Marie's type and 
thus give Marie the credit for the development of our knowledge of hereditary ataxia. 



616 HEREDITARY ATAXIA 

It was, however, Fr. Schultze who broadened and developed our knowledge 
of the anatomic changes. He showed that Clarke's columns, the cerebellar 
lateral column tracts, the pyramidal lateral column and anterior column tracts 
may be degenerated. He pointed out that possibly a hypoplasia of the spinal 
cord and of the medulla oblongata, which might be considered as an arrest of 
development, were the origin and final cause of Friedreich's disease. The 
following years furnished a number of necropsy reports which we need not 
consider in detail. The discussion between Schultze and Senator may be 
mentioned as a historic factor, for Senator assumed that a congenital hypo- 
plasia of the cerebellum was the anatomical lesion of Friedreich's ataxia. The 
dispute may be regarded as closed, for later autopsy reports proved that both 
authors were correct. 

The following lesions have been found in the post mortem examinations: 

In the spinal cord there is a constant gray degeneration of the posterior 
columns, principally in (roll's, less constant in Burdach's columns, being more 
or less extensive both in the transverse section and in the longitudinal axes, 
even up to the nuclei of the posterior columns. At one time the lumbar 
cord, at another, the dorsal or cervical areas are implicated to a greater extent. 

The pyramidal lateral column tracts, to a greater or less extent, are fre- 
quently found to be sclerosed. Sometimes only the areas adjacent to the 
posterior horns, at other times the border portions at the junction of the ante- 
rior and posterior horns, in still other instances large areas of the lateral 
columns are involved. Occasionally there are lesions throughout the entire 
length of the cord, sometimes even up to the pons; at times only in isolated 
portions in the cervical, dorsal, or lumbar regions. Much less constant are 
the lesions of the non-decussated pyramidal anterior columns from the decus- 
sation downward. Here the extent of the degenerated portion varies greatly 
in the longitudinal axis of the cord. In the majority of cases the cerebellar 
lateral column tracts and Gowers's bundle are more or less involved, the latter 
from the lumbar cord up to the olives. Lesions of Clarke's columns, of the 
posterior roots and occasionally also of the posterior and anterior horns are 
almost as constant as in the posterior columns. Here, also, there is the great- 
est variation in the degree and intensity as well as extent of the anatomic 
process. 

If we add to this the occasional degeneration of the peripheral nerves and 
of the spinal ganglia, the changes in the pia mater (chronic leptomeningitis) 
and of the central canal (occlusion, division, etc.) we may understand how 
exceedingly manifold is the anatomic picture and how it is hardly possible 
to speak of a disease of only special fiber systems. 

One important finding has not been sufficiently dwelt upon: the hypo- 
plasia of the cord or of the cerebellum or of both together, found in so many 
cases. There are several reports of a hypoplasia of the entire central nervous 
system. In these, minute reductions in size are not considered but marked 
ones which would be obvious at first glance. Hypoplasia of the cord had 
already been pointed out by Schultze, but Nonne was the first to call attention 
to a hypoplasia of the cerebellum as the anatomical cause. While it was first 
believed that hypoplasia of the cerebellum produced Marie's heredo-ataxie cere- 
belleuse and hypoplasia of the cord and medulla gave rise to Friedreich's type, 
subsequent investigation revealed that the relations were not so simple: clin- 



PATHOLOGIC ANATOMY AND HISTOLOGY 617 

ically the symptoms referable to heredo-ataxie were noted without atrophy of 
the cerebellum being present post mortem, and inversely cerebellar atrophy 
was observed without the clinical picture corresponding to the Marie type. 

In addition to this apparent contradiction is the fact that in some cases 
the autopsy revealed no decrease, neither in the cerebellum nor in the cord, 
so that the entire pathogenetic importance of congenital hypoplasia, to which 
Friedreich and Schultze attached great importance and which is probably 
recognized by all authorities at this time, became very questionable. But quite 
unjustly so ! Although in a few isolated cases hypoplasia has been missed 
macroscopically, we must not forget that there are degrees of hypoplasia 
which we are unable to recognize in the usual gross manner in which our 
examinations are made. At all events, the few, exceptional cases, in which 
hypoplasia has been missed, are calculated to overthrow our views regarding 
the pathogenetic importance only when they have been proved by positive 
means, to be such exceptional instances. 

"We note here, as in the clinical picture, a great variation in the post mortem 
findings; but also as in the clinical picture a definite symptom-complex is 
found to dominate. So again in the autopsy reports we note a definite group 
of changes recurring with great constancy. These almost constant lesions 
are: hypoplasia, degeneration of the posterior columns, a portion of the pyram- 
idal tract, the cerebellar lateral column tract, and Clarice's columns. 

The regularity of these lesions led a number of authors to the view that 
in hereditary ataxia we were dealing with a " combined system disease" i. e., 
according to the definition of Kahler and Pick, the simultaneous affection of 
several fiber systems due to a common cause. This view was held for some 
time. Only recently, from the analysis of a large number of autopsy reports, 
have our opinions undergone a change. In part, this is the consequence of 
the knowledge that we cannot regard the posterior columns, the pyramidal 
lateral column tracts, etc., as functional substantive fiber systems, as was 
done formerly. The following considerations will explain this more in detail. 1 
According to our present views, the posterior columns serve to transmit the 
sensation of position and of motion (sensibility of the deeper areas). Most 
authors combine the property of coordination with these " sensation fibers/' 
It has, however, been determined by numerous investigations, that a large 
part of Golfs and Burdach's columns pass through the posterior column nuclei 
directly to the cerebellum, but not to the cerebrum. Therefore these fibers, in 
the true sense of the word, cannot produce " sensations " (for this presup- 
poses an activity of the cerebrum). Another portion, however, passes to the 
cerebrum and arouses the corresponding conceptions. Thus we may explain 
why in some cases of " degeneration of the posterior columns " we have a 
high graded disturbance of the sense of position and of motion without ataxia 
(in certain forms of tabes) while in the pure forms of Friedreich's ataxia 
there are severe alterations of coordination with intact deep sensation. We 
must,, therefore, differentiate at least two fiber systems in the posterior col- 
umns : a cerebellipetal fiber system, in which destruction produces severe 
ataxia, and another system which passes to the cerebrum, a lesion of which 
gives rise to anesthesia of the deeper parts. Quite similar is the course of 

1 1 follow Stcherbek's descriptions. 



618 HEREDITARY ATAXIA 

the centripetal tracts of the skin (lateral column tracts and Gowers's bundle) 
in part to the cerebrum (sensory perceptions), in part to the cerebellum 
(coordinating fibers). 

The " cerebellar " tract of the posterior columns functionally corresponds 
to the cerebellar lateral column tract, which is interrupted by Clarke's col- 
umn as the latter is interrupted by the posterior column nuclei. All these 
cerebellar tracts (cerebellar bundle of the posterior columns, the cerebellar 
lateral columns, Clarke's columns and parts of Gowers's bundle) degenerate 
in Friedreich's ataxia. Degeneration also appears in the centripetal tracts 
of the brain-stem and occasionally in the centrifugal cerebellar tracts (ante- 
rior column lateral bundle and the intermediate bundle of the pyramidal lat- 
eral column tract, which resembles degeneration of the pyramidal tract itself) 
as well as in the cerebellum. Therefore in Friedreich's disease we are not 
concerned with a combined system disease but with a " pure system disease " 
of the centripetal and centrifugal cerebellar tracts. 

These views of Stcherbek are very enticing; but we must not forget that 
degeneration often proceeds far beyond this system and in consonance gives rise 
to other clinical symptoms (sensory and trophic lesions, pareses, etc.). Never- 
theless we must admit that Stcherbak's explanation of the pathologic condi- 
tion may be correct for the uncomplicated cases of Friedreich's disease. But 
such uncomplicated cases are very rare and the longer the disease lasts the 
more complicated the condition. Correspondingly it would be far better to 
drop these diagrammatic limits: in Friedreich's disease we are confronted 
neither with a combined system disease nor with a pure system affection but 
we are dealing with a progressive malady which is due to an uncontrollable 
degeneration in the cord and in the brain. The anatomical process conforms 
to a certain regularity of the sequence in which the systems are attacked, so 
that clinically a very characteristic symptom-complex results which we desig- 
nate as hereditary ataxia. But this timely and topographic regularity is by no 
means constant. 

We will hardly have an opportunity of observing autopsies, in which, cor- 
responding to the view of the uninterrupted, progressive nature of the ana- 
tomical process larger areas of degeneration will be found than have been 
noted up to the present time. For the limit of the anatomical lesions is the 
limit of the capability of life for the possessor of these lesions. 

This almost standard, timely sequence, in which degeneration appears and 
its limitation by the lethal consequences of the somatic condition, have prob- 
ably led to the opinion that the condition was one of combined system disease. 
But this view cannot be justified after we have learned to recognize that the 
degeneration, neither in the transverse section of the spinal cord nor in its 
longitudinal axis and even beyond the cord, is halted by any fiber system. 

We must now add a few remarks in regard to the histologic processes. 
These details we primarily owe to Schultze. There is always a secondary glia 
proliferation in areas in which there has been primary atroph}^ or complete 
absence of the nerve fibers. In addition there is also occasionally perivascular 
proliferation of the connective tissue with thickening of the walls of the ves- 
sels. This view of the secondary glia proliferation has not been undisputed 
and especially Dejerine and Letulle, from their own researches and their his- 
tologic findings, have expressed the opinion that the sclerosis of Friedreich's 



PATHOLOGIC ANATOMY AND HISTOLOGY 619 

ataxia was of a special nature. According to these authors there is a primary 
proliferation of the neuroglia tissue, a true gliosis, with secondary degenera- 
tion of the specific nerve elements. They arrived at the opinion that there 
was a special variety of sclerosis, from the peculiar course of the proliferating 
glia libers in the transverse section of the cord: most all of them did not run 
vertically but in the transverse plane of the spinal cord and at their termina- 
tions revealed peculiar rolls (tourbillons) . Weigert soon after rejected this 
opinion, for he showed that the nature of the sclerosis in Friedreich's disease 
differed in no way from that of ordinary tabes, of ascending and descending 
secondary degeneration, of multiple sclerosis, etc. Bing rendered it probable 
that the radiating arrangement of the glia proliferation develops only during 
a period in which the glia bundle still has a horizontal arrangement ; the glia 
proliferation therefore " retains the topographical characteristics of the youth- 
ful mother tissue." The " tourMllons " observed by Dejerine are to be regarded 
as secondary contracting processes. 

The ganglion cells of Clarke's columns are either atrophied or have disap- 
peared in great part. In the degenerated area:- the walls of the vessels are 
thickened. The lesions found in the anterior and posterior horns consist 
mainly of atrophy or degeneration of the ganglion cells. In the peripheral, 
sensory fibers a predominance of the non-medullated (embryonal?) fibers has 
been found. 

In the cases in which the autopsy showed a hypoplasia of the cerebellum 
important histologic changes were not constant. Nonne in his classical cases 
missed histologic changes in the cerebellum. Fraser and Menzel observed a 
decrease of Purkinje's cells in the gray substance of the cerebellum; no scle- 
rotic processes were ever present. 

AYe now approach the interesting question in how far the clinical symp- 
toms may be explained by the anatomical lesions? Only a partial answer can 
be given, for no disease of the central nervous system appears to be in greater 
contrast, regarding our views of the functions of the fiber systems in the 
cord, than Friedreich's ataxia: there is degeneration of the pyramidal tracts 
— and yet no paresis! there is degeneration of the posterior columns as in 
tabes — and yet no sensory or sphincter disturbance. If we consider further, 
that in " combined column sclerosis " with very similar areas of degeneration 
anatomically, an entirely different clinical picture may arise, these contradic- 
tions appear to indicate that within these coarse fiber systems there must be 
finer differences of a functional and systemic nature, in other words, that we 
cannot conceive as substantive systems the pyramidal tracts, or Golfs and 
Burdach's columns. I mention this only so that an answer can be given to 
a question that must have arisen : if the pyramidal tracts are the motor tracts 
why do pareses not appear when they are degenerated as in Friedreich's dis- 
ease, or why are sphincter and sensory phenomena absent in spite of the fact 
that the posterior columns are so constantly implicated to a considerable extent ? 
The most plausible explanation is perhaps the one given by Stcherbek : degen- 
eration of the pyramidal tracts is only simulated by degeneration of the cere- 
bellifugal tracts which run within the pyramids (intermediary bundle). And 
in the posterior columns only those fiber contingents are degenerated which 
pass to the cerebellum after interruption by Goll's and Burdach's nuclei; 
these serve the cerebellum for purposes of coordination, while the tracts, which 



620 HEREDITARY ATAXIA 

enter the cortex of the cerebrum and give rise to sensory impulses remain 
intact. Only when in the later stages true pareses appear, with sensory and 
sphincter disturbance, may we assume that the corresponding motor tracts, 
as well as the posterior and lateral columns, are affected. 

We may, therefore, explain the incoordination of Friedreich's disease by 
the assumption of an elective, partial degeneration of the posterior columns, 
of the pyramidal tracts, the cerebellar lateral column tract and Clarke's col- 
umns, as well as by an affection of the cerebellum itself. Spastic conditions 
which are sometimes present (there always is dorsal flexion of the toe!) as 
well as vasomotor disturbances which are noted now and then, find their 
anatomic explanation in a degeneration of the reflex inhibitive and vasomotor 
fibers which are assumed to lie within the pyramid contingent. That the 
patella reflex is abolished in the majority of cases is easily understood by the 
fact that the degeneration of the posterior columns is always most intense 
in the lumbar cord, in which its reflex is situated. Whether the speech alter- 
ation is to be explained, as Stcherbek believes, by a degeneration of the cere- 
bellar tracts in the crus cerebri, the choreiform movements by disease of the 
centrifugal tracts of the cerebellum, cannot be decided at the present time. 
The centers that are here involved have not as yet been sufficiently studied. 
The same theories serve to explain the nystagmus. Intention tremor, which 
is often mentioned, requires no special anatomical explanation; for we are 
not dealing with an oscillating tremor clonus but with a simple ataxic dis- 
turbance (Bing). Lancinating pain, which is observed now and then is due 
to a root irritation from chronic leptomeningitis which is often present. 

PATHOGENESIS 

As a well founded view we may assume that hereditary ataxia is an agenesis 
of the spinal cord, of the medulla oblongata and in many cases of the cere- 
bellum, perhaps also of other portions of the central nervous system. Schultze 
and Friedreich had already pointed out the fundamental importance of hypo- 
plasia of the cord and medulla. From this fact hereditary ataxia approaches 
a large group of hereditary, or family diseases which are due to similar con- 
genital defects, the clinical picture being dependent upon the seat and extent 
of these anomalies. The intimate relation of these diseases was only recently 
emphasized by Jendrassik. 

As hereditary ataxia almost always develops relatively late in youth we 
may conclude further that the anatomical changes, especially the degenera- 
tion, develop after birth in the congenitally hypoplastic sections of the central 
nervous system. That these hypoplasias are not the result of secondary or 
inactivity atrophy is proved by the marked degree of the hypoplasia — for ex- 
ample even where there has been an absence of function in certain tracts after 
amputations performed decades previously there is not an atrophy which at 
all approaches this hypoplasia. Some cases reported by Rennie are, however, 
decisive : here 18 months after the appearance of the first clinical symptoms 
an autopsy was held. This revealed a high graded hypoplasia of the spinal 
cord while the areas of degeneration were not extensive; this could not have 
been a case of secondary atrophy. Records of this kind are of the greatest 
importance in regard to the question under discussion. In connection with 



PATHOGENESIS 621 

this is the frequent simultaneous occurrence of other defects of development 
(obliteration or a double central canal, occurrence of non-medullated embry- 
onal nerve fibers). But now the question arises why does degeneration take 
place in these organs that have a tendency to hypoplastic conditions? 

I believe, that we must agree with Edinger and his pupil Bing, that the 
appearance of these degenerations may be accurately explained by Edinger's 
compensation theory. I may be permitted to digress here and consider some- 
what more in detail Edinger' s theory, all the more so, as according to Bing 
hereditary ataxia is a typical example for the elucidation of the compensation 
theory. 

The theory depends upon the law of Weigert and Roux in regard to the 
condition of equilibrium of the normal tissue which also has such a large part 
in the law of productive inflammation. "When cells are destroyed anywhere 
in the organism and the equilibrium of the tissue is disturbed thereby other 
cells soon take the place of those that have disappeared so as to bring about 
the normal tension of the tissue. This equilibrium, in which the cells stand 
to one another, will, however, be disturbed when some cells become feebler 
in comparison with their neighbors. The adjacent cells, in proportion to this 
debility, gain the preponderance and this exists until compensation again 
takes place. 

Every function causes a decrease in the power of the cell, as the function 
of the cells is associated with the use of certain cell products. Under normal 
conditions this is soon compensated by the presence of new nutritive mate- 
rial and the equilibrium is restored at once. But if for any reason this sub- 
stitution does not occur, the cell which has been weakened in function is de- 
stroyed by the preponderance of power of the neighboring cell. Thus it is 
explained that a function which uses up more material than can be compen- 
sated for, always leads to the destruction of the nerve fiber. In this manner 
the development of many nervous diseases may be explained " in that insuffi- 
cient substitution occurs within the ganglion cell. The function produces the 
symptom picture; the disturbance in compensation renders this possible" 
(Edinger). 

An insufficient compensation of this kind may arise in two possible ways : 
Either the possibility of substitution, for any reason (which usually remains 
unknown) is less than under normal conditions. Then a damage in the power 
of function must appear which does not exceed the average of life. Or 
compensation is normal, as in healthy individuals, but the function itself is 
permanently increased beyond the average. Such a condition, a relatively too 
slight power of compensation, is, perhaps, the cause of occupation neuritis, 
while an absolute, too feeble substitution caused by the hypoplasia of the 
organs in question, leads to degenerative conditions in hereditary ataxia even 
without excessive employment of the function of the nerve tracts in question. 

If this theory is correct, the anatomical changes and correspondingly the 
clinical symptoms in hereditary ataxia must develop to the degree and in the 
sequence which would correspond to their employment during life. 

Let us consider whether such a condition can be recognized in Friedreich's 
disease. 

In this affection we have a spinal cord or cerebellum — or both together — 
which are characterized by an abnormal, congenital lack of development. 



622 HEREDITARY ATAXIA 

" The claims made upon such a central organ by the exigencies of normal 
life are surely too great, relatively " ; sufficient substitution certainly cannot 
be brought about. The tracts which are most employed, namely, the centripetal 
tracts which regulate motion and tonus, are first affected. Clinically there 
is ataxia and anatomically degeneration of the corresponding peripheral sen- 
sory neurons (spinal ganglia, posterior roots, posterior columns). On account 
of the lack of regulation by the posterior columns, compensation must be 
brought about by the cerebellar tracts and by those fiber systems which pass 
to the senso-motor regions of the cortex in the lateral columns (according to 
Stcherbek, not to the cerebrum but to the cerebellum) ; there is here in addi- 
tion to an insufficient capability of compensation an additional vicarious func- 
tion, so that besides degeneration of the posterior columns the same lesions 
appear in a portion of the pyramidal columns, the cerebello-lateral column 
tract, and in Gowers's bundle. 

We observe, therefore, rapid consumption of the parts that serve to main- 
tain equilibrium, which are utilized almost continuously by the child learn- 
ing to walk. On the other hand we observe those tracts which are only excep- 
tionally in function, such as the pain tracts, only rarely attacked, and then 
late in the course of the disease. 1 

" The compensation theory, therefore, explains satisfactorily " — I am here 
quoting Edinger literally — " all of the symptoms which depend upon the 
anatomical lesions with which we are familiar. It is not, however, able to 
give a satisfactory explanation for nystagmus, spasm, the intention tremor, 
and the speech disturbance. But this is no reason to reject the theory as 
unsatisfactory ; for up to this time we are not acquainted with the anatomical 
lesions which produce these disturbances; we do not know where and how 
they are localized. It is not impossible that a view which explains so much 
more than the previous ones may subsequently also present reasons for what 
has thus far remained unknown in regard to its development." 

I believe that we really cannot escape the impression that Edinger' s com- 
pensation theory is calculated to explain in a concrete manner the pathogene- 
sis not only of hereditary ataxia but of quite a number of diseases of the 
nervous system. We cannot conceal that some things are left unexplained — 
and Edinger is the first to admit this. Thus we may ask why in hereditary 
ataxia the tracts of the will, which are first and most frequently employed, they 
also having a predisposition to hypoplasia, are not attacked or at least only 
late in the disease and then undergo degeneration. Why do we note in tabes 
(in this affection the compensation theory has also been invoked to explain 
the condition) that the pain tracts are involved so early although they are 
so rarely employed spontaneously? But apart from these difficulties he may 
still be correct: "In favor of the theory in tabes (as well as in Fried- 
reich's disease) is the fact that it serves to explain the symptoms better than 
any other theory." 

i A case reported, by Bezold is very remarkable ; in this patient the ataxia devel- 
oped first in the arms. The man was a merchant and he was required to write very 
much, so that the nerve tracts employed for this purpose were involved to a greater 
extent and much earlier than otherwise — quite contrary to the usual condition in which 
ataxia appears first in the legs (quoted from Bing) . 



DIFFERENTIAL DIAGNOSIS 623 



ETIOLOGY 

The hereditary character of the affection has already been emphasized ; but 
it must be stated that the term " hereditary " is employed here in the widest 
sense. Not only direct heredity, from one generation to another, is implied, 
but also those cases are included which occur in several members of the same 
generation; we have even noted that Friedreich's disease may occur sporadic- 
ally. It is more proper, therefore, to speak of " family ataxia,"' as has been 
proposed by various authors. But as we have seen that in most cases, perhaps 
in all, there is a congenital agenesis or hypoplasia of the cord or cerebellum, 
the etiologic factors must be present in the ascendents ; in this sense we speak 
of hereditary ataxia. 

In regard to the etiologic factors in the ascendency various opinions have 
been expressed. The most undoubted role is played by the neuropathic pre- 
disposition in the widest sense. Signs of degeneration of the most varied 
kind are mentioned; Nonne, in the cases reported by him, found that con- 
spicuous asymmetry of the face was noted in several members of the family. 

Consanguinity of the parents appears to play a part; at least this has 
been very frequently mentioned in the cases. In how far alcoholism or syphi- 
lis, in the ascendents, play a role cannot be determined with certainty; by some 
authors, alcoholism among the parents, is considered very important. 

A remarkable factor, which has as yet not been explained, is furnished by 
a number of acute infectious diseases. Quite often the first symptoms of the 
disease have been observed after some acute infection, such as enteric fever, 
variola, meningitis, measles, scarlatina, diphtheria, acute articular rheuma- 
tism, dysentery, pneumonia and rotheln; Allen Starr is even of the opinion 
that in hereditary ataxia there is less of a congenital lack of development than 
an affection of the entire nervous system appearing in connection with an 
infectious disease. It is all the more difficult to estimate the importance 
of the acute infectious diseases, for, in connection with them very similar clin- 
ical pictures are observed which have been described under the name of acute 
ataxia. In the latter affection, however, the patella tendon reflex is exagger- 
ated and it almost always terminates in recovery. 

We may assume that the tracts which serve coordination are especially 
exposed to the toxic action of the acute infections. In favor of this view 
is the fact, emphasized by Friedreich, that if in the course of hereditary 
ataxia an infectious disease (enteric fever) develops, the course and the sever- 
ity of the clinical picture are influenced in an exceedingly unfavorable manner. 
We must, therefore, consider the etiologic importance of the acute infectious 
diseases in the manner that their toxic action is readily transmitted to the 
fiber systems which are hypoplastically predisposed, which are, therefore, the 
locus minoris resist cut ice. Immediately after an acute infection the first 
symptoms of ataxia may appear. 

DIFFERENTIAL DIAGNOSIS 

The symptoms of an uncomplicated case are decisive in making a diagno- 
sis: the family appearance, onset in relatively early life, the predominant 
cerebellar character of a gradually developing ataxia which, as a rule, begins 



624 HEREDITARY ATAXIA 

in the legs, then affects the trunk and upper extremities and the typical speech 
disturbance. Sensory and sphincter symptoms, as well as pareses are absent 
in typical cases. If the patella reflex is absent but nystagmus, scoliosis of the 
vertebral column, and dorsal flexion of the great toe are present (equino 
varus), we may speak of the spinal type; if on the other hand the patella reflex 
is present or even increased, as well as symptoms on the part of the optic, 
or oculo-motor nerves, we designate this the cerebellar type of hereditary 
ataxia (heredo-ataxie cerebelleuse of Marie). But we have seen that a sharp 
differentiation is impossible, that transitional cases occur and besides depend- 
ing upon the stage of the disease, how quite a number of supplementary symp- 
toms may appear. But as soon as the fundamental symptoms, which have 
been briefly sketched, are present, a diagnosis of hereditary ataxia may always 
be made. 

A special discussion of the differential diagnosis from tabes dorsalis hardly 
seems necessary. The reflex pupillary rigidity, the almost constant pains of 
paroxysmal character, the absence of speech disturbance, the typical spinal 
character of the ataxia, the involvement of the sphincters and of the sensory 
tracts, etc., readily permit of a differentiation. 

In well developed cases disseminated sclerosis will rarely give rise to diffi- 
culties in diagnosis. The behavior of the patella and abdominal reflexes, the 
spastic symptoms, the special nature of the speech disturbance (scanning), 
the history of apoplectiform attacks, must be investigated in doubtful cases. 

Huntingdon's chorea has sometimes been the cause of mistakes and in 
fact, in well advanced cases of Friedreich's disease that are complicated with 
marked choreiform movements, it may be quite difficult to make a differen- 
tial diagnosis from hereditary chorea. In such cases the analysis of the motor 
disturbance may be exceedingly difficult; in addition to this is the fact that 
Huntingdon's chorea is preeminently hereditary. Usually with accurate ob- 
servation it is possible to analyze the movements. In favor of hereditary 
chorea, in doubtful cases, is the late onset (after the 35th-40th year) and the 
presence of severe mental symptoms. 

In tumors of the cerebellum, the symptoms of pressure on the brain such 
as headache, vertigo, vomiting, and convulsions, which are rarely absent, will 
aid in the correct diagnosis. 

It is remarkable that in spite of the profound anatomical agreement in 
some cases that have been described by Striimpell as hereditary or family 
spastic spinal paralysis and Friedreich's disease they should differ so much 
clinically, as a rule, and that their differential diagnosis is so easily made. 
Anatomically, in spastic spinal paralysis, there is degeneration of the pyram- 
idal columns but also of the cerebello-lateral column tracts and of Goll's 
columns. The clinical symptoms are of an exclusive spastic-paretic character ; 
ataxic symptoms are not present. As this disease is, for the most part, lim- 
ited to the lower extremities it can rarely be confounded with Friedreich's 
disease. 

" Subacute ataxic paralysis " which has recently been described by several 
American and English authors may, in its early stages, be mistaken for Fried- 
reich's disease. The disease begins with mild spastic pareses of the lower 
extremities and ataxic symptoms. The spasms in this stage serve to differ- 
entiate the disease from Friedreich's ataxia. In the later stages of this ataxic 



PROGNOSIS, COURSE AND TREATMENT 625 

paralysis a mistake is hardly possible: a sensory and motor flaccid paralysis 
develops, with degenerative-atrophic conditions and lost reflexes (Seiffer). 

In my experience the differential diagnosis of acute ataxia after infectious 
diseases and hereditary ataxia may occasionally give rise to great difficulties. 
I reported 3 children in the same family who during an attack of enteric 
fever, in a certain stage, developed severe ataxic phenomena in all the muscles 
of the body, a marked tottering and swaying gait, nystagmus, lalling speech, 
and choreiform movements. Sensory disturbances were absent, as well as 
symptoms relating to the sphincters ; the patellar reflexes were increased. The 
affection, therefore, revealed the characters of a family disease with the symp- 
toms of hereditary ataxia. As Friedreich's disease may show its first symp- 
toms in connection with an acute infection it is quite impossible to decide the 
question at any given stage of the malady. The further course alone is de- 
cisive : while acute ataxia almost invariably terminates in recovery, hereditary, 
Friedreich's ataxia, is a progressive disease, slowly but surely terminating in 
death. 

PROGNOSIS, COURSE AND TREATMENT 

The prognosis is exceedingly unfavorable, no recoveries having been re- 
ported as yet. This is due to the nature of the anatomical process and to its 
congenital substratum. Now and then remissions are observed and even transi- 
tory improvement. But this is exceedingly rare, so rare that the absence of 
these remissions may be used as an important differentio-diagnostic factor in 
contrast to certain forms of multiple sclerosis. 

The course, as a rule, is very slow but nevertheless progressive. Cases have 
been reported in which the affection lasted 30-40 years. Death is due to in- 
tercurrent diseases. With extraordinary frequency an acute infectious disease 
is the terminal stage in the life of these unfortunates. 

In regard to treatment we are helpless ; now and then slight improvement 
has been reported from the employment of electricity (Eulenburg), from mas- 
sage and electricity (Zabludowski). Actual, permanent and decided improve- 
ment has never been reported. 

Frenkel's exercise therapy, which has been employed, is without effect. 
This is easily understood if we consider Edinger's compensation theory ; every 
function, therefore, especially systematic exercise, must have a deleterious 
action. Friedreich's disease is rather a disease in which skillful nursing 
should be employed than one in which medical treatment is to be considered. 

I have attempted to give a picture of our present knowledge in regard to 
Friedreich's disease. I have purposely avoided monographic details as these 
are not suitable to the character of a clinical article. Those who are more 
deeply interested in the subject will find an opportunity in the study of the 
comprehensive literature. 

Literature 

The pioneer labors of Friedreich and Schultze will be found in : 

Friedreich, (1) "Bericht der Versammlung Deutscher Naturforscher und Aerzte 

zu Speyer," 1861; (2) Archiv /. Psychiatrie u. Nervenkrankheiten, VII; (3) Virchow's 

Archiv, XXVI, XXVII, LXVIII und LXX. 
41 



626 HEREDITARY ATAXIA 

Fr. Schultze, (1) Virchow's Archiv, LXXIX: "Ueber combinirte Strangsklerosen 
in der Medulla spinalis"; (2) Deutsche Zeitschr. f. Nervenheilkunde, 1894, V, p. 27: 
"Ueber die Friedreich'sche Krankheit und ahnliche Krankheitsformen nebst Be- 
merkungen iiber nystagmusartige Zuckungen bei Gesunden"; (3) Berliner klin. 
Wochenschr., 1894, Nr. 33, p. 760: "Erwiderung auf den zweiten Artikel von Senator 
iiber hereditare Ataxic" 

Further special articles particularly in regard to nosology: 

L. Rutmeyer, "Ueber hereditare Ataxic," Virchow's Archiv, 1883, XCI, p. 106. 
— P. Menzel, "Beitrag zur Kenntnis der hereditaren Ataxie und Kleinhirnatrophie." 
Archiv f. Psychiatrie, 1891, XXII, p. 160. — M. Nonne, "Ueber eine eigenthumliche 
familiare Erkrankungsform des Centralnervensystems." Archiv f. Psychiatrie, 1891, 
XXII, p. 283. — H. Senator, "Ueber hereditare Ataxie (Friedreich'sche Krankheit)." 
Berliner klin. Wochenschrift, 1893, Nr. 21, p. 489. — H. Senator, "Ueber hereditare 
Ataxie (Friedreich'sche Krankheit)." Berliner klin. Wochenschrift, 1894, Nr. 28, p. 
639. — R. Stintzing, "Ueber hereditare Ataxie." Munchener med. Wochenschr., 1887, 
Nr. 21. — P. Marie, "Sur l'Heredo-Ataxie cerebelleuse." Semaine medicate, 1893, p. 
444. — Kahler und Pick, "Ueber combinirte systematische Erkrankungen des Riick- 
enmarks." Archiv f. Psychiatrie und Nervenheilkunde, VIII, 7, 251. — Edinger, "Eine 
neue Theorie iiber die Ursachen einiger Nervenkrankheiten." Volkmann's Samm- 
lung klin. Vortrage, 1894, Nr. 106. — Edinger, "Friedreich'sche Krankheit." Eulen- 
burg's Real-Encyclopadie, 1895, 3. Aufl. — Bing, "Die Abniitzung des Ruckenmarks 
(Friedreich'sche Krankheit und Verwandtes)." Deutsche Zeitschrift f. Nervenheil- 
kunde, 1904, XXVI, p. 163. — A. Stcherbek, "Ueber die Kleinhirnhinterstrangbahn 
und ihre physiologische und pathologische Bedeutung." N eurologisches Centralblatl, 
1900, p. 1090. — Higier, "Ueber die seltenen Formen der hereditaren und familiaren 
Him- und Riickenmarkskrankheiten." Deutsche Zeitschr. f. Nervenheilkunde, 1897, 
IX, p. 1. — W. Seiffer, "Ueber die Friedreich'sche Krankheit und ihre Trennung in 
eine spinale und cerebellare Form." Charite-Annalen, 1902, Jahrg. 26, p. 413. — 
Jendrassik, "Beitrage zur Kenntnis der hereditaren Krankheiten." 3. Mittheilung. 
Deutsche Zeitschr. f. Nervenheilkunde, 1902, XXII, p. 444. — Erb, Ziemssen's Handb. 
d. spec. Pathologie u. Therapie, 1878, XL 

It is impossible to quote all of the other literature in detail. I refer to the fol- 
lowing which contain comprehensive reports : 

Londe, These de Paris, 1895: "Maladies familiales du systeme nerveux." "De 
l'Heredo-Ataxie cerebelleuse," p. 250. "Hier besonders die Literatur iiber den 
Marie 'schen Typhus der hereditaren Ataxie, iiber Kleinhirnatrophie, spastische Spin- 
alparalyse," etc. — Crozer Griffith, A Contribution to the Study of Friedreich's Ataxia. 
Transactions of the College of Phys. of Philadelphia, 1888, X, p. 271. — M. Badrian, 
"Beitrag zur Casuistik der Friedreich 'schen Krankheit." Inaug. -Dissert., Leipzig, 
1898 (aus der Klinik von Mendel). — Ladame, "La maladie de Friedreich." Revue 
medicate de la Suisse rom., 1889 (mir nicht zuganglich). 

A very thorough compilation of the literature is also found in : 

Menzel (s. o.), "Beitrag zur Kenntnis der hereditaren Ataxie." Archiv f. Psy- 
chiatrie, 1891, XXII, p. 183 (Literatur iiber hereditare Ataxie und Kleinhirnatrophie). 
— Combes, "Maladie de Friedreich." These de Montpelier, 1902, p. 78. — Brousse, 
"De l'ataxie hereditaire." "Maladie de Friedreich." These de Montpelier, 1882, 
Nr. 37.— Ribel, "De la Maladie de Friedreich." These de Paris, 1894.— Toby Cohn, 
" Vollstandige Zusammenstellung der Literatur seit 1897 in den einzelnen Jahrgangen 
der Jahresberichte fur Neurologie und Psychiatrie." 



SPASTIC SPINAL PARALYSIS, AND HEREDITARY 
SPASTIC SPINAL PARALYSIS 

By E. REDLICH, Vienna 

In 1875 Erb described as spastic spinal paralysis, and soon afterwards 
Charcot as tabes dorsal spasmodique, a peculiar spinal disease which in their 
opinion was chiefly characterized by spastic paresis of the lower extremities, 
its anatomical lesion being considered to be an isolated degeneration of the 
pyramidal lateral column tracts. Naturally, the views of such prominent 
authors in the realm of nervous diseases were accepted with absolute confi- 
dence; and since that time text-books upon neuropathology have invariably 
devoted a chapter to spastic spinal paralysis. But objections from various 
distinguished clinicians were not lacking. The clinical picture described by 
Erb and Charcot was, in its clear-cut simplicity, easily recognized, but the 
subsequent course of many cases apparently belonging to this category, par- 
ticularly the autopsy findings of these, was absolutely different from the sup- 
positions of Erb and Charcot, i. e., not a simple primary degeneration of the 
pyramidal lateral column tracts was found, but quite unlike lesions, for exam- 
ple, multiple sclerosis, compression myelitis, and s} r philitic processes, even 
some of a cerebral nature. This demonstrated that the clinical picture of 
spastic spinal paralysis is primarily merely the consequence of an interruption 
of the motor tracts, due either to cerebral or spinal processes. 

A few years ago, Schule, a pupil of Erb's, expressed himself as convinced 
of the existence of spastic spinal paralysis, and Erb himself has lately come 
forth, and, upon the basis of additional necropsy results, has positively demon- 
strated a form of pure spastic spinal paralysis in the sense postulated by 
Charcot and himself. 

But we must first analyze the facts. Except for the cases occurring in 
children, in whom, as a rule, the symptom-complex of spastic spinal paralysis 
is accompanied either by cerebral processes or the so-called form of hereditary 
spastic spinal paralysis, the clinical picture develops in either sex between the 
ages of twenty and forty. Little is known of the etiology ; sometimes syphi- 
lis is said to be the cause, in which case, of course, the so-called syphilitic 
spastic spinal paralysis is not considered. Oppenheim mentions the puerperal 
state, acute infectious diseases, trauma and lead poisoning. Lathyrus poison- 
ing — after the ingestion of bread prepared from various kinds of lathyrus — 
has produced symptoms corresponding to those of spastic spinal paralysis. In 
these cases no anatomical lesions have yet been observed, and in other cases 
of spastic spinal paralysis we are ignorant of the etiology. 

627 



628 SPASTIC SPINAL PARALYSIS 



SYMPTOMS 

In regard to the clinical condition we must mention first that the course 
is extremely chronic, the duration being from 10 to 20 years, and even longer ; 
this is one reason why these cases are so difficult to judge, for such a patient 
rarely remains permanently under the observation of one physician. The 
symptoms begin gradually in the lower extremities, usually bilaterally, rarely 
in one leg. After a prolonged walk the patients are conscious of fatigue and 
stiffness in the legs. Gradually the symptoms become more pronounced, and 
the patients have a permanent sensation of stiffness in the legs ; upon exami- 
nation there is increased rigidity, passive movement of the legs meets with 
resistance, but the motor power, certainly in this stage, is good or shows but 
little deterioration. The gait now becomes spastic, the steps of the patient are 
shorter and more clumsy. After a long time, perhaps after years, we find a 
further increase in the symptoms. The patient's power of locomotion becomes 
decidedly limited. The legs are in a state of extension and slightly adducted 
at the hips; the contractures can hardly be overcome, the gait being conspicu- 
ously spastic, the feet seeming to cling to the floor, and in walking the tip 
of the foot merely glides over the surface. Now the motor power also is dis- 
tinctly diminished, but the power of walking, although under difficulties, is 
usually retained until near the end. The tendon reflexes which were in- 
creased from the onset become markedly so, there is patellar and foot clonus, 
and, according to recent observations, Babinski's sign is present. In his most 
recent work Erb included this among the classic symptoms of spastic spinal 
paralysis. On the other hand, we find no marked emaciation of the legs, 
no fibrillary muscular tremor, and no disturbance of the electric contractility. 
In the late stages there may be slight spastic conditions with a certain decrease 
of power in the upper extremities and increased tendon reflexes. In the stage 
of onset there are occasional reports of pain in the legs, but, otherwise, sen- 
sory disturbances are absent, as well as disorders of the bladder and rectum, 
vasomotor or trophic symptoms. 

If a case is to be considered as spastic spinal paralysis it follows that, after 
a course lasting for years, no other symptoms will have appeared than those 
just described. The symptoms are limited to spasm, to weakness of the lower 
extremities, perhaps also of the upper, an increase of the tendon reflexes with 
clonic phenomena and Babinski's sign. As a matter of fact this is actually 
true in a considerable number of cases ; but in a much larger number the sub- 
sequent appearance of new symptoms of another kind reveals an entirely differ- 
ent clinical picture. 

PATHOLOGY 

What does the autopsy reveal in these clinically pure cases? In his last 
report Erb described 11 necropsies, which he regarded as proving the existence 
of spastic spinal paralysis, in which there was isolated primary degeneration 
of the pyramidal lateral column tracts or in which this formed at least the 
most predominant change ; for example, besides degeneration of the pyramidal 
lateral column tracts there was slight degeneration of the cerebellar lateral 
column tracts, or of the posterior columns, or of both. This disease of the 



DIAGNOSIS 629 

lateral columns extends throughout the entire spinal cord, and with decreasing 
intensity up to the medulla, beyond which point it does not exist. It is not 
always strictly limited to the area of the pyramidal lateral column tracts ; Erb 
has recently referred to a degeneration of the posterior half of the lateral 
columns. Among the cases he cited as positively proving the existence of the 
disease are Bischoff's cases of two brothers suffering from hereditary spastic 
spinal paralysis; these will be described later. In a prior case, reported by 
Striimpell, cerebral symptoms were noted, and the slight atrophy in the gan- 
glion cells of the anterior horns and the nucleus of the hypoglossal nerve which 
is here mentioned cannot be wholly ignored. Friedmann's case merits consid- 
eration because this revealed an endarteritis obliterans of the entire basilary 
artery, and the implication of the cerebellar lateral column tracts and of 
Gowers's bundle is not unimportant. But we do not desire to become weari- 
some by a too minute criticism; the case of Donaggio is pure, the last case 
described by Striimpell is almost pure. We agree with Erb that the patho- 
logical anatomy of diseases of the spinal cord should not be too minutely 
analyzed, hence cases of spastic spinal paralysis such as we have just described 
may from a clinical standpoint be acknowledged as forms of this disease. But 
we must be certain that they are rare cases, the pathology of which is still 
obscure. Above all, we must remember that the picture of spastic spinal 
paralysis is still only a syndrome which may be produced by many causes, and 
that a clinical diagnosis of spastic spinal paralysis is only possible when this 
picture develops very gradually and presents nothing new after some years' 
duration. 

We will briefly review the affections which may transitorily resemble this 
disease, and discuss those features which enable us to make a differential 
diagnosis. 

DIAGNOSIS 

Caries of the vertebral column with compression of the spinal cord may for 
a long time be manifested only by spastic phenomena in the lower extremities, 
while there is at first little if any pain in the vertebral column. In the course 
of time, however, symptoms referable to the vertebrae appear, a protuberance 
forms, sharp radiating pains are felt in the vertebral column, spinal symptoms 
such as paresthesia, hyperesthesia, and anesthesia are added, finally disturb- 
ances of the bladder, even complete incontinence, the paresis in the legs becom- 
ing paralysis, etc. In syphilitic spinal paralysis, unlike simple spastic paral- 
ysis, at the onset or soon after the appearance of the symptoms that have been 
described, slight sensory and bladder disturbances are noted. We know that 
multiple sclerosis may at first be manifested only by spastic phenomena in the 
lower extremities, but in the course of time the picture becomes more dis- 
tinct, for symptoms attributable to the optic nerve develop, above all, char- 
teristic pallor of the papilla, nystagmus, or paralyses of the muscles of the 
eye, and subsequently intention tremor, marked paralytic phenomena, or 
ataxia. Some forms of combined system disease may run their course like 
spastic paresis, even continuously, while in others we note in addition symp- 
toms referable to the posterior columns — ataxia, or absence of tendon reflexes. 

Amyotrophic lateral sclerosis may sometimes begin with spastic paresis 



630 SPASTIC SPINAL PARALYSIS 

of one leg; more rarely spastic paresis then attacks the other leg before 
atrophy is observable in the member first affected. Hence, in a few cases, 
beginning amyotrophic lateral sclerosis may be mistaken for beginning spastic 
spinal paralysis, while, as a rule, if atrophy appears and progresses it soon 
makes the differential diagnosis clear. Chronic myelitis, which is more read- 
ily recognizable clinically than anatomically, may also for some time simulate 
the picture of spastic spinal paralysis ; but its course is, as a rule, much more 
rapid, and symptoms appear which are unlike those of spastic spinal paralysis, 
namely, sensory disturbances, affections of the bladder, trophic phenomena, etc. 
Of other spinal processes which may sometimes cause perplexity, I must men- 
tion syringomyelia and tumors of the vertebral column and of the spinal cord, 
without minutely discussing the differential diagnosis. Finally, we must con- 
sider cerebral processes, particularly the bilateral. As these are most likely 
to occur in children, and the distinguishing factors are also applicable in the 
differential diagnosis of so-called hereditary spastic spinal paralysis, I shall 
defer the discussion of the differential diagnosis of this form until we describe 
the latter affection. 

We owe the first reported case of this affection to Striimpell, who subse- 
quently described others. Then cases were published by Bernhardt, Tooth, 
Erb, Hochhaus, Newmark, Eaymond, Kiihn and others, and recently by 
Bischoff. Lorrain has minutely described the affection. Of course, these 
published cases present no uniform and conclusive picture, which to a certain 
extent is also true of many other hereditary diseases of the nervous system. 
While the cases occurring in one family may almost completely correspond 
with one another, the cases published by different clinicians often disclose cer- 
tain differences from, and transitions into, other related forms of hereditary 
disease. 

As the name indicates, this is a hereditary affection. As is evident from 
the reports of Bernhardt or Kiihn, the disease occurs in successive generations ; 
more often it attacks several children in one family, in which the hereditary 
predisposition shows itself in the ascendency in the presence of other nervous 
diseases. A case described by Kiihn reveals a typical clinical picture, although 
it occurred alone. Males predominate among the patients. In a series of cases 
reported by Newmark, Bischoff and Kiihn, the disease appeared in early 
youth, between the tenth and eighteenth year, while in others, for example, 
those reported by Striimpell and Bernhardt, the symptoms appeared only in 
adult life. We have no accurate knowledge of the etiology. 

The main feature of the clinical picture is invariably the syndrome of 
spastic spinal paralysis, such as we have minutely described, and which needs 
no repetition; therefore we observe a gradual stiffening of the legs which in- 
creases in the course of years and, with a slight but at first scarcely noticeable 
decrease in motor power, leads to spastic paresis of the lower extremities 
and the typical spastic gait, also contractures, an -increase of the tendon re- 
flexes with clonic phenomena and Babinski's toe reflex; occasionally pes varus 
or pes calcaneus has been observed. The functions of the bladder and rectum 
as well as sensation remain intact; there is neither emaciation of the muscles 
nor other trophic disturbance. The upper extremities and the cranial nerves 
are not implicated — we are here speaking of typical cases. The course is ex- 
tremely chronic, the disease lasting more than twenty years; there is a very 



DIAGNOSIS 631 

gradual, and scarcely noticeable, aggravation of the symptoms, which may be 
arrested for years or even improvement occur, the patient's power of locomo- 
tion being retained to the last. 

And now as regards the deviations from this picture. It is of little impor- 
tance that Striimpell observed in one case a slight decrease of the temperature 
sense and Kiihn noted the same diminution of the pain sense. In some cases 
an affection of the cranial nerves has been observed, as in the cases of Bern- 
hardt, in which besides intention tremor there was considerable difficulty in 
speech and deglutition as well as slight disturbance of the innervation of the 
muscles of the eye; these cases perhaps account for the opinion, sometimes 
expressed by French authors, that this affection undergoes a certain transition 
into multiple sclerosis. One of StriimpelPs cases had a slight impairment of 
speech ; and Bischoff has reported slightly defective movements of the muscles 
of the lips and tongue. The latter cases so far deviated that, in the more 
advanced stages, there was a certain spastic paretic disturbance in the upper 
extremities, in the muscles of the trunk, and in the nape of the neck, and in 
the terminal stages there was even a decrease of tonus with emaciation, as 
well as psychical alterations quite unlike anything in previous observations — 
a gradually increasing dementia having appeared. 

Necropsy reports were published by only two investigators. In Strum- 
pell's case a degeneration of the pyramidal lateral column tracts was found 
in the spinal cord, increasing in intensity downward, but also implicating 
the cerebellar lateral column tract; in GolFs column also there was slight 
degeneration. Bischoff's case, too, showed anatomical peculiarities. Here 
there was a conspicuous degeneration of the pyramidal lateral column tract, 
increasing from the lumbar cord upward, but less marked in the cervical 
cord, and not perceptible in the medulla, where, however, the degeneration 
extended somewhat beyond the actual area of the pyramidal lateral column 
tracts. In the posterior column there was slight degeneration, limited in 
the lumbar cord to Burdach's column, and in the proximal areas to Goll's 
column. In the last stages a decrease in the volume of the muscles corre- 
sponded with the decided atrophy of the anterior horn cells. Finally, an 
extreme internal hydrocephalus must be mentioned, the cortex of the brain 
showing histologically no change. 

What has been stated regarding spastic spinal paralysis is mainly true 
of the differential diagnosis of hereditary spastic spinal paralysis, the proof 
by the history of its hereditary or family appearance being the main condi- 
tion. One point only needs a somewhat more minute description, and that 
is the differentiation from so-called Little's disease, or infantile bilateral 
cerebral paralysis, the chief symptom of which is spastic paresis of the 
lower extremities. This is all the more necessary because hereditary spastic 
spinal paralysis, as we have seen, appears in youth, while, on the other hand, 
Little's paralysis may be noted in several children of the same family. A 
prominent feature of Little's disease, in comparison with hereditary spastic 
spinal paralysis, is the following: The early appearance of the symptoms, 
these being occasionally recognizable immediately after birth, while in 
hereditary spastic spinal paralysis the affection is prone to appear about 
the tenth year or even later, and the demonstration of the well known eti- 
ologic factor of infantile cerebral paralysis (premature birth, trauma during 



632 SPASTIC SPINAL PARALYSIS 

birth, infectious diseases, etc.), while in hereditary spastic spinal paralysis, 
aside from heredity, no other etiologic factors are known with certainty. 
In contrast with the majority of cases of hereditary spastic spinal paralysis, 
in cerebral infantile paralysis we frequently observe epileptic attacks and 
strabismus, sometimes paralysis of the cranial nerves and even pseudobulbar 
symptoms, and intellection is often impaired to the point of actual idiocy. 
That these factors are not absolute proof is shown by the cases of Bischoff 
(here the concomitant hydrocephalus may also have played a role) and Bern- 
hardt. Finally, in hereditary spastic spinal paralysis hereditary syphilis and 
the spinal diseases attributed to this must be excluded, and this, as a rule, is 
not difficult. 

TREATMENT 

Little can be said of the treatment. Therapy is ineffectual in this grad- 
ually progressive disease; in the main, it is less a question of treatment than 
of careful nursing. Mild massage or electricity should be tried, and, of 
course, all highly irritating methods are to be avoided. Mild hydrotherapy 
may be beneficial. In the late stages, tenotomy or transplantation of tendons 
may be considered. 



PROGRESSIVE MUSCULAR ATROPHY (DYSTROPHY), 

PROGRESSIVE SPINAL MUSCULAR ATROPHY 

AND BULBAR PARALYSIS 

By FR. SCHULTZE, Bonn 

For a long time the progressive muscular atrophies have been the step- 
children of clinicians and physicians. Neither surgeons nor internists de- 
voted special attention to them. Thus I remember from 1869-1871, my 
student years in Berlin and Bonn, that Traube, Frerichs, or Riihle, and 
among the surgeons, v. Langenbeck and Busch never demonstrated this con- 
spicuous and curious disease. I saw the first case in Friedreich's Clinic in 
Heidelberg. This has probably been the experience of many other physi- 
cians. And yet Duchenne about 1850 had devoted considerable attention to 
this affection; he gave a thorough description and endeavored to determine 
its anatomical foundation. Later many other investigators took up this work, 
in France, Cruveilhier, Charcot and Dejerine, in England, Lockhart Clarke, 
and in Germany, Friedreich, v. Leyden, Kussmaul, Erb, J. Hoffmann and 
Striimpell. 

The reason of this prolonged negligence of progressive muscular atrophy 
was probably due to the fact that the pathogenesis and the anatomical nature 
were obscure, and particularly that treatment was ineffective; for the malady 
can hardly be overlooked. Only the muscular atrophy, especially in children 
and women, may be unrecognized on account of the profuse development of 
fatty tissue. The affection may also be confounded with secondary atrophy 
after disease of the joints. But we are not now considering any partial mus- 
cular atrophy, but purely that variety which progresses from muscle to mus- 
cle, and from one muscle region to another. That a circumscribed muscle 
atrophy, which remains isolated, does not arise suddenly but more or less 
gradually, or in a progressive manner, is obvious; but we are not consider- 
ing this condition. 

1. PROGRESSIVE DYSTROPHY (MYOPATHIE PRIMITIVE, 
OF THE FRENCH) 

After these preliminary remarks I shall discuss the various chief forms 
of the disease, and first those which have been known for the longest time 
and in which our present methods of anatomical investigation usually reveal 
only changes of the muscle substance itself, the varieties called by Erb pro- 
gressive muscular dystrophy. 

Infantile pseudo muscular hypertrophy was first described. This affection 
is comparatively common and has been investigated very often, both clin- 
42 633 



634 



PROGRESSIVE MUSCULAR ATROPHY 



ically and anatomically. It is commonly noted in several children of the 
same parents and may be transmitted through several generations. When 
completely developed the clinical picture is very characteristic. 

The following is the history of a boy, aged 13, who had healthy parents, brothers 
and sisters ; there is no similar disease among his relatives. He never learned to walk 
or stand properly; but, with the exception of pains in the back, never complained. 
An increase in the volumen of the calves was early noted without the affected muscles 
showing a corresponding power. Upon examination an enlargement of the calves is 
noted at once, which is all the more prominent on account of the thinness of the thighs. 
The cutaneous fatty tissue is richly developed, so that in the upper extremity and in 
the shoulder girdle there appears to be a normal configuration. Nevertheless upon 
careful palpation the trapezei, the deltoidei, the pectorales, the latissimi dorsi, and the 

serrati antici ma j ores are markedly atrophic and 
very weak, as is proven by weakened resistance 
movements. Active raising of the arms, as well 
as abduction and adduction and rotation in the 
shoulder joints are not vigorous, while move- 
ments of the elbow and wrist joints are much 
stronger; the muscles of the hands, both in re- 
gard to size and power, are normal. Yet promi- 
nence of the supinatores longi upon strong flexion 
of the elbows cannot be noted. 

It is further conspicuous that the infraspinati 
are over-sized and like the muscles of the calf 
are upon palpation coarser than normal. If we 
attempt to raise the patient by the shoulders, they 
seem to slip upward (" loose shoulders "), as they 
cannot be held down by the muscles of the arm 
which have this function. Active flexion and 
extension of the thigh is as little possible as ab- 
duction, adduction, and rotation, although the 
corresponding muscles contract somewhat and 
even become prominent. Nor is extension of the 
leg possible, while the peronei and the muscles of 
the calf act quite well. On the other hand the 
abdominal muscles have no power; only with the 
aid of the arms and hands is it possible for the 
patient to rise from the recumbent posture after 
supporting himself upon the elbows ; lateral 
movements of the trunk are incomplete. 

Standing is possible, the sacrolumbales be- 
coming very prominent posteriorly but at the 
same time the lumbar vertebral column shows 
marked lordosis; when the patient assumes the 
sitting posture, kyphosis of this portion of the 
spinal column is noted. Walking is possible only 
with the aid of a cane; it is very slow and 
tedious; the gait is waddling, like the walk of a 
duck. The slightest push is sufficient to cause 
the patient to fall. It is impossible for him to sit 
down slowly; the patient lets himself fall. If he falls too far or upon the floor, he 
cannot rise unaided. 

The muscles supplied by the facial, hypoglossal, fifth, and pneumogastric nerves, 
as well as the sternocleidomastoids and the ocular muscles, are normal. Nowhere in 
the naked body, even upon prolonged standing, are there fibrillary contractions. The 
skin presents a peculiar bluish marbling, especially in the legs and in the region of 
the buttocks; this is less marked in the arms. 

Electric examination shows a decrease or an absence of response to faradism and 




Fig. 184. — Infantile Muscular Dys- 
trophy. (After Moritz.) Pseudo- 
hypertrophy of the Muscles. 



PROGRESSIVE DYSTROPHY 635 

galvanism; nowhere is there DeR; mechanical contractility is also either decreased or 
absent. Sensation for all qualities is everywhere normal ; there is no pain. Degluti- 
tion, speech and respiration are intact. The vegetative functions and the intelligence 
are normal. The patella and Achilles tendon reflexes as well as those of the arms are 
absent; this is also true of the abdominal reflexes. The pupils are normal in reaction. 

It is hardly possible to mistake this clinical picture or to fail in recog- 
nizing it. There are no symptoms such as appear in cerebral affections or in 
diseases of the sensory or coordinating portions of the nervous system; all 
refers to the motor-trophic sphere of the muscles and all symptoms may at 
once be explained by a disease of the muscle substance; for those sequels 
are lacking which appear in a primary chronic disease of the peripheral motor 
neuron extending from the motor spinal ganglion cell to the motor nerve 
termination in the muscle, namely DeR and fibrillary contractions. In fact 
upon anatomical examination we find in nearly all cases — I purposely say 
nearly — that the muscles only are diseased. The large motor ganglion cells 
of the spinal cord are of normal size and even upon examination, with the 
most improved methods — as we were able to note in two cases of our own 
that were investigated by NissPs method — there is no demonstrable change 
in structure. Of course we cannot maintain that the most minute chem- 
ical and physical properties of the ganglion cell are normal in every respect. 
But it is astonishing enough that no gross changes, above all no atrophy, 
is present, such as appears secondarily after amputation of members, or as 
takes place in young animals when motor peripheral nerves are early removed. 
It appears, therefore, that the motor innervation, which makes its way from 
the brain unhindered, as well as the reflex ones which act from the skin and 
the deeper areas also in an unobstructed manner, are usually sufficient to 
prevent atrophy and a demonstrable change in structure of the ganglion cells. 

The peripheral nerve trunks and the nerve terminations, which could 
be investigated in a very incomplete manner only, have generally been found 
unaltered, while the muscle fibers and their intermediary substance reveal severe 
lesions. Briefly, besides hypertrophy of some fibers with nuclear increase and 
with very frequent vacuole formation, there are atrophic changes of varying 
extent and intensity. Whether a hypertrophic stage always precedes the 
atrophy cannot be definitely answered. Marked increase of the interstitial 
tissue is also very conspicuous, either in the form of an increase of fatty 
cells or of connective tissue cells. This produces the increase in size of the 
different muscles which has been described. 

An increase of nuclei may also be found around the vessels, as I was 
able to convince myself from the autopsy of a boy aged 3 who suffered from 
this malady. This is probably a secondary process, such as commonly takes 
place after primary destruction of parenchymatous elements. 

Therefore these findings justify us from the anatomical standpoint in 
assuming that the clinical foundation of the pathologic picture is a dis- 
ease of the muscles, which in some cases is congenital, but in most cases 
develops early in youth in a family or hereditary manner. For this reason 
an abnormal predisposition of the muscles has been mentioned. The devel- 
opment of muscles is independent, to the widest extent, of that of the nerve 
apparatus in embryonal life, so that without spinal cord and without motor 
ganglion cells muscle fibers may develop. This abnormal predisposition of 



636 PROGRESSIVE MUSCULAR ATROPHY 

the muscles may be so marked that a proper development of muscle fibers 
does not take place, it may be so constituted — similar to the diabetic pre- 
disposition — that at first there is a normal muscular system which, sooner 
or later, under different influences, alters in a progressive pathologic man- 
ner and finally almost disappears. 

Symptoms. — Returning to the description of the clinical picture of pseudo- 
hypertrophy, there are other manifestations which must be described. The 
first symptoms do not permit a positive diagnosis. The waddling duck-like 
gait, which occurs comparatively late, may lead to the view that a congenital 
bilateral dislocation of the hip is present. 

I remember a case in which not even the combined endeavors of my 
surgical colleague Schede and myself were sufficient to assure a physician 
that in a certain case of pseudohypertrophy, which had only involved some 
of the muscles of the back and pelvis and a few of the leg, that the correct 
diagnosis was pseudohypertrophy and not a dislocation. At the present time, 
X-ray examination is an aid which determines dislocation of the hip in a 
decisive manner. Difficulties may appear in elderly persons in the differ- 
entiation from osteomalacia, which is so often overlooked. But in this affec- 
tion pains develop early, both in the bones of the pelvis as well as in the lower 
ribs and within the vertebral column; these pains are caused by external 
pressure, walking, and motion. Pain of this kind is foreign to the picture 
of muscular dystrophy. If it is present, in addition to nervous symptoms 
of a hysterical nature, it is not limited to the bones and depends upon psy- 
chical influences. The late stages of osteomalacia produce certain changes 
in the bones which do not occur in muscular dystrophy. 

Pain of another kind which is localized in the muscle appears in the 
disease, but it must be regarded as partly due to exertion; this is more liable 
to occur in the diseased muscles upon slight action than in the normal ones. 
Another early symptom which was not present in the case whose history I 
related is that children fall much more frequently in jumping and running, 
and when executing complicated movements of the trunk and legs. This 
naturally may be due also to other causes. Moreover there are other nervous 
diseases such as Friedreich's disease with its disturbance in coordination; 
Little's disease, the spinal amyotrophies and neurotic atrophies which will 
be described later on. Then arthritic affections and lesions of the bones 
of a rachitic and tuberculous nature may be present; all of these must 
be carefully investigated, for in rare cases they may be associated with mus- 
cular dystrophy. 

In general, however, certain "disturbances in movement are characteristic, 
these are the following: 

At the onset of the disease, perhaps in the brothers or sisters of a child 
affected with pseudohypertrophy, a sign may be observed which has hardly 
as yet been described. Such a child, while the symptoms that are to be 
described are still lacking, finds it impossible to rise from a stooping posi- 
tion unaided. In one of my cases of this kind only the muscles of the calf 
were somewhat thickened and there was a slight lordosis of the vertebral 
column, which in the sitting posture changed to a slight kyphosis. Further, 
the 9 year old child could not rise from the horizontal posture without the 
aid of the arms, which otherwise, on account of the early weakness of the 



PROGRESSIVE DYSTROPHY 637 

abdominal muscles is impossible in the early stages of the affection. In sit- 
ting down the child permitted itself to fall upon the seat. 

If the atrophy and weakness of the muscles of the trunk and legs has 
made greater advances we observe those peculiar movements, which they re- 
quire to assume the erect posture from the horizontal one. The patient first 
turns upon the side, then assumes the abdominal position, braces himself 
with the hands, brings the feet into the kneeling position, raises the knees 
from the floor while extending the legs so that only the hands of the extended 
arms and the feet of the extended legs are in contact with the floor. Finally, 
first one hand is braced upon the knee of the same side, then the other one 
and in this manner the patient climbs up upon himself, the hands reaching 
higher levels upon the thighs. Xaturally these movements are not always 
performed in precisely the same manner, but they may be modified, one or 
the other point being omitted or changed according to the degree and extent 
of the weakness and atrophy of the existing condition. 

It must be remembered that a similar condition, in regard to the patient 
rising from the ground, may be present when there is disease of the vertebrae 
or some other affection of the muscles, as in a rheumatic inflammation. The 
disturbance that has been described depends upon the fact that an exten- 
sion of the trunk upon the pelvis is only possible to an abnormally slight 
degree, and the bracing of the hands upon the thighs takes the place of the 
sacrolumbales muscles, that, further, the extension of the pelvis upon the thigh 
and that of the thigh upon the leg succeeds only with the greatest exertion 
(especially with weakness of the glutei and of the quadricipites femoris). 
But there may be difficulty in motion in all of these muscles from inflam- 
matory affections, or disease of the vertebrae may give rise to a similar diffi- 
culty in movement of the muscles which are commonly involved in dystrophy. 

The differential diagnosis, apart from the more rapid development of the 
symptoms, will be possible from the fact that painful pressure points over 
the vertebras or muscles are present, quite apart from marked changes in 
the bones; a condition that may be readily recognized by an alteration in 
shape of the vertebrae or by an X-ray examination. 

The ascent of stairs in dystrophy is early rendered difficult from weak- 
ness of the glutei maximi; for in this movement power must be exerted by 
these muscles. The feebleness of the glutei medii is chiefly responsible for 
the waddling gait. 

Often at the beginning of the disease and even in the advanced stage 
the alterations in the muscles of the arms and shoulders are overlooked, first 
because no examination is made and second because there appears to be no 
change. If, however, the power of the individual muscles is tested those of 
the hand are usually found normal; of those of the forearm the supinator 
longus upon both sides is atrophic simultaneously with the biceps and brachi- 
alis internus. In the upper arm the deltoid and triceps are hypertrophic 
bilaterally, but weakened, therefore, pseudohypertrophic; bilaterally at the 
shoulder girdle the serratus anticus, latissimus dorsi, the major and minor 
pectorales with the exception of their clavicular attachments are affected. All 
of these muscles are weak and atrophic. The trapezius with the exception 
of the upper acromial or clavicular portion and the supraspinati reveal the 
same conditions. On the other hand the infraspinati are commonly volumi- 



638 PROGRESSIVE MUSCULAR ATROPHY 

nous and even in the late stages of the affection are prominent in the midst 
of the general atrophy although they do not always possess their normal 
power. These muscle atrophies produce many abnormal positions and dis- 
placements of the shoulder which cannot be separately discussed and which 
are familiar from our knowledge of the pathology of peripheral paralyses. 

The conspicuous symmetry of these changes is remarkable although slight 
asymmetry may be present in some of these pseudohypertrophies. The sterno- 
cleidomastoid and the muscles of the head are usually not involved; but we 
shall see that in certain cases they are attacked and may even be the first 
to reveal the condition. 

In the early stages of the disease, therefore, disturbances are present in 
the upper part of the trunk and in the arms as well as in the lower parts 
and in the legs. It is also noteworthy that primarily the proximal muscles 
are implicated and further that in the upper half of the body as well as in 
the- lower half certain muscles are especially involved ; in the upper portion 
the deltoids, the tricipites and particularly the inf raspinati ; in the lower the 
sacrolumbales, not infrequently the glutei, nearly always the gastrocnemii and 
solei and often the tensores fasciae latse as well as the sartorii. In other in- 
stances other muscles show the condition ; now and then many simultaneously. 

As the affection advances, as is the ordinary course, the force and size 
of the muscles first attacked decreases, even those that are hypertrophic; in 
the muscles of the legs, especially those of the calves, retractions and con- 
tractures appear, which are probably due to the fact that the length of the 
muscle fibers decreases. Walking, and finally standing become impossible ; the 
use of the arms and hands becomes limited for atrophy may also attack the 
muscles of the forearm and hand in which exceptionally there may also be 
an early hypertrophy and atrophy. 

Finally the respiratory muscles may be involved and particularly the dia- 
phragm so that dyspnea appears. In some cases the heart appears to be in- 
volved; at least in some autopsy reports atrophy of its muscle fibers, and 
increase of the interstitial substance has been reported. In a recent case 
reported by Meerwein clinically by the aid of percussion as well as with 
X-ray examination a decided hypertrophy of the heart could be demonstrated. 
The many reports of acceleration, slight irritability and irregularity of cardiac 
action may be explained in many ways, and cannot be definitely referred to 
atrophy or pseudohypertrophy of the heart muscle. 

Quite often the tongue is attacked in the pathologic process ; unilateral in- 
volvement (hypertrophy) has occasionally been reported. The masseters and 
the facial muscles may be involved. Usually such patients succumb early 
although Oppenheim saw a patient aged 58, who suffered since his earliest 
childhood from pseudohypertrophy. They are often attacked by tuberculosis 
probably on account of enforced living in closed, infected rooms. Sometimes 
pneumonia in connection with bronchitis, is the lethal cause; the reason 
being the insufficiency in respiration due to the muscle involvement that has 
been described. At all events the disease is a pitiable one which is more 
easily borne by children than by adults. One of my patients committed suicide 
by hanging. 

Some of the complications are noteworthy. First those rare cases associ- 
ated with general atrophy of the hones, such as described by Le Gendre, Fried- 



PROGRESSIVE DYSTROPHY 639 

reich, and myself ; an atrophy which may be regarded as a coordinate affection. 
Then the more frequent anomalies in the formation of the skull and of the 
bony structure of the body, such as hydrocephalus, asymmetry, clinocephalia ; 
protrusion of the upper jaw as noted by Lorenz ; changes in the thorax (wasp- 
waist by Marie), funnel-shaped chest, spontaneous fractures and the like. 
Xow and then cerebral symptoms are noted, such as weakness of mind and 
imbecility; this condition is, however, very readily simulated to a certain 
extent, as children with pseudohypertrophy are kept from school. 

This briefly is the picture of pseudohypertrophy of the muscles of which 
there may be several varieties; these were formerly described as special affec- 
tions but Erb quite properly combined them under the general term " pro- 
gressive muscular dystrophy/' as the other term " progressive muscular atro- 
phy " is of too general a nature and includes the spinal and neurotic atrophies 
and does not include the conspicuous symptoms of hypertrophy and pseudo- 
hypertrophy. 

Thus v. Leyden described a special form of " hereditary muscular atrophy" 
which is characterized by: first, heredity or family predisposition, second, by 
weakness appearing primarily in the muscles of the small of the back and 
legs late in childhood or during puberty, and, third, by the lack of pseudo- 
hypertrophy of the calves and only after years are the muscles of the shoul- 
der and of the upper extremity implicated. As, however, some muscles, even 
in this form of the disease, may show pseudohypertrophy, as further, the 
ordinary form of pseudohypertrophy may appear in children of the same 
family, and, finally, heredity and the late infantile occurrence is observed 
often enough in ordinary pseudohypertrophy, it is hardly correct to main- 
tain a special form of disease; for all other symptoms such as electric con- 
tractility of the muscles, fibrillary contractions, the presence of motor-trophic 
symptoms and the progressive character are quite the same. 

In other cases the disease may begin in the muscles of the face as described 
by Duchenne; later this type was again made prominent by Dejerine and 
Landouzy, who added a description of the anatomical lesions. In this type 
the affection also begins in early childhood, as is so common with ordinary 
pseudohypertrophy of the muscles, so that the name " infantile form of pro- 
gressive muscular atrophy" which was applied to Dejerine's type possesses 
no differential factor and besides is incorrect, for the malady may arise dur- 
ing advanced life. 

The disease in this form invades the sphincter muscles of the eyes and 
mouth, then the muscles of the forehead and the other muscles supplied by 
the facial nerve. From atrophy and parallel debility the same picture appears 
as from bilateral facial paralysis with inability to close the eyes completely 
so that a space of several millimeters remains. The forehead cannot be 
wrinkled and remains smooth. The mouth is sometimes held slightly opened 
and the lower lip droops ("tapir lip"). The movement of the muscles of 
the mouth is decreased; whistling and showing the teeth is impossible. The 
muscles employed in laughing cannot be used in the normal manner, the 
buccinators do not press the cheek strongly enough against the teeth, the 
face thereby assuming a " mask-like " appearance. The other muscles of the 
head are intact; those of the shoulder and trunk are involved later quite 



640 PROGRESSIVE MUSCULAR ATROPHY 

in the same manner as in pseudohypertrophy ; the inf raspinati are not affected ; 
there is the same lordosis of the vertebral column, etc. Pseudohypertrophy 
of isolated muscles is also observed. The electric contractility, the absence 
of cerebral, mental, and sensory symptoms, the lack of fibrillary contractions 
and the condition of the reflexes corresponds to the type of ordinary pseudo- 
hypertrophy. 

Occasionally, as especially described by J. Hoffmann, at the onset of the 
disease, beside myopathy of the regions supplied by the facial nerve, some of 
the bulbar muscles are involved, so that a complete bulbar type may appear. 
Hoffmann saw 2 children, twins, who had, besides atrophy of the muscles 
supplied by the 7th nerve, atrophy of the tongue, degeneration of the soft 
palate, and in one of these patients also paralysis of the muscles of degluti- 
tion. With this there were the ordinary changes in the muscles of the trunk 
and extremities, such as have been described; while the inf raspinati and leva- 
tores angulae scapulae were spared and the muscles of the calf of good size. 

More frequent than these varieties or types is a form of progressive dys- 
trophy described by Erb, namely the juvenile or scapulo-humeral form, while 
the Duchenne-Dejerine type is called the facio-scapulo-humeral type. 

In this condition there is less of a hereditary or family predisposition 
than in the previously mentioned ones and only after childhood, as a rule, 
from puberty up to the twentieth year does the disease develop. The muscles 
of the shoulder girdle are first affected; most markedly, as in pseudohyper- 
trophy, both pectorales, the trapezii with the exception of the upper portion 
previously described, the serrati, latissimi dorsi, rhomboidei, while the leva- 
tores scapulas, sternocleidomastoidei and inf raspinati are not implicated; the 
deltoids are often normal or like the infraspinati and the triceps increase 
in volume.' The bicipites become atrophic, while the muscles of the fore- 
arm, with the exception of the supinatores longi, are normal. In the lower 
extremities and in the pelvis the glutei and the quadricipites femoris are 
attacked while the muscles of the calf are spared for a long time just as the 
tensores fasciae latae and sartorii increase in size. The distal muscles of the 
extremities are not affected, nor, as a rule, the muscles of the face; later, 
however, as is sometimes the case with the sternocleidomastoidei, they may 
be involved. 

The posture of the body, the movements, the difficulties in rising and 
standing are the same as in pseudohypertrophy, depending upon the distribu- 
tion of the affection. All of the other symptoms are similar, the positive 
as well as the negative ones. The course is slow, usually progressive, some- 
times remaining stationary for a long time or lasting decades. 

Finally, it must be mentioned, that, while in all the types described the 
distal muscles of the extremities are not involved until late in the course 
of the disease or at most simultaneously with the others, J. Hoffmann sev- 
eral years ago reported a few cases, among which, in a man aged 23, with 
a most probable hereditary predisposition, the malady began in the muscles 
of the leg and 6 years later the same changes appeared in the muscles of the 
forearm, while the small muscles of the hands and feet, as well as, during 
the period of observation, the muscles of the shoulders, upper arm, trunk, 
and pelvis were not attacked. In the other case, that of a child, in which 
there was no trace of a hereditary predisposition^ the disease developed first 



PROGRESSIVE DYSTROPHY 641 

in the leg, so that at first a right-sided, and 4 years later a left-sided, equino- 
varus developed and finally a right-sided club foot was added. Besides there 
was weakness of the lower muscles of the back and pelvis with the peculiar 
manner of rising from the floor which has been described and a slight implica- 
tion of the muscles of the shoulder and arm. All phenomena, referable to 
an involvement of the nervous system, were lacking. 

In rare cases, therefore, the malady may begin in more distal regions than 
is ordinarily the case, and occasionally as in a case of Friedreich's which was 
also seen by Erb and myself, simultaneously with the first symptoms of the 
disease the muscles of the hands may be attacked; nevertheless up to now 
positive reports are lacking that the affection had a distal and peripheral onset 
and distributed itself in ascending, which, a priori, is certainly possible. The 
disease may, however, he gin in any transverse stripped muscle, except the heart 
and, as it appears, in the diaphragm, therefore, in the most vital organs. 
Perhaps according to J. Hoffmann the external muscles of the eye may also 
be attacked (a point not mentioned up to now), so that the picture of oph- 
thalmoplegia externa appears. 

In such cases just as in very early involvement of the muscles supplied 
by the facial nerve or in defects of the large pectoral muscles which are often 
noted, the question may arise whether we are dealing with a defective devel- 
opment of isolated muscles, which is by no means rare. Occasionally these 
defects are not congenital but appear during infancy and are progressive for 
some time; they run their course with the corresponding atrophy of their gan- 
glion cell nuclei or may even be produced by this cause. Upon prolonged 
observation we will generally be able to reach a decision from the fact that 
the early muscle defects remain stationary and are not associated with decided 
muscle atrophy. Duchenne, in a case of congenital dystrophy of the muscles 
of the face, observed no progression even after 30 years. 

"We note, therefore, that in all of these forms of disease, we are dealing 
only with varieties of the same affection, a fact that has been determined by 
careful investigations. The differences in the varieties or types relate to the 
varying localization of the muscle atrophy and also to its development at dif- 
ferent ages. Their unity, apart from the similarity in symptoms, is proven 
by the fact that the individual types may be distributed in various ways in one 
and the same family, also that new combinations may arise in the family, 
and, finalty that in one and the same patient another variety may develop 
upon the one already existing. 

The essential point is this, that in the great majority of cases there is a 
permanent absence of symptoms relating to the nervous apparatus and that 
this lack of clinical nervous symptoms as far as our present knowledge reaches 
corresponds to an absence of lesions in the ganglion cells, in the anterior roots, 
and peripheral nerves. In a small number of cases pain may be present, 
which, as has been mentioned, may be referred to exertion but which may 
also be due to irritative conditions (still unknown) within the sensory nerv- 
ous system. 

Now and then DeR may be noted in some muscles, in which it cannot 
be determined, in spite of very careful researches, whether there is a primary 
nervous disturbance or whether the muscle is entirely or partly " without nerve 



642 PROGRESSIVE MUSCULAR ATROPHY 

control." As the changes in the muscle substance which produce a slowing 
of contraction to galvanism are still unknown, we cannot reject the view that 
among these unknown conditions there are some which are independent of 
nervous influences. 

Finally motor irritative symptoms are exceptionally noted, in the form of 
fibrillary contractions, such as occur to a much greater extent in primary atro- 
phy of the motor spinal and bulbar ganglion cells. It is not known whether 
these may not arise without the action of nervous irritative conditions even 
of a peripheral nature, in the muscle fibers. 

A conspicuous tendency of some muscles to hypertrophy and pseudohy- 
pertrophy as well as the localization of the dystrophy which usually shows 
a preference for the proximal muscles and those of the trunk is characteristic. 
I shall refer, later on, to anatomical lesions in the motor ganglion cells which 
may appear to be contradictory to this condition. 

I have already indicated the differential diagnosis of the affection, espe- 
cially in the early stages, from disease of the bones and congenital disloca- 
tion of the hip. As, however, after gross chronic disease of the joint marked 
atrophy appears secondarily, a polyarthritis of this kind may occasionally come 
into question in differential diagnosis. Upon careful examination the signs 
of an arthritic affection of this nature can hardly be overlooked; besides 
there is no indication of pseudohypertrophy or even of true hypertrophy of 
the muscles. Joint pain, due to pressure or motion, is absent in dystrophy 
and so a diagnosis can be made in those arthritic cases in which a more or 
less general atrophy of the muscles of the trunk or extremity is present. 

Now and then the question may arise, whether or not a hypertrophy of 
the muscles has been produced from a primary venous change with chronic 
stasis of the venous blood. This condition has occasionally been observed. 
However, this circulatory anomaly, with its consecutive cyanosis and venous 
dilatation, can readily be determined and it is only necessary to know that 
especially after chronic venous thrombosis muscular hypertrophies may develop. 

Of other diseases of the muscles only polymyositis, with or without simul- 
taneous dermatitis, may give rise to diagnostic difficulties, for, as Oppenheim- 
Cassirer and myself have determined, this condition may be succeeded by 
general atrophy. 'In this very rare affection there is an acute process accom- 
panied with pain, which is regressive and not progressive as in dystrophy, 
leaving at most stationary residues not accompanied by hypertrophy. If dys- 
trophia muscularis is regarded as a progressive polymyositis, as was done by 
Friedreich at one time, anatomically we are then concerned with the same 
condition; the only difference would be in development and the lack of pro- 
gression. Neuromyositis, a very rare affection, begins much more acutely and 
is accompanied by pressure pain of the peripheral nerves and other sensory 
irritative symptoms as well as those due to absence of function. , The differ- 
ential discussion from the neurotic and spinal forms of progressive muscu- 
lar atrophy had better be deferred until these varieties have been described. 

Etiology and Pathogenesis. — In regard to the etiology and pathogenesis it 
must be stated that our knowledge is still very deficient, although a clear 
insight would be exceedingly important from the standpoint of treatment. 
The most interesting factor in the etiology is heredity, which is observed so 



PROGRESSIVE DYSTROPHY 643 

commonly in all varieties of dystrophy. How this affection is transmitted 
from parents to children, or how the affection appears in children without the 
same malady being present in the parents is unknown. At all events there 
are no relations of dystrophy to the infectious diseases affecting the parents, 
or of the mother during the period of pregnancy. Syphilis has no recog- 
nizable influence. Whether intoxications of any kind are active cannot be 
answered. The influence of alcoholism has been most considered. It can by 
no means be always detected, either in the ascendants or in the patients. It 
is remarkable that in some regions the occurrence of hereditary dystrophy and 
of hereditary nervous diseases is much more common than elsewhere. This 
observation can hardly be due to the personality of the investigator ; for exam- 
ple, in the Palatinate and in Heidelberg many of these affections have been 
reported, although it must be admitted that not everywhere is the same atten- 
tion devoted to these maladies as in this district. It is also noticeable that 
the disease occurs frequently in the members of the working classes, espe- 
cially, as it appears in the population of the country districts. Accurate sta- 
tistics are, however, not at hand. 

The actual cause of the disease is unknown. We must assume that often 
a predisposition is present, which many and I also believe to be resident in 
the muscular and nervous systems. This predisposition is, therefore, the essen- 
tial part. The affection is endogenous; but it may be caused by external 
causes, above all by exertion and trauma. In how far other factors are oper- 
ative, such as improper nutrition, infectious diseases, intoxication, and the 
influence of cold, is unknown. 

Friedreich attached great importance to overexertion. He assumes that a 
condition which damages the muscular tissue in a nutritive and formative 
manner " by which a lessened resistance and a greater tendency to nutritive 
disturbances is brought about " is the cause. Even the " normal function," 
therefore, in children learning to walk is exertion in one predisposed and 
"acts as a pathologic factor"; just as Edinger has expressed this later for 
quite a number of diseases. This relative overexertion also explains why the 
affection in children appears first in the legs and back, while in later life 
the arms are first involved. However, it remains questionable whether, in 
early life, the legs are most subject to " exertion " ; besides we note some- 
times that walking and standing are impossible from the start, while the hands, 
which are certainly much used by children (even in the recumbent posture) 
are not affected. 

This factor is, therefore, not a satisfactory explanation; but it would be 
improper to deprive it of all value. It is remarkable that members of the 
working classes are especially affected by the " juvenile " form of dystrophy, 
which has its localization in the muscles of the shoulder and upper arm. It 
certainly cannot be doubted that a true hyperexertion affecting muscles that 
are already diseased is deleterious. 

We must not place too much reliance in the report of an accident case, 
that a trauma was responsible for a dystrophy which is already widely dis- 
tributed. In a case of this kind we determined positively that dystrophy, 
which was denied by the patient, had existed prior to the injury. Then also 
the disease may escape the observation of the patient for some time. It will 
hardly be believed how much may be accomplished with a defective muscle 



644 PROGRESSIVE MUSCULAR ATROPHY 

substance, which is even capable of undergoing hypertrophy. It is a well 
known fact that decided muscular defects may be found in the pectoral mus- 
cles of healthy workmen. Therefore if we desire to refer a dystrophy, scien- 
tifically to trauma, a normal condition of all muscles prior to the accident 
must have been present. 

Although this requirement cannot be fulfilled in any given case, there 
are, nevertheless, quite a number of instances — Erb recently collected several 
from literature — in which it is at least likely that serious trauma was re- 
sponsible for the appearance of the affection. On account of the great num- 
ber of accidents it is quite unlikely that a person suffering from dystrophy 
will be spared, especially as a dystrophic debility of the lumbar and pelvic 
muscles may be the cause of a fall or accident; nevertheless it would still be 
improper to deny, a priori, that a severe trauma might aggravate or give rise 
to the malady, especially if it affect the muscular or nervous systems. In 
regard to the action of trauma of this kind we cannot form a clear concep- 
tion, as the pathogenesis of the disease is unknown. 

It is now necessary to enter upon a brief consideration of the pathogenesis, 
on account of the importance of the question. Friedreich assumed that the 
primary element of dystrophy was a peculiar inflammation of the muscles, 
which is not rarely propagated to the peripheral nervous system and even to 
the spinal cord. Were this view correct, we could understand that trauma, 
which implicates a large mass of muscle in a predisposed person, might give 
rise to atrophy and then in some unknown manner, perhaps by propagation 
through the nervous system, distributes itself more widely. There is lacking 
in the typical cases of dystrophy the element which we recognize as true 
inflammation, an early implication of the vessels and of the interstitial tissue. 
Besides there would still remain the question, why an orderly and symmetric 
distribution of hypertrophy, pseudohypertrophy and atrophy should arise in 
consequence of progressive inflammation, which, under other circumstances, 
is so much more irregular. Finally the direct proof of the distribution of 
inflammation is entirely lacking. 

After what has been stated it cannot be doubted that on account of the 
persistent absence of anatomical lesions in the nervous system, disease of the 
muscles is the essential element. These exceptional cases in which, as I ob- 
served myself, the ganglion cells of the cord and also the peripheral nerves 
revealed marked atrophy cannot disprove it. In the first place these changes 
may be of a secondary nature, i. e., in consequence of the muscle atrophy and, 
secondly, there may be coordinative disturbances, in that the underlying un- 
known cause of the disease at the same time also attacks the motor nervous 
elements, so that these lesions may be due to complications. It is difficult 
to assume that these nerve lesions are of a primary nature because by atrophy 
of the same ganglion cells other symptoms are produced, as we shall see 
later on. 

Erb, for reasons that I cannot discuss here, has thought that functional 
changes of a dynamic nature might be at fault in the motor spinal ganglion 
cells, which produce a change in nutrition of the muscle fibers, so that finally 
a central disturbance becomes the cause of dystrophy. If these dynamic 
changes are very severe, a direct atrophy of the ganglion cells might result. 
If this view could be substantiated it could readily be understood that a 



PROGRESSIVE DYSTROPHY 645 

trauma, capable of producing shock, would greatly alter the function of the 
spinal, or in rare cases also of the bulbar, cells; this would give rise in pre- 
disposed persons to an extensive dystrophy. The chemical or physical com- 
position of the ganglion cells would be so damaged that the regulation of 
the nutrition of the muscle fibers would suffer seriously. Hence a condition 
resembling diabetes mellitus would be present in which lesions, not yet demon- 
strated, must be present in the composition of certain cells of the body; 
lesions which under the influence of trauma produce functional insufficiency 
and thence diabetes, and such a condition is not at all unlikely. Against Erb's 
view, in my opinion, it may be said that it is unnecessary to the explanation 
of dystrophy, for the assumption that there is an abnormal predisposition of 
the muscle fibers and that they degenerate is sufficient to explain the develop- 
ment of dystrophy. 

Further it is difficult to understand why the functional weakness of the 
ganglion cells upon prolonged duration of dystrophy does not ordinarily lead 
to atrophy, but only exceptionally, and why it is not usual for a secondary 
degeneration of the motor nerves to appear. Jamin recently, in very ingenious 
experiments on dogs " has not been able to demonstrate a special trophic action 
of the nerve centers upon the muscles except when they are stimulated to 
produce a contraction (motor function)/' Nevertheless we are not in a posi- 
tion to state anything definite in regard to these views and thus to reject 
definitely one or the other of them. 

If we accept the view of a primary myogenic development of dystrophy 
it becomes difficult to regard a local traumatic effect upon individual mus- 
cles, usually the case in trauma, as the causative factor of general symmetric 
dystrophy. If we believe that trophic changes in the spinal ganglia are at 
fault, the development of dystrophy from shock of the spinal cord substance 
with a predisposition already present could be understood, but this could 
hardly occur from local trauma of the extremities. For the present it is 
by no means certain that trauma causes dystrophy. That it may aggra- 
vate one that is already present, is easily conceived according to general path- 
ological laws. 

Treatment. — In regard to the therapy we are in the same unfortunate 
position as in other congenital pathologic diatheses — such as hemophilia and 
the hereditary nervous diseases — for we possess no specific remedies. In a 
prophylactic respect we must advise parents who have a dystrophic child that 
it is advisable not to rear any more children. Dystrophic males and females 
should be cautioned against marriage. 

It is of great advantage to recognize the affection as early as possible 
so as to prevent its progress and to avoid improper treatment. Cautious exer- 
cise and passive motion of the diseased muscles is most important. Although 
the patient must avoid prolonged walking if there is weakness of the legs, 
and manual labor when there is difficulty in the muscles of the shoulder, 
nevertheless methodic exercise of the individual muscles, if possible as pro- 
posed by Hoffa, upon command is desirable. These movements must be 
practiced in all joints, several times daily and at short intervals, so that 
exhaustion does not appear. In this connection passive gymnastic exercises 
may be employed, particularly to prevent the development of pes equinus, 



646 PROGRESSIVE MUSCULAR ATROPHY 

retraction of the muscles, flexor contractures in the knees and stiffness of 
the joints. To increase circulation in the atrophic muscles, warm baths and 
massage are suitable. The use of dry heat, or of Bier's stasis, is still prob- 
lematical. It is likely that the nutrition of the atrophic muscles may be 
increased by these means. The employment of electricity is of no value 
although it has been in this affection applied in all possible forms. In very 
rare cases in which the prolonged use of electricity is said to have brought 
about a cure there was most likely a diagnostic error. 

Orthopedic measures to improve the abnormal position of the foot con- 
sist of tenotomy, transplantation of muscles and tendons and in some cases 
the application of light corsets when the scapulae are far apart. According 
to Hoffa corsets are badly borne as a rule. I must strenuously advise against 
the use of extensive apparatus, especially a la Hessing, the same caution being 
also expressed by Hoffa. This heavy apparatus presses too much upon mus- 
cles which are already sufficiently damaged; besides they prevent the patient 
from employing his individual exercises. When there is much adipose tissue 
and great lipomatosis, suitable diet is necessary so that the body does not 
become too heavy, and in the late stages of the disease when the patient can 
no longer stand and walk that respiratory and circulatory disturbances do 
not appear. 

2. PROGRESSIVE NEUROTIC MUSCULAR ATROPHY 

(J. HOFFMANN) 

We must now discuss another variety of progressive muscular atrophy which 
is much rarer than dystrophy, and in t}^pical cases differs vastly from it; 
the forms will be described later on. I may be permitted to mention, in a 
historic review, so as to rectify some incorrect reports that I recognized this 
affection (which had been previously described, but associated with other 
maladies) in 1884 and I regarded it as a disease of the peripheral nerve trunks. 
I described it as a peculiar progressive atrophic paralysis of an infantile and 
hereditary nature. Later Charcot and Marie agreed in the main. J. Hoff- 
mann gave the affection a positive, short name, described and studied it 
thoroughly in all directions (1888 and later). 

Symptoms. — The clinical picture of the disease is quite simple; above all 
it has a hereditary and family character. It usually develops in childhood, 
frequently in youth, but it may develop during maturity even up to the 40th 
year. The symptoms develop in the distal portions of the members, usually 
first in the feet, more rarely in the hands or even simultaneously in all ex- 
tremities. According to Charcot it is likely that the small muscles of the 
feet are first affected, thereby producing certain, not very obvious, difficulties 
in walking. Later a distinct paralysis and atrophy of the muscles supplied 
by the peroneal nerve, with all the consequences develop: foot drop, steppage 
gait in consequence of pes varus or pes equinus. Naturally such patients are 
more liable to fall than normal persons. Club foot may attain such grades 
that the patient walks upon the dorsum of the foot. In addition to the 
peroneal paralysis there is a gradual development of atrophic paralysis of the 
muscles supplied by the tibial nerve, so that the leg is characterized by extreme 
leanness in comparison to the thigh (Fig. 181). After a year or several 



PROGRESSIVE NEUROTIC MUSCULAR ATROPHY 



647 



years there is an acute exacerbation in typical cases: the hands are then 
attacked. 

Atrophy and paresis of the small muscles of the hand supplied by the 
ulnar and median nerves with all of the consequences of this condition develop ; 
the muscles of the forearm gradually atrophy and are palsied. 

After a prolonged period the muscles of the upper arm and thigh are in- 
volved; but the muscles of the shoulder, trunk and face are not entirely exempt. 
Dubreuilh describes a mask-like change in the features, due to implication 
of the facial muscles. But the most essential is the early appearance of distal 
atrophies and paralyses. 

Electric examination of the muscles and nerves in certain stages of the 
affection reveals DeR in contrast to the exceptional and late occurrence in 
dystrophy. In the late stages of the disease there 
is naturally no electric response. An interesting 
condition observed by Ormerod, Hoffmann, and 
others, is this, that in the muscles which are not 
demonstrably diseased there is a decrease of con- 
tractility to both currents and, according to Hoff- 
mann, in the entire motor apparatus, nerves, as 
well as muscles. 

Fibrillarv, lightning-like contractions of the 
usual kind are absent; according to Hoffmann, 
and Charcot-Marie, there is a widely distributed 
contraction, or a permanent unrest, more of a 
choreic nature, which need not necessarily cease 
during sleep. Dejerine has observed ataxic phe- 
nomena; tremor has been noted in the muscles of 
the face; painful spasms in the calves is occa- 
sionally reported. 

The tendon reflexes are never increased, but de- 
crease gradually and finally disappear in the ten- 
dons belonging to the muscles showing atrophy. 
The cutaneous reflexes, and particularly the plan- 
tar reflex, are retained ; but they may be abolished. 
There is no report in regard to the abdominal re- 
flexes. The pupillary reflex to light was absent in 
a remarkable case of Siemerling; in a patient re- 
cently seen by me it was sluggish. 

Sensation is by no means permanently normal. 
Not rarely pains, even of a lancinating character, 
are present in the extremities, now and then even 
in the back. Sensitiveness to pressure over the 
nerve trunks is rare; hyperesthesia has only once 
been reported. Tactile hypesthesia, as well as 
hypalgesia (especially to farado-cutaneous stimulation) is particularly marked 
in the distal portion of the members. Hoffmann has also noted a slight 
decrease of farado-cutaneous sensation in the trunk and head. In a case re- 
ported by Charcot the muscle sense was disturbed; Hoffmann once noted 
Romberg's symptom. 




Fig. 185. — Xetjrotic Muscu- 
lar Atrophy. (Case of 
Schultze- Hoffmann.) The 
atrophy of the muscles of the 
hands is not clear enough. 



648 PROGRESSIVE MUSCULAR ATROPHY 

Vasomotor symptoms are present in the form of cyanosis and marbling 
in the distal portion of the limbs. Trophic disturbances are absent, except 
the arthritic changes due to paralysis and atrophy. In the case previously 
mentioned, I observed marked atrophy in the bones of the extremity, espe- 
cially the distal areas, therefore, an analogous condition to the osseous atrophy 
present in dystrophy of the muscles. The functions of the bladder, rectum, 
as well as of the brain, with the exception of slight psychical disturbance, 
of the heart, and organs of respiration, are normal. 

The course is very slow, with periods of arrest lasting years and decades. 
It is impossible for the patient to earn his livelihood on account of incapa- 
bility of using his hands. Death is due to some intercurrent malady. 

This description reveals the differences from dystrophy, which does not 
exclude the fact that in some atypical cases there may be great similarity in 
both diseases. Thus Oppenheim and Cassirer report a case in which the 
same localization of the atrophy was present as in the typical cases of neu- 
rotic atrophy with DeR, but with the involvement of the muscles of the face. 
According to the autopsy, however, it was a case of dystrophy with an unusual 
localization, for the nerves and the medulla spinalis were not involved. 

There is the greatest resemblance between the symptoms of polyneuritis 
and those of neurotic muscular atrophy; only the course of the former is 
more rapid and progressive. But the distal localization, the slight implica- 
tion of sensation, the pains which are sometimes present, DeR, the lack of 
bladder and rectal symptoms are common to both affections. Ataxia, and 
slight rigidity of the pupil to light, also occur in severe polyneuritis; these 
symptoms therefore give an appearance of ataxic disturbance. The slight 
alteration of the muscle sense, seen now and then, Romberg's symptom and 
the " stamping gait " observed by Hoffmann may be regarded in the same 
sense. Besides some forms of polyneuritis have been described as pseudo- 
tabes. Thus the pathology of the disease was regarded by myself and others, 
especially Dejerine, as a hereditary polyneuritis, which, on account of the 
involvement of the distal parts of the limbs, might be called an acroneuritis. 

The few anatomical investigations have shown, however, that besides an 
atrophy of the muscle fibers of varying degree with nuclear and late con- 
nective tissue increase, but without marked hypertrophy, and in addition to 
the peripheral nerve disease, there were peculiar lesions in the spinal cord. 
Particularly a degeneration of the posterior columns, such as we meet with 
in severe alcoholic neuritis; besides atrophic changes in the anterior horn 
cells, such as occur in some cases of lead paralysis which commonly also gives 
rise to peripheral nerve changes. Finally atrophic changes in Clarke's col- 
umns; in one case also lesions of the spinal ganglia and even in the lateral 
columns. The peripheral degeneration in the nerve trunk was always most 
severe in the periphery and in a case of Dejerine's it was associated with 
interstitial hypertrophy. As there are no reports of autopsies of the early 
stage of the malady it is impossible to determine which lesions are the first 
to arise, whether in the central cells, or peripheral nerves, or in both simul- 
taneously. The circumstance that there is DeR so early and regularly favors 
according to our present experience, a primary peripheral and not a primary 
central affection. 

But no matter how interesting we cannot enter upon a discussion of the 



PROGRESSIVE SPINAL MUSCULAR ATROPHY 649 

questions which these lesions force upon us. It appears to me to be the 
proper tiling to adhere to the name which Hoffmann has given the malady, 
for this prejudices nothing, although other terms have been proposed, such 
as the spinal-neurotic form of progressive muscular atrophy, by Bernhardt. 

It is clear that quite different lesions have been revealed than in dystrophy 
and for this reason (as is required by clinical observation) the separation from 
dystrophy is justified. 

Etiology. — Here even more frequently a hereditary predisposition has been 
found as the essential element; a debility which, in contrast to dystrophy, 
is to be sought in the nervous system. All other deleterious factors, such as 
exertion, infectious diseases, trauma, are only contributing causes, and in 
part may play a localizing role. In regard to the conditions in the parents 
of children with neurotic muscular atrophy, Hoffmann's disease, absolutely 
nothing is known. 

The prognosis is obvious from the description of the symptomatology ; cures 
are unknown. 

Treatment. — The therapy is the same as that of dystrophy. But orthopedic 
measures are more likely to be successful ; especially as regards walking. But 
with the progressive tendency of the malady this does not last long, as I had 
an opportunity of convincing myself in one of my cases. 



3. PROGRESSIVE SPINAL MUSCULAR ATROPHY WITH AND 
WITHOUT BULBAR PARALYSIS 

Awhile in the affection just described and also in dystrophy, the hereditary- 
family factors were early recognized, the occurrence of hereditary transmis- 
sion in the affection now to be discussed was discovered quite late. Werdnig 
was the first, in 1891, to point out this condition and it was again J. Hoff- 
mann who placed this new hereditary form of disease upon a broader clinical 
and anatomical foundation and distinguished it from the other varieties. The 
disease represents an infantile, hereditary form of spinal muscular atrophy 
and may be designated Werdnig-Hoffmanns disease. Its description very 
naturally follows that of neurotic atrophy. 

(a) INFANTILE HEREDITARY FORM OF PROGRESSIVE MUSCULAR 
ATROPHY (WERDNIG-HOFFMANN) 

Symptoms. — As in neurotic atrophy we are here concerned with a very 
rare affection, which usually appears in early childhood, during the first years 
of life. It is preeminently a family disease so that J. Hoffmann was able to 
note 19 cases in 3 families. The parents of these children were healthy; the 
birth of the children was normal nor could anything abnormal be discerned 
in them. In the course of weeks or months, without an assignable cause, the 
movements in the hip- joints become feebler and feebler. The child can neither 
flex nor extend the legs in the hip-joint, cannot abduct nor adduct them. Soon, 
also in an insidious manner, a feebleness of the muscles of the back and abdo- 
men follows, so that the child, that may have already learned to walk, which 
faculty it had lost, can neither sit up nor turn in the horizontal position. 
After months or years the muscles of the nape, neck and shoulders are attacked 



650 



PROGRESSIVE MUSCULAR ATROPHY 



and at the same time the atrophic paralysis of the legs descends. In conse- 
quence of paralysis of the muscles of the neck, the head of the child falls 
back when it is raised. Later the paralysis and atrophy implicate the mus- 
cles of the forearm so that the child is quite helpless, all the muscles of the 
trunk and extremities being paralyzed. The facial, hypoglossal, and fifth, 
nerves are not affected. Only once Werdnig saw incomplete closure of the 
eyelid during sleep in a child. 

The paralysis is flaccid. The muscle atrophy may be concealed by layers 
of fat; pseudohypertrophy is not present. If there be no fat there is a 

wretched condition of emacia- 
tion. Hypotonia gives rise to 
immoderate hyperextension in 
the joints upon passive move- 
ment, and the paralysis of the 
muscles of the back produces 
kyphoscoliosis. 

Electric examination re- 
veals decrease or absence of 
contractility, even DeR. Fi- 
brillary contractions are mostly 
absent but they may be very 
prominent; the tendon reflexes 
are abolished. 

Sensation, in the main, is 
normal; but sometimes pains 
are present. The nerve trunks 
are neither thickened nor sen- 
sitive to pressure. The func- 
tions of the bladder and rec- 
tum are normal as well as those 
of the brain, the special senses, 
the pupils, etc. Bulbar symp- 
toms are commonly absent; 
only Werdnig in his cases ob- 
served difficulty in deglutition. 
The course is more rapid 
than in the previously de- 
scribed varieties of atrophy; 
death is the result of disease of the bronchi and lungs, due to an involvement 
of the muscles of respiration. 

The majority of patients succumb in from 1-4 years after the onset of the 
affection. A patient of Bruns attained the age of 15. 

The necropsy, differing from the ordinary dystrophies, reveals very decided 
atrophy of the spinal motor ganglion cells and of their motor nerve roots 
and peripheral nerve fibers, therefore, of the peripheral motor neuron. This 
coincides with the clinical condition. In addition, atrophy of the muscles, 
much as in the experiments of Jamin, and analogous to those of Stier, charac- 
terized by simple atrophy of the muscle fibers up to complete disappearance. 
Even nuclear increase is lacking; hypervoluminous fibers are scant. Fatty 




Fig. 186. — Infantile Hereditary Spinal, Muscular 
Atrophy. (Case of Thomson and Bruce. Child 
aged five years and ten months.) 



PROGRESSIVE SPINAL MUSCULAR ATROPHY 651 

degeneration may be present, interstitial fatty cells are deposited. It is re- 
markable that the so-called " muscle spindles," peculiar structures which con- 
tain a number of muscle fibers, nerve fibers and vessels, structures whose nerve 
constituents are reckoned among the sensory elements, have also been found 
degenerated by Hoffmann. There are no other lesions in the sensory areas 
of the nervous system. 

The diagnosis, differential diagnosis and prognosis is evident from what 
has been stated. The cause can be assumed to be only an early damage of the 
peripheral motor neuron, especially of the motor ganglion cells, a so-called 
abnormal predisposition, which for some unknown reason proceeds in this 
manner. Why the ganglion cells of the muscles for the legs and arms are 
not first affected is not clear ; an especially marked and relative hyperexertion 
of the muscles of the hip and trunk cannot be proven. Infections and intoxi- 
cations have not been demonstrated. 

The treatment is ineffectual. 

In connection with this affection which is well characterized both clinically 
and pathologically there are some cases which have not as yet been thoroughly 
studied, for the anatomical analysis is lacking; they must, however, be men- 
tioned on account of their interest and for the sake of completeness. 

Facio and Londe have reported some cases in which a hereditary, bulbar, 
atrophic paralysis appeared in early childhood. Instead of the spinal, motor, 
peripheral neuron being affected, as in Werdnig-Hoffmann's disease, the bulbar 
tracts were attacked. There was atrophic paralysis of the facial nerve, con- 
spicuous atrophy of the tongue, difficulty in deglutition, paralysis of the vocal 
cords with complete and partial DeR, so that a primary muscular dystrophy 
must be excluded. 

Bernhardt and Striimpell have reported cases which resembled the ordi- 
nary form of progressive spinal atrophy; such as we shall describe subse- 
quently. In Striimpell's case there was anatomically, marked degeneration of 
the motor ganglion cells; in that of Bernhardt^ there was no autopsy. In 
both cases there was heredity; the disease appeared in adult life. 

We now come to those forms of spinal amyotrophy or spinal, progressive, 
muscular atrophy which were regarded for a long time as the most common 
and by its discoverers was given the name Aran-Duchenne' s disease. Later it 
was necessary to differentiate syringomyelia which was most likely the under- 
lying affection in the majority of so-called Aran-Duchenne's cases. Leyden 
isolated certain polyneuritic affections after Charcot had already defined amyo- 
trophic lateral sclerosis, an affection which we shall describe later on. 

Therefore we can hardly longer refer to Aran-Duchenne's disease, but it 
has become usual to consider it when the maladies just mentioned, besides 
dystrophy, neurotic muscular atroplry, and Werdnig-Hoffmann's disease can 
be excluded, and, further, when progressive muscular atrophy has a localiza- 
tion and a course that are now to be described. How rare is the malady in its 
nosologic limitation may be gathered from the fact that in the year 1886 I 
made a careful search through literature and could find but 9 cases; more 
cases were reported later and from a recent compilation by Lorenz, in 1904, 
there were only 9 additional ones. 



652 PROGRESSIVE MUSCULAR ATROPHY 

(b) PROGRESSIVE SPINAL AMYOTROPHY, ARAN-DUCHENNE TYPE 

Symptoms. — The affection develops in adults, beginning in the small mus- 
cles of the hand, therefore differing from .dystrophy and Werdnig-Hoffmann's 
disease, and, finally also from most cases of neurotic muscular atrophy. Either 
the muscles of the ball of the thumb or the interossei first become atrophic and 
accordingly weaker, particularly upon the right side. Gradually atrophy and 
weakness, which are parallel, implicate all of the muscles of the hand and 
produce a condition of the hand as in ulnar paralysis, the claw-hand, or the 
ape's hand, as in median paralysis, or any combination of these. Then the 
muscles of the shoulder are commonly involved; but those of the forearm 
may be sooner affected. In some instances paralysis of the radial nerve may 
be prominent from the onset. 

The muscles of the shoulders that are affected besides the deltoid are those 
that pull the arm down: the pectorales majores and latissimi, the trapezius 
and anterior serrati, supra- and infraspinati, the adductors and abductors of 
the shoulder blades. Gradually a high-graded emaciation appears in these re- 
gions with great groove-like depressions. The muscles of the nape of the neck 
may be affected; only late or in very rare cases are the muscles, of the lower 
extremity implicated. More often the bulbar muscles are affected, especially 
the lower facial branches and those supplied by the hypoglossal and pneumo- 
gastric nerves. Hypertrophy and pseudohypertrophy are lacking, particularly 
of the infraspinati and deltoids which are so often thus affected in dystrophy. 

The affected muscles which are simply soft but not coarse to palpation are 
not diseased in toto but only in areas in which, however, finally, complete 
atrophy may appear. In consequence of this state of affairs there may be 
function of these muscles for a considerable time. In the affected muscles 
there are marked and widely distributed fibrillary contractions and very often 
in those that are beginning to be diseased. 

In the later stages these may disappear; now and then, in spite of care- 
ful observation, they have been found absent. By these fibrillary contractions 
we understand lightning-like contractions of a small number of muscle fibers ; 
if larger areas are attacked fascicular contractions are spoken of. They may 
occur in health in consequence of the effect of cold, or under mental emo- 
tion ; but then they are noted in many muscles simultaneously. When consid- 
ering their pathologic importance both of the previously mentioned factors 
must be excluded. 

Spastic conditions, therefore stiffness of the muscles in walking or in the 
recumbent posture are almost never noted; the tendon reflexes of the affected 
muscles disappear gradually. 

Electric examination of the muscles, which have almost disappeared, reveals 
complete absence of contractility ; in those less involved all varieties of decrease 
up to complete DeR may be noted. Sensation remains normal', also the func- 
tions of the bladder and rectum. Symptoms referable to other organs are not 
present. The disease exclusively involves the spino-bulbar peripheral motor 
neuron. 

The malady runs a very protracted course lasting for decades. If the 
bulbar neurons are involved or the nerves of the muscles of respiration then 
death occurs in consequence of respiratory or circulatory disturbances. 



PROGRESSIVE SPINAL MUSCULAR ATROPHY G53 

The prognosis is, therefore, unfavorable. An arrest of the symptoms does 
not take place; marked permanent improvement or even a cure has not been 
noted. 

In regard to the cause, only a hereditary agenesis of the corresponding 
nerve divisions is known. Whether this is always present cannot be answered 
but it is very likely. Exertion has a localizing and aggravating influence. 
In regard to the effects of trauma nothing positive is known. Infections and 
intoxications have not been demonstrated. 

Treatment has a very limited sphere of action. Exertion of all kind must 
be avoided as this always aggravates the condition. By means of the cau- 
tious use of electricity, for a prolonged period, even though the treatment be 
interrupted, the muscles may be exercised. Massage may be employed in the 
same manner. Passive gymnastic exercises are necessary in those joints endan- 
gered by muscle atrophy. Local heat serves to improve the circulation, to 
decrease the coldness of the hands and exerts a favorable influence upon the 
disturbed nutrition of the muscles. Dry heat, as well as baths may be used; 
local as well as general. An attempt at relief may be made with the admin- 
istration of strychnin as advised by Gowers. 

The differentiation of this form of muscular atrophy from the typical 
cases of dystrophy or from neurotic atrophy (Hoffmann) and from Werdnig- 
Hoffmann's disease is easy. It may be difficult and for a time even impos- 
sible to separate it from syringomyelia when, as is sometimes the case, the 
sensory manifestations are absent. In this connection I must refer to the 
differential diagnosis of syringomyelia. Amyotrophic lateral sclerosis, with 
and without bulbar symptoms, may be mistaken for the affection but this 
will be described later on. 

Hypertrophic pachymeningitis of the cervical cord, a very rare affection, 
begins with radiating pain, which is often severe; tumors of the spinal mem- 
branes present the same symptoms. If pain is absent, which is sometimes the 
case in tumor, paresthesia and spastic paralysis of the legs are present, which 
are also noted in pachymeningitis; besides the progress of paralysis of the 
arms is much more rapid. Disease of the vertebra?, in the region of the 
neck, in addition to local and radiating pain in the neck, produces com- 
pression paralysis of the legs, quite apart from the deformities which soon 
develop. 

It has happened now and then that arthrogenous muscular atrophies have 
been mistaken for this affection, especially when disease of the shoulder- 
joints has been overlooked. It is true both affections, spinal muscular atrophy 
of the humero-scapular type and disease of the shoulder-joint, may occur con- 
jointly and the development of the latter may be favored by the primary 
atrophy; in arthrogenous atrophy the fibrillary contractions are absent, while 
in Aran-Duchenne's disease there are no pains in the shoulder-joints, not even 
upon passive movement of the latter, even backward and downward. 

Oppenheim pointed out that peripheral neuritic processes, such as arise 
after local overexertion from continuous or professional movements, may be 
confounded with the much more unfavorable prognostic condition of spinal 
muscular atrophy. This was the case in one of my own patients. As a 
rule a neuritis of this kind is unilateral, associated with paresthesia and hypes- 



654 PROGRESSIVE MUSCULAR ATROPHY 

thesia; but it is necessary in some instances at first to make a provisional 
diagnosis. 

It is impossible np to the present time to determine by clinical methods 
of differentiation whether, in cases of progressive muscular dystrophy, there 
is simultaneously a more or less decided atrophy of the ganglion cells; this 
condition has been frequently noted by Kahler, Erb, Strumpell, Heubner and 
myself. 

This brings us back to the discussion of the relations between atrophy of 
the ganglion cells and dystrophy, which was touched upon in the discussion 
of the pathogenesis of this disease. As it is difficult to assume that a pro- 
gressive atrophy of the ganglion cells at one time produces a dystrophy and 
at another time spinal progressive atrophy of the Aran-Duchenne type, it is 
also difficult to appreciate how a supposed dynamic change of these motor 
ganglion cells gives rise to the severest muscular dystrophies without a demon- 
strable alteration in their structure, while in other quite similar cases the 
structure suffers greatly. Hence the following appears to me to be the sim- 
plest solution: 

Under the influence of unknown deleterious agents, acting upon hereditary 
predisposition, the muscles at first appear to suffer alone and thus the picture 
of muscular dj^strophy in its various forms arises. But a degeneration of the 
motor neuron may appear in addition, which may also be the result of a 
hereditary predisposition. Such a combined system disease of the muscles on 
the one hand and of the motor nerve tracts and nervous apparatus on the 
other hand might be placed upon the same plane as the combined system 
diseases within the nervous system itself, such as Friedreich's ataxia or Hoff- 
mann's neurotic muscular atrophy, or other combined affections of this kind, 
perhaps also the combination of dystrophy and of the rare form of atrophy 
of the bones. Most likely these affections do not result simultaneously but 
are separated by an interval of time. 

If muscular dystrophy has advanced to any extent, atrophy of the motor 
ganglion cells can change the clinical picture but little, as for instance in 
Heubner's case. If this is not the case degeneration of the cells which sets 
in earlier may give rise to a combination of symptoms, those of dystrophy and 
of spinal atrophy; then DeR, fibrillary contractions and a different local- 
ization of the atrophy may result. This conception appears to me to be the 
most reasonable one but it has by no means been scientifically proven. 

A few words must be devoted to another affection which bears a very 
close resemblance to the Aran-Duchenne type of progressive muscular atro- 
phy, so that they have even been regarded as identical, namely chronic ante- 
rior poliomyelitis. As we do not know whether this affection has the same 
etiology as Aran-Duchenne' s disease and as there are certain clinical differ- 
ences it is better to describe these affections separately. 



(c) CHRONIC ANTERIOR POLIOMYELITIS 

Symptoms. — The few typical cases of this affection run their course much 
more rapidly than the Aran-Duchenne type of progressive muscular atrophy 



PROGRESSIVE SPINAL MUSCULAR ATROPHY 655 

and are not always progressive in the same sense. Just as in acute polio- 
myelitis an extensive paralysis develops in a few days and later becomes 
regressive so that finally there are but the remains of pathologic conditions, 
so also may chronic poliomyelitis, after advancing for months, become sta- 
tionary. 

In adults, in the course of weeks, an increasing debility may occur in 
one or the other extremity, without atrophy being noticeable from the onset 
as in Aran-Duchenne's disease. The terminal parts of the extremities are 
by no means alone implicated. Any muscle group may be involved. The 
paralysis is flaccid and permanently of a motor nature. Later on other ex- 
tremities are involved so that finally all may be paralyzed. The sequence of 
the paralysis varies. The muscles attacked finally become atrophic and usu- 
ally show fibrillary contractions. Electric examination reveals various forms 
of DeR; finally galvanic contractility disappears completely. The tendon 
reflexes in the involved muscle areas disappear; sensation as well as the 
functions of the bladder and rectum are normal. 

The course is usually this, that after the atrophic paralysis has spread 
for a few weeks or months, a period of arrest appears. In other instances 
the malady becomes progressive. The muscles of the trunk and of the neck 
are involved ; finally, in consequence of the paralysis of the respiratory nerves, 
difficulty in breathing appears, to which the patient succumbs after a previous 
period of immobility. The duration of these paralyses, according to Oppen- 
heim, is from 1-3 years. These cases, therefore, differ only from the Aran- 
Duchenne type by the localization and the rapidity of the course. 

Anatomically, there is mainly, as in the Aran-Duchenne type, an atrophy 
of the motor ganglion cells; whether the vessels, which may be thickened, 
are always secondarily diseased or whether this is only coordinated, or in some 
cases a primary involvement, cannot be answered. In a case reported by 
R. Ewald, there was, simultaneously, a decided thickening of the pia with 
cellular increase and thickening of the vascular walls, so that here there was 
an analogous condition to the lesions in acute poliomyelitis rather than to 
those of simple atrophy of the ganglion cells. 

In the differential diagnosis, polyneuritis must be primarily considered. 
In this affection there are commonly sensory irritative symptoms such as 
pain and paresthesia, also sensitiveness to pressure over the nerve trunks and 
the muscles. Later there are sensory phenomena of absence of function which 
are completely absent in chronic poliomyelitis; besides some etiologic factor 
may usually be discerned, such as alcoholism or the effects of arsenic. Lead 
paralysis presents the characteristic localization, the lead line on the gums, and 
lead colic. 

The differential factors, distinguishing this disease from dystrophy of the 
muscles, are obvious from the previous description. In regard to syringo- 
myelia I must refer to the description of this mafady; amyotrophic lateral 
sclerosis will be next discussed. It can always be differentiated by the spastic 
symptoms and by the increased reflexes from simple atrophy of the ganglion 
cells of the Aran-Duchenne type and from chronic poliomyelitis. 

In regard to etiology very little is known; hereditary influences have not 
been noted. Now and then in persons who have had acute poliomyelitis in 
childhood, chronic pathologic processes of the kind just described develop. 



656 PROGRESSIVE MUSCULAR ATROPHY 

Among the infectious diseases, in exceedingly rare cases only syphilis need 
be considered; of the intoxications only lead can come into question. The 
affection has been noted in the course of pregnancy. Nothing is known re- 
garding the effects of exertion and of cold. But shock, therefore severe trauma, 
according to the observations of Erb, may give rise to a clinical picture which 
closely resembles progressive chronic poliomyelitis, but this has not yet been 
proven anatomically. As shock, however, may produce minute chemical and 
physical alterations in the white substance, it is very likely that the ganglion 
cells — especially with an existing predisposition — may be so influenced that 
degenerative processes may gradually develop in them. 

In treatment we must insist, especially in the course of the affection, upon 
absolute rest. This is much more important than any chemical or physical 
action. Later when the symptoms are no longer progressive, when arrest 
has taken place, just as in the Aran-Duchenne type, the application of heat 
and all the other measures, such as the cautious use of electricity, massage, 
and of active and passive gymnastic exercises, may be employed. 

4. AMYOTROPHIC LATERAL SCLEROSIS AND AMYOTROPHIC 
PROGRESSIVE BULBAR PARALYSIS (CHARCOT'S DISEASE) 

I have already referred to amyotrophic lateral sclerosis, the disease with 
the long name, especially if we include with it amyotrophic progressive bulbar 
paralysis. For this reason the designation, Charcot's disease, has been chosen 
because this investigator was the first to define the affection clinically and 
anatomically. I may mention that I proposed the term motor tabes, as it is 
in direct contrast in the motor field to tabes dorsalis which implicates the 
sensory areas. Pierret proposes " tabes moteur " for the malady. However 
the term amyotrophic lateral sclerosis has found general acceptance, and it 
has the advantage of describing the lesion which is present in the vast major- 
ity of cases. 

The symptom-picture of the disease is quite distinct and very much resem- 
bles that of spinal progressive muscular atrophy. But besides atrophic paral- 
ysis of the upper extremity, which develops much more rapidly than in the 
Aran-Duchenne type, there is spastic paralysis of the lower extremities and, 
commonly atrophic paralysis of some of the bulbar nerves. The malady 
develops during mature age, but has been observed among brothers and sisters 
in childhood. It begins with gradually increasing feebleness of the upper 
extremities, which is often unilateral and may involve only one hand; then 
it proceeds to the forearm and upper arm and only later to the legs. In the 
upper extremity, in addition to the weakness, there is wasting of the muscles 
to which sooner or later paresis is added. The muscle wasting is associated 
with frequent contractions of the muscle bundles of a fibrillary as well as 
fascicular character; this sign appears, as a rule, first in the small muscles 
of the hand. Here and there these symptoms may be almost unilateral. Of 
sensory phenomena only paresthesia is present; tearing, excessive pains are 
very rare. Hypesthesia or even anesthesia is always absent; if present they 
are due to complications, such as hysteria. 

In the legs there is first retardation in walking and stiffness ; the patients 
are only able to take small steps; later the picture of spastic paresis and 



AMYOTROPHIC LATERAL SCLEROSIS 657 

spastic paralysis develops. If the atrophic paralysis is advanced in the hands 
and arms the claw position develops; the hands are pronated and flexed, the 
forearm is flexed. The arms are pressed toward the trunk. These abnor- 
mal positions are the consequence of rigidity and permanent contraction of 
the corresponding muscles. In the later course of the malady the muscles of 
the trunk and neck are affected; finally the head can no longer be raised, 
lowered, or rotated. 

Electric examination of the paralyzed muscles shows first a decrease of 
electric contractility as soon as the wasting appears, later there is often par- 
tial DeR, in some muscles even complete DeR. 

The muscles of the legs are implicated late and to a comparatively slight 
extent in the atrophy; Oppenheim saw a few instances in which there was 
early and great atrophy of the muscles of the lower extremities. The tendon 
reflexes are greatly exaggerated. There is bilateral foot-clonus, often also 
patellar and hand clonus, and even chin clonus. In all tendons, fasciae, mus- 
cle fibers, and muscles of the legs, there is decided contraction not only in the 
individual muscles but generally and also in the crossed groups. When iso- 
lated muscles are wasted entirely, the associated reflex also disappears. 
Babinski's reflex is nearly always present, i. e., isolated extension of the great 
toe upon irritation of different parts of the skin of the sole of the foot. 

The development of bulbar symptoms is of great importance. Sometimes 
at once, occasionally only after several years, there develops a disturbance in 
speech which is especially noticeable to the patient. Speech becomes slow, 
difficult, at first only transitorily, but later permanently. Sudden exacerba- 
tions are also noted. The faculty of modulation is lost; speech becomes 
monotonous, lalling, nasal and, finally, unintelligible and unarticulated. Any- 
one who has once heard this peculiar " bulbar " change in speech easily recog- 
nizes it. 

Upon close investigation the tongue is found altered. In the first stage 
of the malady the rapidity of its movements has suffered; but its size is still 
normal. Marked fibrillary contractures are observed in its muscles and then 
gradually atrophy appears, especially at the tip of the tongue. Finally the 
organ is greatly reduced in size, there are great groove-like depressions, and 
permanent glistening; it has lost the power of motion. 

The lower branches of the facial nerve are affected, and from their 
paralysis add to the disturbance in speech; this may be studied in all of its 
details, especially early, by having the patient recite the alphabet. We may 
thus readily determine in how far the lingual, labial, and other letters are 
involved. From debility of the muscles of the lips there is difficulty, and, 
finally, impossibility in puckering the mouth and closing it; therefore it 
remains open and the lower lip droops. As the orbicularis oris wastes the 
lips become abnormally thin. 

The muscles which raise the mouth and the alae of the nose become feeble 
and atrophic; this causes the naso-labial folds to disappear; the upper lip 
also droops. The patient can no longer show his teeth. Blowing and whistling 
are no longer possible for. finally, the muscles of expiration are also involved. 

In contrast to this affection of the lower branches of the facial nerve, 
in the greatest majority of cases the behavior of the upper branches is nor^ 
mal; however, in some cases these may also be affected. 
43 



658 PROGRESSIVE MUSCULAR ATROPHY 

In the area of distribution of the motor fifth nerve as well as in that of 
the hypoglossal and facial nerves the muscles of mastication are affected 
although not to the extent of the other muscles. The jaws can no longer 
be firmly closed. In addition to altered speech there is sooner or later diffi- 
culty in deglutition. As the soft palate is paralyzed, in consequence of dis- 
ease of the pneumogastric and glossopharyngeal nerves, in deglutition, espe- 
cially of fluids, the nasopharyngeal space and the cavity of the nose are 
inundated. The power of deglutition is finally lost. If, at the same time, 
the constrictors of the larynx are involved, food and fluids introduced into 
the mouth may find their way into the larynx and into the bronchi and lungs. 

Often there is also paralysis of the vocal cords, so that the voice is lost; 
the cough is then also toneless, and constantly becomes weaker, which is also 
accounted for by the debility of the expiratory muscles. 

In addition there are symptoms on the part of the heart, due to paresis 
of the vagus, such as periodic tachycardia, which may be associated with 
all forms of irregular cardiac action. 

A conspicuous symptom is the flow of large quantities of saliva from 
the open mouth; this may even occur in the early stage of the disease and if 
the patient is still able to use his hands he is kept busy with his handkerchief 
in removing the secretion. Whether the amount of saliva is abnormally in- 
creased or not, has not been determined. To increase the terror of the affec- 
tion, the mental powers are not involved. Intelligence is retained and the 
functions of the special senses are normal. Attacks of constrained laughing 
and weeping are to be regarded as automatic processes, as in multiple sclero- 
sis. The bladder and rectum are normal. 

The duration of the disease is shorter than that of dystrophy, neurotic 
atrophy and of the Aran-Duchenne type of myelopathic muscular atrophy ; it 
is estimated by Charcot at about from 2-3 years; but it may often be much 
longer up to 1Q years, even when bulbar symptoms are the first to appear. 
The insidious onset is exceedingly difficult to determine with accuracy. Ray- 
mond and Cestan recently calculated the average duration of their 18 cases 
at 28 months. The affection is alway fatal, death being due to deglutition 
pneumonia and a gradual increasing inanition. 

Typical lesions are found, in the majority of cases, in the central nervous 
system. Primarily there is atrophy of the large motor ganglion cells as well 
as of their nerve roots and the motor portion of the peripheral nerves-, this 
lesion in the cord is most prominent in the cervical portion. In consonance 
with the clinical symptoms, the ganglion cells of the hypoglossal nerve, the 
motor portions of the vagus, spinal accessory, and trigeminal nuclei, as well 
as some parts of the facial nerve are involved. The pyramidal tract, from 
the central convolution to the lumbar cord, is often degenerated; the deeper 
parts are always implicated, beginning at the crus cerebri. The remaining 
part of the anterior lateral columns in the cord is degenerated, while the 
posterior columns usually escape. Nevertheless Goll's columns have been found 
affected. Finally, Charcot and Marie have found degeneration of the large 
ganglion cells of the central convolutions from which the pyramidal tracts 
originate. 

The muscles present about the same lesions as in the infantile form of 
spinal muscular atrophy. 



AMYOTROPHIC LATERAL SCLEROSIS 659 

It has been found, in general, that when there was a clinical predominance 
of the spastic symptoms, degeneration of the pyramidal column tract was 
especially severe; when the muscle atrophy was profound there was marked 
degeneration of the ganglion cells. However, this parallelism does not always 
exist. There may be spastic symptoms with a lesion of the spinal portions of 
the pyramidal tracts and inversely there may be lesions of the pyramidal 
tracts without contractures. The rapidity in the appearance of the affection 
and its localization which at one time appears early in the pyramidal tracts 
at another time in the ganglion cells is responsible for this lack of parallel- 
ism. However, this does not explain everything; but I shall not discuss this 
very interesting theoretic point which at present is not of such great impor- 
tance. 

The etiology is still obscure. The statement that females are especially 
affected has not been proven. The age at which the disease develops most 
often is given by some at from 30-50, by others at from 35-50. A family 
incidence has been noted in children although the correctness of the diag- 
nosis in these cases has been questioned. As, however, simple atrophy of 
the motor ganglion cells is hereditary and shows a family appearance, and 
as lateral sclerosis, running its course with simple spastic paresis, occurs 
as a hereditary affection as is proven by Strumpell, it is very likely that the 
summation of these 2 symptom complexes, namely amyotrophic lateral scle- 
rosis is due to a hereditary predisposition of the peripheral as well as of the 
central motor neuron. But the agenesis of these neurons has not been proven. 
On the other hand it does not appear that infections or intoxications precede 
this affection. Overexertion may have a deleterious action and perhaps deter- 
mine the localization of the symptoms. Xevertheless the peculiar limitation 
of the malady to the lower branches of the facial nerve as well as to the 
lower motor nuclei of the bulbus is incomprehensible without the aid of aux- 
iliary hypotheses. 

Gowers and lately others (Oppenheim and Giese) have ascribed the affec- 
tion to the action of trauma particularly if mental shock and exertion were 
also present. As the actual cause of the disease is X it cannot be denied, 
a priori, that trauma, especially that form which affects the blood-vessels, such 
as great fear, may have an action upon this X. It is not unlikely, especially 
according to the rich material which has been accumulated, that it may have 
a causative influence upon the disease and certainly aggravate an already 
existing malady. 

Differential Diagnosis. — Charcot's disease may be differentiated from sim- 
ple trophy of the anterior horn cells by the presence of spastic symptoms 
and the exaggerated reflexes; from Aran-Duchenne's type by its more rapid 
development. 

Syringomyelia is characterized by the peculiar sensory symptoms ; only very 
rarely is there a cavity formation of the anterior gray substance with degen- 
eration of the pyramids alone, and correspondingly the symptoms of amyo- 
trophic lateral sclerosis. But even in these cases the atrophy of the muscles 
is not so symmetric as in Charcot's disease and the malady is much more 
prolonged. If the medulla oblongata is involved, the affection may be uni- 
lateral. 



660 PROGRESSIVE MUSCULAR ATROPHY 

Chronic myelitis of the cervical portion may give rise to very similar symp- 
toms but also presents sensory as well as bladder and rectal symptoms. Pachy- 
meningitis cervicalis, besides atrophy of the upper and spastic paralysis of the 
lower extremities, produces local pain which radiates into the arms and later 
there are sensory signs and absence of function, with bladder and rectal phe- 
nomena. The stiffness of the cervical vertebrae, in addition to the other 
symptoms, may be present in tuberculous and other maladies of the cervical 
vertebral column. Confusion with the other forms of muscular atrophy, dys- 
trophy, and neurotic atrophy, is impossible even though occasionally, in amyo- 
trophic lateral sclerosis, there is a lipomatosis of some few muscles. 

The symptom-complex of amyotrophic progressive bulbar paralysis may 
appear without spastic symptoms and just as it may be associated with sim- 
ple nuclear atrophy of the motor spinal ganglia, it may precede it and may 
even be present alone. The symptom-picture itself is not altered thereby; 
all these diseases, fully developed amyotrophic lateral sclerosis, and the isolated 
atrophies of the motor nuclei in the spinal cord and in the bulbus belong 
together and as far as we may judge have the same etiologic factors in com- 
mon, so that what has been stated previously in regard to bulbar paralysis 
requires no additional comment. 

In regard to the differentiation of amyotrophic bulbar paralysis, which 
often appears first or which alone may be present permanently from other 
maladies of the bulbus, it may sometimes be difficult to differentiate syringo- 
bulbia. This is rarely an isolated affection, presents sensory symptoms in the 
distribution of the fifth nerve and finally either unilateral or at least asym- 
metric disturbances in the motor tracts of the hypoglossal, pneumogastric, and 
spinal accessory nerves. 

Compression of the medulla from within or externally by tumors gives 
rise not only to motor and myotrophic symmetric symptoms but is often asso- 
ciated with pain and stiffness of the neck. Finally there are also the signs 
of cerebral pressure which are foreign to amyotrophic bulbar paralysis. 

Asthenic bulbar paralysis or myasthenia (Jolly), or Erb's disease, does not 
produce such typical atrophies as amyotrophic sclerosis nor is there DeR. On 
the contrary the myasthenic reaction is present and finally the rapid fatigue of 
the voluntary muscles is characteristic. The differentiation from some of the 
dystrophies of the bulbar muscles, particularly in children, may be very diffi- 
cult, but dystrophy also involves the muscles supplied by the upper facial 
branches and spares the smooth muscles of the pharynx. 

Finally diphtheritic or toxic paralyses of a different nature which affect 
the muscles of the soft palate and the pharynx must be excluded ; this is easily 
accomplished for these paralyses appear suddenly and do not show amyo- 
trophy. We must also by direct examination exclude purely mechanical diffi- 
culties in speech, deglutition, etc. 

Oppenheim calls attention to the possible confusion of rare localized forms 
with acute polioencephalitis of children; these may sometimes involve the 
bulbar nuclei. However the development of such an affection is neither in- 
sidious nor progressive, but rapid, sudden, and regressive. Besides the con- 
spicuous symmetry of the disorder, as in amyotrophic bulbar paralysis, is 
absent. 



AMYOTROPHIC LATERAL SCLEROSIS 661 

Treatment. — This is very difficult in these serious maladies. There is 
no specific and an attempt must be made to delay the disease and to prevent 
some of the sequels. The greatest care is necessary from the onset, and 
extreme moderation in indulgence of alcohol. The electric current is to 
be employed for the paralysis, especially in the case of the tongue and diffi- 
culty in deglutition. It is serviceable in producing deglutition movements 
with the aid of the galvanic current, by opening and closing the current. 
The internal use of tincture of nux vomica or strychnin hypodermically 
should be tried; arsenic and other nervines are of value. The spasm may 
be overcome by luke warm baths, mild massage, and cautious active and 
passive gymnastics. Roberant nourishment must be given as long as deglu- 
tition is still possible ; later, when there is difficulty in this direction, or paral- 
ysis of deglutition, the feeding tube must be employed. 

I hope that I have been successful in giving a brief and somewhat lucid 
description of our present knowledge of progressive muscular atrophy. An 
interest in these maladies is of practical importance to-day as these ques- 
tions are very liable to come up in the examination for life insurance and 
in accident claims. 

In regard to diagnosis the most important factor is a frequent examina- 
tion of the patient. We must never content ourselves with the demonstration 
that a progressive muscular atrophy is present for this is no more than deter- 
mining that a febrile condition or dropsy is present in a given case; it is 
therefore not a diagnosis. 

We must first examine the muscles of the entire body from head to foot. 
The presence of hypertrophy or of relative increase in size must be deter- 
mined and particularly the infraspinati and gastrocnemii should be examined. 
We must look for fibrillary contractions and the electric reactions must be 
taken. The mechanical contractility should be investigated, for in cases with 
sluggish contraction there is also a slow contraction upon direct galvanic 
stimulation; but one method is not a substitute for the other. 

Following this, the sensory functions are to be examined so as to deter- 
mine whether beside motor disturbance, therefore, in addition to dystrophy 
and atrophy of the muscles, there are also other anomalies of the nervous 
system, for instance, in the peripheral nerves, as in neurotic progressive 
atrophy, or in the spinal cord, as in syringomyelia. Then the tendon reflexes 
are to be examined, and this is of particular importance in the investigation of 
a central disturbance, particularly of the pyramidal tracts. 

After testing the other spinal, cerebral, and sensory functions and deter- 
mining the condition of the body organs it is generally easy, in most cases, 
to make a diagnosis of the different forms of the malady by following the 
description which has been detailed. 

In a small number of cases it is impossible to make a diagnosis at once, 
largely on account of the many transitional varieties, or better, mixed forms. 
The anatomical diagnosis must then remain open. But these difficulties are 
also encountered in other realms of diagnosis and they must not prevent but, 
on the contrary, they should stimulate us, to increase and widen our knowl- 
edge in all possible directions, 



PARALYSES OF THE PERIPHERAL NERVES 

By M. BERNHARDT, Berlin 

1. PARALYSIS OF THE FACIAL NERVE (MIMETIC PARALYSIS, 
PROSOPLEGIA, BELL'S PALSY) 

In" the last few decades the study of diseases of the nervous system has 
received such an impetus as to make it impossible, even for a specialist in 
this branch of medical science, to follow carefully and comprehensively all 
of its rapid advances. 

Although the study of diseases of the peripheral nerves, and especially 
their paralyses, forms only one branch of this science, yet because of the 
great variety of symptoms which it presents we have such a mass of material 
that we must presuppose a certain amount of endurance on the part of the 
lecturer, and inexhaustible patience and interest on the part of the listener, 
if this great and interesting branch is to be studied merely in the abstract. 
We may readily comprehend how the different peripheral nerves constantly 
innervate different groups of muscles, how the various nerve trunks or branches 
may be diseased, and by what very unlike symptoms these individual condi- 
tions are manifested. Without other prelude I shall begin at once with the 
subject under discussion, and shall present the most important points in the 
etiology of individual paralyses, their symptomatology, diagnosis, prognosis 
and treatment, and, with this end in view, I shall also describe several cases 
of facial paralysis (prosoplegia, mimetic paralysis of the face). 

Three patients of different ages, a girl aged 6, a young man aged 22, and an old 
lady aged 63, suffered from peripheral facial paralysis. In fact, facial paralysis (as 
also radial paralysis which is probably the most common form of peripheral paralysis) 
occurs at all ages, but it is most common in both sexes between the 20th and 50th 
years. Prosoplegia may also be congenital, as we shall see later. 

If these three patients are closely observed while the face is in repose, character- 
istic symptoms are noted; these, however, are much more distinct when imitative move- 
ments are attempted. On the paralyzed side the forehead is smooth and unwrinkled, 
the palpebral fissure in the eye of the affected side is larger and wider than upon the 
normal side; the eye cannot be closed. We may distinctly perceive that the eyeball 
is drawn first upward and inward, and then upward and outward, a movement long 
familiar to us, and first described by the celebrated English physician Bell, which, 
however, has recently again become especially interesting to investigators. Without 
entering into the numerous discussions stimulated by this phenomenon, to which the 
name of Bell's sign has been given, it may be stated that, at least in man, it is prob- 
ably due to an impulse of the will by which the eye (in the tract of the facial nerve) 
is closed, and by which also one or several impulses of innervation are conveyed to 
the muscles of the eyeball from the oculomotor nerve (the inferior oblique, perhaps 
also the superior rectus), while it has an inhibitive effect upon the tract of another 
branch traversing the same region (the levator palpebral superioris). 
662 



PARALYSIS OF THE FACIAL NERVE 663 

Defective vision in the eye which remains open ( lagophthalmos ) , of which these 
patients complain, is chiefly to be attributed to a flooding of the eye with tears. We 
note that the area near the nose on the paralyzed side is smooth, that the naso-labial 
fold is obliterated ; wrinkling the nose is impossible. The nasal opening is narrower 
upoa the paralyzed than upon the normal side, and the tip of the nose may deviate 
to the normal side. The angle of the mouth upon the affected side is lower than that 
upon the normal ; on opening the mouth only the healthy side of the upper lip rises, 
and by the unilateral action of the muscles of the lower lip (only upon the healthy 
side) the mouth appears to be crooked, much more so than when the face is at rest. 
Saliva flows from the angle of the mouth upon the paralyzed side; upon inflating the 
cheeks the air escapes from the paralyzed side; in consequence of paralysis of the 
muscles of the lips the patient cannot whistle, cannot blow out a candle, or can do 
these only from the paralyzed side. On account of deficient formation of the labial 
sounds, the speech becomes indistinct ; mastication is difficult, or, more correctly speak- 
ing, the proper revolution of the bolus and the power to force it from the mouth is 
limited on account of diminished or lost power in the muscles of the lips and cheeks; 
therefore it lodges in the paralyzed side of the mouth. 

When these patients attempt to put out the tongue, it appears to be closer to the 
paralyzed angle of the mouth than to the normal, and this is due to the crooked posi- 
tion of the mouth. At other times the tongue does not deviate to the paralyzed but to 
the normal side, as was first noted by Hitzig, and this is attributed to an unconscious, 
voluntary innervation in regard to the correct position of the tongue to the angle of 
the mouth under ordinary circumstances. The observation of Fr. Schultze that in 
facial paralysis the tongue is lower upon the paralyzed side than upon the normal 
could not be confirmed in my cases. 

Whether, under these conditions, as unquestionably sometimes occurs, there is a 
paresis of the stylohyoid and of the digastric muscles I cannot state. The probable 
implication in the paralysis of the muscles of the lobe of the ear, as can be readily 
demonstrated in man, is of only subordinate importance, while the implication of the 
platysma myoides, as recent observations have shown, is almost invariable. 

The changes in the facial expression of all patients with unilateral facial paralysis 
need no special mention. Psychical irritations, such as crying and laughing, lead to 
grimaces which, no matter how indifferent the individual may be, in the majority of 
persons suffering from them result in a feeling of profound depression and psychical 
alteration. 

While the majority of authors formerly assumed that the facial nerve influences 
the innervation of the soft palate, and that when this nerve is paralyzed changes in 
position and movement occur here as well as in the uvula, careful anatomical and 
experimental investigations have led to the opinion that the facial nerve bears no 
relation to the innervation of these structures. This nullifies a symptom previously 
regarded as significant in localization, as will appear later. The deviations in the 
function of the soft palate actually seen in a few cases of facial paralysis are probably 
due to the implication of the pneumogastric nerve, which nerve, as we know to-day, 
innervates the soft palate. The position of the uvula, which varies even in normal 
persons, is even less calculated to be used in the diagnosis of the localization than that 
of the soft palate. 

Since affections of the middle ear frequently cause disease of the nerves of the 
face, anomalies and disturbances in hearing are often found in facial paralysis. In 
severe affections with suppuration, caries, and necrosis of the middle ear, this compli- 
cation and its origin may be readily understood. Since, however, as will be explained 
later, many cases of facial paralysis follow the milder affections of the ear, the coin- 
cidence of slight deafness, of perverted hearing, etc., at the onset of facial paralysis 
is not surprising. 

Besides the anomalies of hearing which have just been named, in some cases of 
facial paralysis as, for instance, in the case of one of my patients, the faculty of per- 
ception of musical sounds and noises shows a peculiar change which was first described 
by Roux, Wolff and Landouzy, and which is known as abnormal acuteness of hearing, 
hypercusis Willisiana, oxyekoia. The disturbance is probably due to a lesion of the 
nerve above the point where the stapedius nerve branches off, and therefore in paralysis 



664 PARALYSES OF THE PERIPHERAL NERVES 

of the stapedius muscle, which diminishes pressure in the labyrinth; the compensatory 
action of the tensor tympani muscle no longer taking place, the tension in the tympanic 
membrane rises, and also simultaneously the pressure in the labyrinth ( Lucae ) . 

Another important symptom of localization is a disturbance of taste in the anterior 
two-thirds of the paralyzed half of the tongue. This anomaly affects the sen^ of 
taste as well as the reaction to galvanism; on the other hand, subjective disturbances 
of taste are occasionally perceptible by the patient as the first symptom of a subse- 
quent facial paralysis, and he so reports them. There is no doubt that the chorda 
ty«ipani supplies the fibers of taste in the anterior two-thirds of the tongue, while the 
posterior third and the base of the tongue, etc., are innervated by the glosso-pharyn- 
geal nerve. 

These taste fibers in the chorda tympani come from the trigeminal nerve through 
the spheno-palatine ganglion and the geniculate ganglion (of the facial nerve), and 
are conveyed by the vidian nerve to the facial nerve which then transmits these fibers 
for some distance in its trunk, and introduces them into the lingual nerve through 
the chorda tympani. These conditions will later be discussed in the description of 
paralysis of the fifth nerve. 

Less important and more rare are anomalies in the secretion of saliva, sometimes 
observed as the consequence of paralysis of the facial nerve. In some cases there is a 
decrease, in others (perhaps as an irritative symptom) there is a decided increase of 
the saliva furnished by the sub-lingual and sub-maxillary glands; of anomalies in the 
secretion of saliva from the parotid nothing positive is known. Some patients also 
report that their sense of smell is impaired upon the paralyzed side of the nose. This 
defect may possibly be due to an insufficient dilatation of the nasal opening, although 
in some cases we may certainly assume with v. Frankl that the same damage which 
has affected the facial nerve has simultaneously implicated the olfactory nerve. 

I have stated that patients who suffer from facial paralysis complain of dimness 
of vision; this is caused by the failure of the lacrymal tubes to drain off the tears, 
owing to paralysis of the muscles which close the eye. Goldzieher, Jendrassik and 
Koster have lately called attention to the fact that in a few cases of facial paralysis 
the secretion of tears is very slight or wholly lacking in the eye of the paralyzed side ; 
therefore, in crying tears flow only upon the healthy side. Although, up to this time, 
experiments in animals have failed to demonstrate the innervation of the tear ducts 
through the facial nerve, clinical observation in man seems to show, what Koster 
attempted to prove, that a lesion of the facial nerve in the geniculate ganglion pro- 
duces impairment of hearing as well as a diminished secretion of tears. We shall 
again revert to this. 

Besides the secretions of saliva and of tears a possible change in the secretion of 
sweat in the paralyzed half of the face has been described. We know that the secre- 
tion of sweat may be decreased as well as increased because, as Koster has shown, 
irritative and paralytic phenomena may appear conjointly. 

Disturbances in sensation may be objectively demonstrated in the paralyzed half 
of the face, as v. Frankl pointed out, but they are unimportant in the symptomatology 
of facial paralysis. I once called attention to a slight diminution of sensation in that 
part of the tongue which is supplied with taste fibers by the chorda tympani, but the 
symptoms produced by this are of little practical interest. 

The same is true of the slight vasomotor anomalies which, according to v. Frankl, 
manifest themselves in a certain puffiness and porcelain-like luster of the paretic half 
of the face. 

Trophic changes, especially in paralyses of considerable duration, may lead to 
wasting of the muscles of the face, and in rarer cases, as Schultze and Salomonson 
have reported, to slight changes in its bony structure. 

The appearance of herpes is among the most interesting but by no means common 
symptoms observed in connection with facial paralysis. This may assume the form 
of herpes occipito-collaris and, as we have reason to assume, be caused by the same 
infection, as yet unknown, which produces the facial paralysis or it is limited to the 
region of the trigeminal nerve and combined with facial paralysis. In these somewhat 
uncommon cases, the vesicles appear on the palate, on the uvula ? and on that half 
of the tongue on the paralyzed side, 



PARALYSIS OF THE FACIAL NERVE 665 

Owing to the frequency with which facial paralysis occurs, and the zeal 
with which this interesting affection has been studied for many years, it 
will cause no surprise when I state that the foregoing embraces but a por- 
tion of the symptomatology of facial paralysis. 

The question may be asked, How does peripheral facial paralysis make 
its appearance? 

In many cases the onset is apparently sudden, in others progressive, and 
in the course of a few hours or a day the paralysis gradually becomes com- 
plete. In my experience contractions are rarely observed prior to the paral- 
ysis which later affects the muscles. Frequently the patients complain of 
tinnitus aurium, of slight difficulty in hearing, of vertigo, of pain in the 
head and face, not rarely there is a mild febrile condition or general malaise, 
perhaps the signs of a general infection of the organism. 

That symptoms on the part of the ear should precede the paralysis is not 
to be wondered at if we bear in mind the prominent part which diseases of 
the ear play in the etiology of prosoplegia. 

I must call attention to a symptom which may either precede or accom- 
pany facial paralysis, and w r hich in my judgment has been somewhat over- 
looked in practice. This is pain in the head, in the ear, in the neck, over 
the forehead, over the temporals and zygomatic arches, etc., which most likely 
depends upon the implication of numerous sensory nerves (trigeminal, vagus, 
alosso-pharyngeal, and upper cervical). The significance of these pains, partic- 
ularly in facial paralysis, will be described when discussing the prognosis. An- 
other important point is their effect upon the patient, as well as their treatment. 

It sometimes happens, as I know from experience, that the patients do not 
mention their paralysis nor ask to have it treated, but they seek relief from 
the pain, frequently excruciating, which robs them of sleep, and may be 
felt for more than a week before the paralysis appears. 

The symptomatology of facial paralysis would be incomplete without a 
somewhat minute description of the state of electric irritability which has 
been especially studied in this form of paralysis. 

The following statements in regard to the electric irritability in periph- 
eral facial paralysis will apply equally well to all paralyses of peripheral 
nerves, no matter whether of the cerebral nerves, the nerves of the extremity, 
or of the trunk; thus unnecessary repetition will be avoided. I shall pur- 
posely refrain from discussing the more minute points in electro-diagnosis 
which, although exceedingly interesting and well worthy our close study, 
are of less practical importance to the physician in comparison with other 
conditions. Of course, those interested may study these rarer electrical changes 
in contractility from the literature of the subject. 

Until very recently the opinion was prevalent that in a so-called, mild, 
peripheral paralysis there were no changes in contractility; these paralyses 
in fact are called mild. We know that they may completely disappear in a 
few weeks. Opposed to these mild forms are the severe ones with which we 
have long been familiar, in which the electric contractility for the faradic 
as well as for the galvanic current gradually decreases after the second day, 
and in the course of from eight to twelve days response to either current 
is wholly lost. Since the pioneer investigations of Erb, these electrical changes 
have been designated by the term reactions of degeneration. 
44 



666 PARALYSES OF THE PERIPHERAL NERVES 

. The study of the reactions of degeneration is so extensive that their de- 
scription and presentation would necessitate a special chapter on electro- 
diagnosis. With reference to this subject, I will state that one of my patients, 
an elderly lady, had a left-sided facial paralysis which had existed for four 
weeks. In distinct contrast to the healthy right side, there was no reaction 
from the most powerful faradic current which could be borne upon the face, 
and upon indirect irritation the same was true of the galvanic current. On 
direct irritation with the constant current upon the healthy side the muscles 
showed a prompt and lightning-like contraction with two or three milliam- 
peres. This occurred upon the paralyzed side which did not respond to the 
faradic current, and followed a very much weaker current with a strength of 
only 0.5 to 1 milliampere and — a point to be especially noted — the contrac- 
tions were slow and sluggish. It was also evident that the preponderating 
effect of the cathode which is noted in normal muscles — cathodal closure con- 
traction as is well known occurs before anodal closure contraction — was here 
less marked than that of the anode, and cathodal opening contraction, which 
in normal muscles and nerves appears late and only with very strong currents, 
in this case soon appeared upon the diseased side and with a very feeble 
current. We know that such conditions last for weeks; what the action may 
be after recovery or after the paralysis becomes incurable, how it may be 
modified or changed, we shall not here detail. Nor shall we just now dis- 
cuss the importance of these reactions in making the diagnosis and prog- 
nosis, although we must mention a modification of electric contractility which 
has recently attracted the attention of investigators. 

Besides the mild and severe forms of facial paralysis which have just been 
described, there is, as in all other peripheral paralyses, a third which has 
been designated the medium because it occupies a place between the mild 
and severe forms. 

In this form indirect and direct faradic contractility are somewhat lessened 
on comparison with the normal side, as well as also indirect galvanic contractil- 
ity ; but it is not wholly lost nor even greatly diminished as in the severe forms. 
Besides a retained or only diminished direct faradic contractility, for about 
two weeks, sometimes even longer, we note upon direct muscle stimulation 
an increased galvanic contractility with sluggish contractions and an alter- 
ation of the normal electrical formula. I describe this form because it warns 
us not to be too sanguine in the diagnosis, and especially in the prognosis. A 
paralysis which appears mild for two or three weeks may in the third week 
assume a medium type, in which the prognosis is always decidedly better 
than in the severe form, but which does not permit the promise of speedy 
recovery. 

On the other hand, if we find eight or ten days after the onset of paralysis 
that the faradic contractility has almost entirely disappeared (I wish to 
state here emphatically that a test by the galvanic current is not absolutely 
necessary) we are dealing with a severe form of paralysis, recovery from 
which can only ensue after some months. When discussing the prognosis 
we will appreciate the importance of this for the family physician, and how 
it will protect him from making an erroneous prognosis, either too favor- 
able or too unfavorable. 

I cannot deny that competent authorities have recently expressed doubt 



PARALYSIS OF THE FACIAL NERVE 667 

as to whether there are any mild peripheral paralyses in the old sense, in 
which changes in electrical contractility do not arise. I cannot here discuss 
this very interesting question, but merely allude to it. 

Before considering the course, the duration, and the outcome of periph- 
eral facial paralysis, I shall briefly recapitulate what is known of the etiology 
of this common affection. It is apparent that wounds of the face, of the ear, 
in the region of the parotid gland, in the lower jaw, or of the skull, particu- 
larly fractures at the base, also inflammatory processes in the region of the 
parotid gland, may lead to facial paralysis. 

In the same sense we may include as trauma the lesions which occur dur- 
ing labor, either from pressure of the forceps or from the pressure of the 
child's head upon the bony pelvis of the mother. 

On account of the proximity of the facial nerve (which runs through 
the petrous portion of the temporal bone) to the tympanic cavity, it is evi- 
dent that this nerve is likely to be implicated by acute inflammatory processes 
of the ear as well as by chronic suppurations, by caries, and by necrosis. Al- 
though the view, held by French authors, that facial paralysis is almost 
invariably due to an inflammatory disease of the middle ear does not quite 
agree with the facts, I must state that the history of patients frequently 
shows that there was difficulty in deglutition for a few days prior to the 
paralysis, or dull pain in the middle ear, and this accounts for the assump- 
tion that an inflammatory process (for example, from the tonsils through 
the Eustachian tube) has attacked the middle ear. 

In the majority of cases, however, the patients tell us that they have taken 
cold. Refrigeration plays a great, if not the greatest, role with the laity in 
explaining the onset of an attack of facial paralysis. The question might 
be, and has been asked why, if chilling of the body is of such great impor- 
tance, is facial paralysis not more frequently observed than is actually the 
case? A number of authors assume that refrigeration is only an occasional 
cause, that the true origin is to be sought in a certain congenital or hereditary 
nervous predisposition of the affected person. As a proof of this, or, better, 
as foundation for this view, it has been alleged that facial paralysis occurs 
in families, among brothers and sisters, or in parents and children, and, fur- 
thermore, that certain persons are repeatedly attacked by paralysis of the 
seventh nerve. 

I have lately studied this so-called relapsing facial paralysis somewhat 
more in detail, and find that it occurs in about 7 per cent, of all cases. Usu- 
ally there is but one relapse, sometimes a second, a third, and, in rare cases, 
even a fourth. The same side is not always attacked; this may be the case, 
but the seat of the paralysis may vary, and sometimes the right, at other 
times the left side may be affected. In an electro-diagnostic sense these 
relapses are more severe than the original attack. 

In about 10 per cent, of the relapsing cases I found chronic inflammatory 
or purulent affections of the middle ear, 6.63 per cent, occurred in syphilitics, 
5 per cent, in diabetics, 13 per cent, in those with hereditary predisposition, 
but in the majority of cases, over 66 per cent., no causal factor could be deter- 
mined. No satisfactory explanation has been offered for the repeated appear- 
ance of facial paralysis in the same individual. 

In the etiology of facial paralysis syphilis plays an important role, in so 



668 PARALYSES OF THE PERIPHERAL NERVES 

far as pathologic processes and products at the base of the brain are concerned, 
such as gummatous tumors, fibrous and gummatous inflammations, etc. Other 
authors as well as I have observed that facial paralysis may occur in the early 
stages of syphilis; whether this is mere coincidence, or whether syphilis acts 
as a predisposing factor by diminishing the resistance of the organism, I cannot 
answer without further experience. 

The important role played in the last decades by pathological processes 
in the nervous system which have been closely studied and aptly designated 
by the term neuritis is well known. Unilateral and bilateral facial paralysis 
occur in acute and subacute neuritis, as well as in those diseases which during 
their existence have generated poisons (toxins) in the organism, have led to 
inflammation of various nerve tracts, and may also lead to similar affections 
of the nerves of the face. Among these maladies I must mention gout, diabetes 
mellitus, tonsillitis, diphtheria, influenza, during the course of which, or as a 
sequel, facial paralysis may appear. To these may be added diseases due to 
poisons introduced into the organism from without, such as alcohol, lead, 
toxic gases (carbonic oxid, carbon bisulphid), etc., all of which may be accom- 
panied by facial paralysis; hence there are many etiologic factors to which 
Bell's palsy may be attributed. 

I shall not describe the affections of the brain or spinal cord which are 
accompanied by facial paralysis as we shall refer to these when discussing 
the differential diagnosis; and as we shall subsequently describe briefly the 
hysterical and congenital (not those arising intra partum) facial paralyses, 
we shall now devote our attention to the course and outcome of peripheral 
facial paralysis. 

Mild cases of prosoplegia usually recover in from three to four weeks; 
cases of the medium form in a somewhat longer time, or four to six weeks. 
In the very interesting medium form, we may note that active motility (which 
is the main thing for the patient) begins to reappear early in the third or 
fourth week, while there is still decreased indirect contractility upon electric 
examination, and DeR may still be demonstrated with direct galvanic stimu- 
lation. By this time the patients often cease to come for treatment, although 
recovery is by no means as yet complete. In the severe form of paralysis 
recovery does not take place until three or four months have elapsed or even 
longer. Here, unfortunately, simultaneously with the return of active motil- 
ity, contractures are observed, and, according to my experience, these cannot 
always be prevented even by the most careful treatment. 

A patient, aged 45, who four months previously was attacked by severe right- 
sided peripheral facial paralysis due to cold, showed this condition of contraction. It 
was noted that the naso-labial fold on the diseased side which had at first been reduced 
had become deeper, that the mouth was no longer, as in the beginning, drawn toward 
the healthy but toward the diseased side, and that the palpebral fissure upon the dis- 
eased side had become narrower than that of the normal side. Under some circum- 
stances, and in this stage, it is sometimes difficult to decide which is the normal and 
which the diseased side. In these cases there are also other peculiarities. If the 
patient is told to close his eyes, the angle of the mouth upon the diseased side is raised 
and wrinkles appear in the chin. If the patient is asked to open the mouth or to 
move the lips, the palpebral fissure on the diseased side partially closes. Briefly, there 
are so-called coordinate movements not previously present. 

Moreover, there are brief, lightning-like contractions from time to time in the 
affected half of the face, apparently spontaneous. If we watch closely, we note that 



PARALYSIS OF THE FACIAL NERVE 669 

these lightning-like contractions invariably appear when the patient, like all of us, 
from time to time winks. Although I believe that in some cases these contractions 
occur spontaneously or, more correctly speaking, are due to unknown causes, they are 
usually coordinate with the closure of the eyelids. 

If these symptoms are very prominent in isolated cases, it may be that 
we are not dealing with paralysis but with a spasm of the facial muscles; 
careful observation, however, will reveal the true condition. Since we know 
that when a peripheral nerve is severely damaged not only the peripheral 
portion but also the center and the nuclear origin of the nerve (therefore, the 
entire neuron) are histologically changed, we incline to the view that the 
symptoms last mentioned are the consequence of changes in the facial nucleus 
caused by the long continued interruption of the nerve tract and the constant 
irritation of the center by endeavors to move the muscles of the face. The 
resistance of the cells is decreased and they react with abnormal ease. Their 
tonic influence upon the muscles is increased, the activity of one portion of 
the nucleus is transferred to other parts, and the cells show a tendency to 
spontaneous discharge. 

Thus we may understand that when recovery from a severe facial paral- 
ysis occurs in the course of six months, it is frequently incomplete. Some 
muscles never regain their normal motility, others remain spastically con- 
tracted, and where, as, unfortunately, sometimes occurs in isolated cases, no 
regeneration of the interrupted nerve conduction takes place, the paralysis 
persists. 

We now come to the important chapter of differential diagnosis. First 
we must be perfectly sure whether we are dealing with peripheral or central 
paralysis. To decide whether facial paralysis exists at all is not difficult after 
the foregoing description. The decision may be best arrived at by using the 
diagram (Fig. 1ST). If the lesion is above the point of origin of the facial 
(central or supranuclear paralysis), for example, at A. the majority of cases 
dependent upon lesions of the cerebrum show that the orbiculo-frontal branches 
are nearly exempt and only the nasolabial nerves are affected. Electric con- 
tractility remains unchanged, the reflexes are normal. Usually a simultaneous 
paralysis of the extremity accompanies the facial paralysis. But here, as 
elsewhere, this rule is not absolute; that deviations from the ordinary type 
occur cannot be denied; I should, however, digress too far from my pur- 
pose of mentioning only what is most essential and important were I to 
discuss minutely all of the symptoms which occasionally cause perplexity. But 
I may here mention that there are facial paralyses due to cerebral foci in 
which the muscles of the face on the hemiplegic side may be voluntarily moved, 
in which, however, the paralysis affects the motions which give expression 
to the face. In such cases, as Bechterew has demonstrated, the fiber tract of 
the cerebral peduncles is probably interrupted while the voluntary impulses 
(Xothnagel) still pass freely from the cortex to the periphery through the 
tracts at the foot of the cerebral peduncles. It may also be stated that, fol- 
lowing lesions in the cerebral peduncles, besides hemiplegia of the opposite 
half of the body and the implication of the facial nerve which is observed 
in other cerebral foci, there is simultaneously a characteristic paralysis upon 
the same side as the focus in the region of the oculomotor nerve (alternating 
paralysis). Lesions of the anterior portions of the pons, so far as hemi- 



670 



PARALYSES OF THE PERIPHERAL NERVES 



plegia is concerned, produce the same pathologic picture as other lesions of 
the cerebrum. If, however, the lesion is situated in the lower portion of 
the pons where the decussation of the facial fibers is already complete, while 
the tracts for the nerves of the trunk and extremities have not yet crossed, 
for example, at B, an alternating paralysis occurs (hemiplegie alterne) in 
which the paralyzed facial muscles upon the side of the cerebral focus show 
the same electro-diagnostic action which we have learned to recognize in the 



Cortical ceni 



medium forms, and which is 
never observed in facial paral- 
ysis due to lesions of the cere- 
brum. • 

We know that in chronic 
progressive bulbar paralysis, 
therefore in a nuclear lesion of 
the facial nerve, particularly 
the nerve branches of the face 
which supply the lips are im- 
plicated; pareses are simultane- 
ously present in the region of 
other bulbar nerves (the glos- 
sopharyngeal, the pneumogas- 
tric, the spinal accessory, and 
the hypoglossal nerves) so that 
with careful consideration it is 
not difficult to recognize this 
form of paralysis, and to avoid 
error. 

Let us assume that the diag- 
nosis of a peripheral facial par- 
alysis is clear. Next we deter- 
mine the exact location of the 
lesion in the course of the nerve. 
We must remember what was 
previously stated of disturbances 
of the secretion of sweat, of 
tears, of the saliva; also of the 
function of the soft palate and 
its very questionable depend- 
ence upon innervation of the 
facial nerve; also of disturb- 
ances in the sense of taste, after which exact investigation as to the presence 
or absence of any one of these symptoms will lead to the localization. 

The farther we advance in knowledge, the more surely will some of our 
theories be shattered and new principles be established. Fig. 188 shows a dia- 
gram first constructed by Erb, which has lately been modified in accordance 
with the investigations of Koster. 

Motor paralysis and possibly disturbances in the secretion of sweat follow 
a lesion seated below that point at which the chorda tympani branches. If 
the rupture of continuity is above this point but below the branch for the 




Fig. 187. — Diagram of the Motor Innervation 
Tract for the Facial Nerves and for the 
Nerves of the Extremities. Frontal Sec- 
tion THROUGH THE CEREBRUM, THE CEREBRAL 

Peduncles, the Pons, the Medulla, and the 
Spinal Cord. (Edinger.) 



PARALYSIS OF THE FACIAL NERVE 



671 



stapedius muscle, disturbances of the sense of taste and in the secretion of 
saliva are added to the other symptoms. If the lesion is above the point at 
which the stapedius nerve is given off, there is in addition a disturbance in 
hearing, a hvperacusis; an 
opinion which Koster did not 
share. 

According to Erb it was 
formerly assumed that a 
paralysis of the soft palate 
followed a lesion of the 
geniculate ganglion; or, more 
correctly speaking, paralysis 
of the velum palati (which 
can rarely be determined in 
facial paralysis) is due to a 
lesion in the region of the 
geniculate ganglion ; to-day 
we are convinced that the 
facial nerve bears no relation 
to the soft palate. On the 
other hand, according to Kos- 
ter, we may assume a lesion 
in this region if there is a dis- 
turbance in hearing caused 
by damage to the audi ton- 



damage 
nerve, and if the secretion of 




rtdfJ' 



Fig. 188. — Facial, Xerve from the Base of the 
Skull to its Exit at the Stylomastoid Fora- 
men. (Erb.) 



tears in the eye upon the 
paralyzed side is diminished 
or absent. When the seat of 
a lesion is above the geniculate, we have all the symptoms of absence of func- 
tion with the exception of disturbance of taste. 

The same is true of nuclear lesions, except that other symptoms char- 
acteristic of affections of the medulla oblongata are added. 

These are the points of view which at present permit the localization of 
peripheral facial paralysis. But we must bear in mind that science is 
continually advancing in new paths, and it is not unlikely that future 
investigations may modify this scheme which at the present time seems 
absolute. 

Pathology. — Finally, if we are asked the nature of the pathologico-ana- 
tomical changes in the supranuclear, nuclear and infranuclear forms of facial 
paralysis, we must refuse at this time to answer because a review of this 
subject would necessitate the description of the entire pathology of diseases 
of the cerebrum, of the pons, and of the medulla oblongata. 

Autopsy reports of purely peripheral paralysis were previously very scarce, 
but in the course of time have become much more numerous. From these 
we know that the changes in the nerves are, as a rule, purely degenerative, 
in the mild cases and those which soon recover mostly very slight, while 
in the severe cases (in which another cause or some other disease usually 
terminates the life of the patient) it has been found that degenerative proc- 



672 PARALYSES OF THE PERIPHERAL NERVES 

esses are present which have led to a subsequent degeneration of those portions 
of the nerve situated below the lesion. 

Before concluding this discussion I must state that the facial nerve is 
implicated not only by diseases of the brain itself but also by a number of 
pathological processes at the base of the skull (trauma, syphilis, basal tumors). 

In diseases of the spinal cord, of course more rarely than in diseases of 
the brain, the facial nerve may be affected, especially when the pathologic 
process is communicated by way of the cervical cord to the medulla oblongata, 
and there affects the nucleus of the facial nerve. This may be the case 
in amyotrophic lateral sclerosis and in spinal progressive muscular atrophy, 
it is the rule in chronic bulbar paralysis, it may occur in tabes dorsalis, 
and has been demonstrated in syringomyelia, more rarely in infantile spinal 
paralysis (acute poliomyelitis) and, a disease we rarely have an opportunity 
to observe, in leprosy. 

In the prognosis of facial paralysis we must differentiate between pure 
peripheral, so-called rheumatic, and refrigerating paralysis, and that form 
due to disease of the brain or at the base of the skull. Here the prognosis 
depends upon the extent of the pathological processes, and is usually less 
favorable. Syphilitic facial paralysis and that due to basal or cerebral, syphilis 
have a greater tendency to early recovery than that which depends upon nuclear 
disease. Furthermore, if the facial nerve is not destroyed by caries or by 
necrosis of the middle ear the prognosis of that form of facial paralysis due to 
simple inflammatory processes in the ear may also be regarded as favorable. 

In rheumatic paralyses the electrical condition of the paralyzed nerves 
and facial muscles forms a valuable guide to a correct prognosis. If we find 
on comparison with the normal side after the lapse of eight to ten days that 
the electric contractility is normal or but slightly impaired, we are justified 
in regarding the paralysis as of mild type and as likely to yield in from four to 
eight weeks. But here we must bear in mind what has been referred to before, 
the late appearance of DeR in the middle forms. A paralysis which was 
at first regarded as mild may under these circumstances be of longer continu- 
ance than was at first thought possible. If, eight to ten days after the appear- 
ance of facial paralysis, the contractility is found to be greatly diminished 
or absent (and I wish to emphasize that this may be demonstrated by the 
use of the faradic current alone) the case is severe, and three to five months 
or even longer must elapse before recovery can be expected ; even then, unfor- 
tunately, recovery is incomplete, inasmuch as contractures and other irrita- 
tive phenomena such as have been described are liable to appear. 

I cannot dismiss this subject without mentioning a symptom previously 
referred to; namely, the pain with which facial paralysis frequently begins. 
French authors (Testaz) have maintained that when the pain preceding or 
accompanying facial paralysis is slight or lasts but a short time the paralysis 
is apt to be slight and transitory, while under inverse conditions it will prob- 
ably be several months before recovery takes place. This is true in many 
cases, but is by no means the invariable rule. The opposite actually occurs, 
and I attach far greater significance in prognosis to the results of electrical 
examination than to the symptom just described. 

Treatment. — We now come to the most interesting and important part 
of our discussion, the treatment of facial paralysis. If besides facial paral- 



PARALYSIS OF THE FACIAL NERVE 673 

ysis we find syphilis, disease of the ear, trauma, diabetes, etc., our attention 
must first be given to these conditions and an attempt made to cure them. 
Whether induced sweating immediately before beginning the treatment, or 
the use of potassium iodid, of leeches, of hydropathic applications, is of spe- 
cial service, has been less definitely determined than the fact that the pain 
from which the patient may be suffering can be relieved by small doses of 
antifebrin or antipyrin (0.25, three times daily). (If seen early attempts to 
allay local inflammation in the throat, ear, neck or side of the head and 
face, to reduce congestion in the middle ear and to overcome any rheumatic 
element, are most strongly indicated. Hot continuous applications to the 
side of the head and neck and various preparations of salicyl have frequently, 
to all appearances, resulted in the immediate recession of the paretic symp- 
toms.) As is obvious from our discussion of the electrical reactions, this 
method of examination is absolutely necessary to enable us to determine in 
which category the paralysis in question belongs, and may be done if the physi- 
cian possesses merely an induction apparatus. Although it is true that mild 
cases may yield without treatment, on the other hand it has been proven that a 
stabile cathode treatment, applied if possible over the area of the supposed 
lesion, decidedly ameliorates the pathologic condition, particularly in mild 
peripheral paralysis. Although, as is generally recognized to-day, severe paral- 
ysis can hardly be influenced by treatment with electricity, nevertheless it is 
wise to give the patient such treatment, at least two or three times a week, 
the anode being placed over the mastoid process and the cathode applied 
to the diseased muscles of the face, currents of but moderate strength being 
employed (2 to 4 milliamperes) and electrodes of about 10 cm. in diameter. 

Certainly in some of the mild cases a moderately strong faradic current is 
very serviceable; yet, a very strong faradic current should never be employed, 
for, not only is it painful to the face, but in severe paralysis it is wholly with- 
out effect in the first month. So far as my experience goes, even with most 
careful use. it does not diminish the very distressing contractures which have 
been described; they should be treated by stretching and by massage of the 
contracted muscles, by mild faradization, perhaps by stabile anodal treatment. 

Furthermore, the patient should be required, particularly at the first sign 
of returning active mobility, constantly to innervate and utilize the diseased 
muscles. Cases which were supposed to be incurable, particularly those due 
to grave diseases of the ear, have lately been treated by French as well as by 
German surgeons (so far as I am aware up to this time only in animals) 
by severing the degenerated facial nerve at its point of exit at the stylo- 
mastoid foramen and sewing the central end of the spinal accessory nerve 
to the peripheral end of the facial nerve, thus producing a new innervation 
for the paralyzed area. As long as this difficult operation gives no positive 
results it is not advisable to resort to such measures, no matter how ingen- 
iously devised. 

In conclusion, I must refer to some interesting, although less common 
types of this affection. First, I will mention congenital facial paralysis. In 
these cases the child is actually paralyzed when brought into the world, and 
the disease does no't depend upon external damage to the face or to the 
nerves of the face occurring intra partum from injury during delivery. The 
following case furnishes an illustration: 



674 PARALYSES OF THE PERIPHERAL NERVES 

Soon after the birth of a child, which is now 10 months old, the mother noticed 
that it could not suck, it did not cry, did not laugh, but the face remained rigid like 
a mask. The mouth was continuously open, the lower lip was curved somewhat down- 
ward, the eyes did not close and were full of tears, but they rotated freely in all direc- 
tions. The child was well formed, and except for bilateral facial muscular paralysis 
there was nothing abnormal in its body; it never suffered from spasms. 

Congenital facial paralysis or, more correctly expressed, a defect in the 
musculature of the face occurs bilaterally as well as unilaterally. Paralysis 
or a defect in the muscles of the eyes is often simultaneously present; more 
rarely there are paralytic conditions or defects in other cerebral nerves (the 
hypoglossal). In the face the ifiuscular tissue disappears either wholly or 
partially. In many cases the muscles of the lips and chin are absolutely 
or more or less exempt (here only the muscles of the lower lip on the right 
side react to strong currents and feebly to intrabuccal irritation, the con- 
tractility of the remaining muscles of the face disappearing completely), 
while in other cases which have been reported the muscles of this region 
alone were implicated, or at least these chiefly. It is not always easy, espe- 
cially with adults, to decide whether paralysis is congenital or peripheral, 
or whether it came on in early youth. At the present time, We are aware 
of but one anatomical investigation of a case of this kind, that of Heubner, 
who found a very extensive aplasia of the motor cerebral nerve nuclei as 
well as of the left olive, and a very meager development of the left pyramid. 
I must state, however, that congenital facial paralysis may be combined 
with other congenital defects and deformities as well as with paralysis of 
the ocular muscles (deformity or absence of fingers or phalanges, web-feet 
or web-fingers, defective development of the muscles of the chest), and that 
this anomaly may appear in one or several members of the same family. 
The abnormality is not always seen in the same muscles, but the conditions 
may vary; these data form the most important features of the disease of 
which, at present, we are aware. 

An accurate history, the consideration of the points just enumerated, the 
absence of sensory disturbances, the disappearance of electric contractility, 
or its great diminution, will lead us to a correct diagnosis. After the fore- 
going remarks I need scarcely mention that there is no satisfactory treatment 
of this congenital affection. 

A special description is also required for bilateral facial paralysis. In 
regard to one form, the congenital, I have already given the important 
points, and we shall now consider bilateral peripheral facial paralysis, which 
is subdivided into two groups according to whether the cause of the paralysis 
is at the base of the skull or has its seat external to this. As causal factors 
for the first category, trauma is important, then syphilitic, carious, or necrotic 
processes or tumors at the base of the skull. Furthermore, a number of 
bilateral facial paralyses have been reported in literature for which no other 
etiology could be determined than that of the unilateral forms ; i.e., refrig- 
eration or unfavorable atmospheric conditions. A bilateral affection of the 
facial nerves may occur in the course of polyneuritis. In such cases, almost 
invariably, one facial nerve is implicated and paralysis of the other follows 
in a short time. 

In either form the severity of the lesion varies, as well as the presence 



PARALYSIS OF THE FACIAL NERVE 675 

or absence of certain symptoms. Double facial paralysis may be due to a 
bilateral cerebral lesion, to a lesion of the pons, or of the medulla oblongata. 
Here I must refer to the central form of labio-glosso-pharyngeal paralysis, 
to pseudobulbar paralysis, to the nuclear form of diplegia facialis in chronic 
progressive bulbar paralysis, finally, to those forms of bilateral paresis of the 
seventh nerve which resemble the symptom-complex of asthenic bulbar paral- 
ysis or myasthenia pseudo-paralytica gravis. 

I shall not discuss in detail the differential diagnosis, as I have already 
referred to the supranuclear, the nuclear and the infranuclear unilateral 
forms of facial paralysis. 

A detailed history, the investigation of the condition of other cerebral 
nerves than the facial, the possible implication in the paralysis of the muscles 
of the extremities, finally, the retained or but slightly impaired electric 
contractility in cerebral prosopodiplegias will aid us in differentiating these 
bilateral cerebral palsies from those of purely peripheral origin. 

Another form of paralysis is the disease described by Duchenne, which 
was later referred to by other French as well as German authors, and which 
is scientifically known either as progressive muscular atroplry of children 
or as progressive atrophic myopathy ( f acio-scapulo-humeral type) ; the mus- 
cles on both sides of the face are implicated in the atrophic process. 

Finally, those cases of bilateral facial paralysis are especially interesting 
in which the paralysis upon the side of the lesion differs from that of the 
other side. Thus, as I observed in a child, a cerebral focus arising in the 
course of diphtheria may produce hemiplegia and a simultaneous cerebral 
facial paralysis, while disease of the middle ear due to the same affection 
may cause a complete peripheral paralysis of the facial nerve upon the non- 
hemiplegic side. Still other remarkable combinations have been observed; 
for instance, as in a case described by Oppenheim, hemiplegia due to cerebral 
syphilis developed in a person who subsequently had a purely peripheral 
paralysis of the facial nerve upon the same side as the hemiplegia, the palsy 
being due to a basal, gummatous meningitis. As a rare occurrence I shall 
mention a case, which so far as I know is unique, of bilateral facial paralysis 
in a child which, no doubt, resulted from the pressure of the forceps during 
delivery, and the rare cases of prosopodiplegia in so-called head tetanus in 
which the wound in the face is in the median line, also a case of diplegia 
facialis, only one of which has been seen, the person having been bitten by 
a supposedly rabid dog. 

In the clinical picture of diplegia facialis there is no unilateral displace- 
ment, no contortion of the face to one side; but the face looks like a mask, 
since no psychical stimulation whatever produces in it any movement. The 
eyes are always open and full of tears, and upon trying to close the lids 
Bell's symptom, previously described, appears bilaterally; the forehead is 
unwrinkled. Mastication and speech are difficult. Labial consonants and 
vowels can no longer be enunciated; saliva and mucus dribble from the 
mouth which the patient cannot close. 

The treatment is along the same lines as that described for unilateral 
facial paralysis; but the prognosis of bilateral facial paralysis is more un- 
favorable. 

Before closing this description I must devote a few words to hysterical 



676 PARALYSES OF THE PERIPHERAL NERVES 

facial paralysis, in which there is true paralysis, or, more correctly, paresis 
only in the rarest cases; on the contrary there is a peculiar spasm of the 
muscles of the tongue and of the lips, sometimes upon the side opposite to 
the hysteric hemiplegia, sometimes upon the same side. There is still much 
skepticism regarding these conditions which are more common in France 
than elsewhere, and I shall therefore not discuss this form of paralysis. 
From the foregoing description it is evident that facial paralysis is one 
of the most interesting and, at the same time, one of the most common 
and intricate conditions in the pathology of peripheral paralyses. 



2. PARALYSIS OF THE TRIGEMINAL NERVE (FIFTH NERVE) 

A. PARALYSIS OF THE PORTIO-MINOR; MASTICATORY FACIAL PARALYSIS 

(ROMBERG) 

Paralytic conditions involving the fifth nerve, particularly those which 
implicate only its motor portion, are very rare. Although in the majority 
of these cases the motor portion of the nerve is implicated simultaneously 
with the sensory parts, it will conduce to a clearer comprehension of the 
subject if we make an artificial division, and separate masticatory facial 
paralysis (Romberg), or paralysis of the portio-minor, from that form which 
chiefly implicates the sensory portion of the nerve. 

It is well known that the motor portion of the fifth nerve belongs to the 
third branch and especially supplies the muscles of mastication. The soft 
palate also receives a small branch which extends to the tensor veli palatini 
as well as to the tensor tympani. 

We have little accurate knowledge of supranuclear central paralysis of 
the muscles of deglutition due to lesions of the cerebrum; I shall, therefore, 
at once turn to those nuclear forms due to injury of the nerve in its intra- 
pontine course or to lesions of the pons. These are common, and impress 
their characteristic stamp upon acute, subacute, or chronic bulbar paralyses. 
Here we must also refer to that disease which has lately so engrossed the 
attention of pathologists, myasthenia pseudo-paralytica gravis, or asthenic 
bulbar paralysis without anatomical lesion, in which the weakness of the 
muscles of mastication, in particular, forms an important clinical symptom. 

When I mention that paralysis of the muscles of mastication occasionally 
occurs also in the course of tabes dorsalis and syringomyelia, I have 
exhausted the etiologic factors of a purely nuclear paralysis of the motor 
division of the fifth nerve. [It also occurs in bulbar palsy. — Ed.] 

In considering the etiologic factors - of paralysis of the trigeminal nerve, 
especially of paralysis of the muscles of mastication which peripherally affects 
the nerve, two points are specially significant. 

The first of these is the great rarity (in contrast with peripheral facial 
paralysis) of so-called rheumatic paralysis (we find but few reports of this 
in literature) ; the second is the relative rarity of traumatic affections (wounds 
of the base of the skull from a fall, or injury to the face except stabbing 
or gunshot wounds). Of course, all pathologic processes affecting the base 
of the skull, especially its middle grooves, which implicate the bone, the 
periosteum, and the membranes of the brain, injure the nerve in its infra- 



PARALYSIS OF THE TRIGEMINAL NERVE 677 

nuclear course just as do pathologic formations which proliferate from the 
spheno-maxillary fossa into the cavity of the skull. 

A congenital absence of both of the upper branches has very rarely been 
observed, and just as uncommon is atrophy of the muscles of mastication; 
in one case this may be combined with atrophy of the subcutaneous fatty 
tissue, in another case with hemiatrophia facialis. 

Limitation of the movements of the jaw and a difficulty in deglutition 
are naturally the most prominent symptoms of paralysis of the motor portion 
of the fifth, nerve. The rise and fall, as well as lateral motion, of the lower 
jaw, are naturally absent, and in bilateral paralysis this symptom is markedly 
developed; disintegration of solid food is almost impossible. 

In myasthenic bulbar paralysis the masseter and temporal muscles may 
perhaps be palpated and atrophy is not very prominent, but weakness of 
these muscles is evident, for they soon become tired and force the patient 
to desist from mastication and to stop for rest. In regard to the electric 
reaction of the muscles of mastication, a few reports demonstrate that the 
atrophic muscles have either lost their contractility or, under galvanism, 
they show DeR. 

In a few cases of paralysis of the fifth nerve, the posterior arch of the 
palate upon the diseased side was observed to be low down, while the anterior 
was symmetrically curved on both sides. Xo severe disturbances of hearing 
have been noted in paralysis of this nerve, even on complete extirpation of 
the Gasserian ganglion, as was demonstrated by F. Krause, who performed 
this operation to relieve severe trigeminal neuralgia; temporarily the patient 
complained of noises in the ear of the diseased side, that is, the side that 
had been operated upon. 

B. PARALYSIS OF THE PORTIO-MAJOR 

A glance at the illustration, Fig. 189, will reveal the portions of the 
face and head supplied by the three branches of the trigeminal nerve which 
contain sensory fibers. Besides the skin, the mucous membrane of the eye, 
of the nose, of the mouth, of the tongue, of the tonsils, of the palate, of the 
Eustachian tube and of the tympanic cavity, also the pulp of the teeth, 
the muscles of the eye-ball and of the face, and the dura mater contain 
sensory fibers. 

The majority of etiologic factors which produce trigeminal paralysis affect 
the motor and sensory fibers simultaneously; but it will conduce to a more 
accurate knowledge of these conditions if we present the symptomatology 
of paralysis of only the sensory portions of the trigeminal nerve separately. 

The main symptom is the hyperesthesia or anesthesia of the affected 
area, which is gradually accompanied by abnormal subjective sensations. 
First, in the areas of the skin and mucous membranes which we have men- 
tioned there is sensitiveness on contact, and later, when the affection has 
advanced, or even immediately if the lesions are severe, sensibility to pain 
may be lost. Some points which Krause observed in his subsequent exam- 
ination of patients upon whom he had operated, and which were confirmed 
by the anatomists Frohse and Zander, are of importance. First, that the 
middle regions of the skin of the face on both sides receive sensory fibers 
from the fifth nerve; secondly, as may be seen from the illustration, on the 



678 



PARALYSES OF THE PERIPHERAL NERVES 



lateral parts of the face branches of the cervical nerves unite with those 
of the fifth. Thus it is evident that, even with severe lesions, sensation is 
not entirely obliterated. 

In severe anesthesia the reflexes of the skin and mucous membrane are 
absent; the secretion of tears may be suppressed, and the sense of smell 




Fig. 189. — Diagram Showing the Distribution of the Sensory Cutaneous Nerves of the 
Head. (After Frohse and Krause.) 

The region of the first (1) and third (3) trigeminal branch shaded, region of the ramus auricularis 
nervi vagi in the concha auris black. 1, first trigeminal branch; 2, second trigeminal branch. 
a, N. inf ratorbitalis ; b, N. zygomatico-f acialis ; c, N. zygomatico-temporalis ; 3, N. auriculo- 
temporalis; 4> N. occipitalis magnus; 5, N. occipitalis minor; 6, N. auricularis magnus; 7, 
Nn. cervicales posteriores ( dorsal es) ; 8, Nn. cervicales lateral es (ventrales) ; 9, N. auricularis 
vagi. 

may surfer owing to deficient moisture in the nose on account of the impli- 
cation of the mucous membrane. 

I shall not here discuss the question whether the fifth nerve, as has been 
assumed, is really the nerve which influences the secretion of the tears or 
whether this function appertains to the facial nerve. In some cases of iso- 
lated paralysis of the fifth nerve the secretion of tears has been found per- 
fectly normal, and when it ceased it could be determined (as it has, for 
instance, in the case of total extirpation of the Gasserian ganglion) that 



PARALYSIS OF THE TRIGEMINAL NERVE 



679 



the large superficial petrosal nerve (from the facial nerve) was injured 
either by the operation or subsequently by the formation of cicatrices. 

In deep anesthesia of the skin of the face, impaired movement of the 
muscles of the face has been noted in spite of an absolutely intact facial 
nerve; this was observed by Bell and Magendie in animals that had been 
operated upon, and was recently confirmed by the investigations of Filehne 
and Exner. The patients feel as if their faces were covered by a mask 
(implication of senso-motility, Exner) ; when implements are placed in the 
mouth or upon the tongue the patients either do not feel them or this sensation 
is indistinct and, with a unilateral affection, they appear as if broken; even 
mastication is decidedly interfered with on account of the diminished sensa- 
tion in the mucous membrane of the mouth. I must mention in passing 
that in unilateral trigeminal paralysis, just as in unilateral affections of 
the facial and hypoglossal nerves, the tongue may be coated upon the side 
on which there is anesthesia. 

Like facial paralysis, our interest in paralysis of the trigeminal nerve 
is not exhausted by discussing the anomalies of motion and sensation. Dis- 
turbances of taste and occasional trophic lesions also invite our attention. 

At this point no exhaustive description can be given of the fibers of taste 
and their course to the central nervous system. Physiologists and clinicians 
have long assumed that the sensation of taste is conveyed through the lingual 
nerve from the third branch of the trigeminal nerve, and that the general 
sensations of the anterior two-thirds of the tongue are simultaneously produced 
in the same way. If the illustration (Fig. 190) is examined, we will see 




Fig. 190. — The Facial Nerve and Its Communications with the Trigeminal and Glosso- 
pharyngeal Nerves. (W. v. Leube.) 



before us the combined results of the latest researches of F. Krause and 
the neurologists, v. Frankl and Cassirer, as to the supply of individual 
portions of the tongue with fibers of taste. The fibers of taste for the 
anterior portion of the tongue are in the basal portion of the trigeminal 
nerve; in rare cases the whole tongue receives its fibers of taste through this 
nerve. In some cases no taste fibers are found in the basal portion of the 



680 PARALYSES OF THE PERIPHERAL NERVES 

trigeminal nerve; certainly in the basal portion of the facial nerve there 
are none. In the overwhelming majority of cases the glosso-pharyngeal nerve 
supplies the posterior portion of the tongue with taste fibers, and, in some 
cases, also the anterior portion. 

Authors admit that the individual variations are so great that we should 
in all cases belonging to this group make a very careful examination, and 
then differentiate them without bias. 

We shall now turn to the " trophic disturbances" and among these the 
so-called ophthalmia neuro-paralytica, which is of particular importance, will 
first occupy our attention. 

Since Magendie long ago showed that division of this nerve in the brain 
of a rabbit may produce an inflammatory affection of the cornea and eventually 
an ulcerative process in this region, the fact has been clinically demonstrated 
countless times in man. We would digress too far were we to enumerate 
the various theories which have been suggested by authors in explanation of 
this process; on the contrary, I will mention only the conclusions of the 
most recent investigators, experimenters as well as clinicians. It has been 
demonstrated that the eye on the side operated upon, or the side affected 
by disease of the trigeminal nerve, offers diminished resistance to external 
deleterious effects, but that the anesthesia assumed by some authors does 
not explain the appearance of the affection. Most likely the injured or 
diseased nerves are in an irritative condition due to neuritis, so that a dis- 
ease of the cornea associated with sensitiveness may occasionally be noted, 
and, upon the other hand, in spite of existing anesthesia the cornea may 
remain exempt because such irritative conditions are absent. 

Herpes is one of the symptoms noted in disease of the Gasserian ganglion, 
or in the path of one of the three branches of the trigeminal nerves, zoster 
ophthalmicus being the most familiar form; by its extension to other struc- 
tures of the eye this affection may become serious. 

Other trophic disturbances in the oral cavity (affection of the gums, 
ulcers of the tongue and cheeks, loosening or falling out of the teeth) are 
probably due to biting and chewing, and no matter how mild the injury 
may be (and this is not observed on account of the anesthesia present) 
cannot be avoided. Anomalies in the secretion of sweat and in the growth 
of hair, as well as vasomotor disturbances, have also been observed by authors 
as occasional sequelae of disease of the fifth nerve. But these manifestations 
are rarely so marked as to acquire especial importance. Whether some cases 
of so-called hemiatrophia facialis may be referred to disease of the Gasserian 
ganglion or to the branches and roots of the fifth nerve, as some authors 
maintain, is still a mooted question. 

Paralysis of the muscles of mastication, or anesthesia in the entire path 
or in the individual branches of the fifth nerve, readily permits us to diag- 
nosticate an affection of the trigeminal nerve. When we remember, further- 
more, that acute as well as chronic diseases of the pons, of the medulla 
oblongata, of the spinal cord (tabes, syringomyelia) may run their course 
with paralysis of the motor as well as of the sensory portions of the fifth 
nerve, we should search in each individual case for symptoms which point 
to a central affection; then we may note, in a given case, that the affection 
of the trigeminal nerve is merely one phenomenon of the clinical picture. 



GLOSSO-PHARYNGEAL PARALYSIS 681 

When we consider, too, that definitely localized lesions of the cerebrum 
(posterior portion of the internal capsule) produce anesthesia in the areas 
of the organs of special sense, in the extremities, and also in the fifth nerve, 
and that even a purely functional disease (hysteria) may cause disturbances 
of sensation in the course of the trigeminal nerve, we must decide in each 
individual case whether or not we are dealing with a central affection. 

A peripheral lesion of the nerve may be positively diagnosticated when the 
typical symptoms of a trigeminal affection follow a fracture of the skull, a 
gunshot wound, or any other trauma affecting the skull. In addition to 
these etiologic factors there are usually tumors at the base of the brain, 
syphilis or tuberculosis, etc., which implicate the nerve or its ganglia. It 
must also be noted that this diagnostic principle, first formulated by Komberg, 
that the seat of the lesion is more likely to be peripheral the greater the 
disturbance, especially the anesthesia, applies to individual branches. 

When a corresponding part of the face is implicated, there is usually 
a lesion of a main branch; when the entire trigeminal area is involved and 
trophic disturbances are evident, the seat of the pathologic focus may be 
assumed to be the ganglion. The implication of other cranial nerves renders 
it very likely that the seat of the disturbance is at the base of the skull. 

The prognosis must depend upon the nature of the causal affection. If 
this be syphilis, if a trauma has preceded, if there is a tumor, antisyphilitic 
treatment, and, in a few cases, perhaps also operative measures, may bring 
about amelioration and improvement. 

Electricity (galvanization, faradization, franklinization) or irritative in- 
unctions may be used with advantage in anesthetic and paresthetic conditions 
of the face; for the pain which is occasionally very great lukewarm or 
hydropathic poultices may be employed; even anodyne remedies may be indi- 
cated, perhaps subcutaneous injections of morphin, etc. 

The paralyses and enfeebled state of the muscles of deglutition are best 
treated by galvanization or faradization, the inflammatory conditions in the 
eye, in the oral cavity, etc., according to the general principles of ophthalmic 
or surgical therapy. 

3. GLOSSO-PHARYNGEAL PARALYSIS 

As paralyses of the glosso-pharyngeal nerves alone have not yet been 
reported, I might limit myself to a description of those pathological changes 
due to lesions of this nerve which occur clinically. Inasmuch, however, as 
this nerve may be implicated in all of the pathologic processes which affect 
the lowest cerebral nerves, it is necessary to relate what is known of its 
functions, at least briefly, so that these disturbances may be accurately appre- 
ciated. The most important physiologic function of this nerve is this, that 
it supplies the sense of taste to the posterior portion of the tongue, to the 
mucous membrane of the soft palate, and to the palatine arches. The glosso- 
pharyngeal nerve terminates at the base of the tongue in the circumvallate 
papillae, v. Yintschgau and Honigschmied maintain that these degenerate 
after the glosso-pharyngeal is severed, an opinion disputed by B. Baginsky, 
but concurred in recently by a number of other authors. Besides the sensory 
fibers which supply taste for certain areas of the tongue, the glosso-pharyngeal 



682 PARALYSES OF THE PERIPHERAL NERVES 

nerve also contains pure sensory fibers which supply the head of the esophagus, 
the tonsils, the arch of the palate, and the tympanic cavity simultaneously 
with fibers from the trigeminal nerve. Besides the pneumogastric and the 
spinal accessory, this nerve also innervates the contrictors of the esophagus, 
the stylopharyngeal muscle, and perhaps also the glosso-palatine muscle; and 
it contains motor fibers the disturbed function of which may complicate 
the clinical picture. 

Etiology. — A lesion of this nerve may be caused by pathologic processes 
which implicate the lowest cerebral nerves at the point whence they originate 
from the medulla oblongata or the base of the skull. Among these are 
injuries which affect the cerebral nerves immediately below the base of the 
skull, tumors of the base or in the cavity of the occiput, syphilitic changes 
at the base of the skull, or inflammatory affection of the cerebral membranes 
in this region. We have already spoken of acute and chronic bulbar paralysis 
due to changes in the medulla oblongata, and of its most typical symptom, 
difficulty in deglutition. The part which the pneumogastric, the hypoglossal 
and the glossopharyngeal nerves play in these disturbances cannot be esti- 
mated in each individual case; it must suffice to know that the glosso- 
pharyngeal nerve is implicated. When, after diphtheria, an alteration of 
sensation in the mucous membrane of the pharynx, the palate, etc., is noted, 
we must consider an implication of the fibers of the glosso-pharyngeal nerve 
and in some individual, functional, nervous diseases, for example in hysteria, 
disorder of this nerve and its branches is likely. 

Symptoms. — The reports of disturbances of taste in purulent otitis media 
are especially interesting. When these disturbances are found in the anterior 
two-thirds of the tongue on the same side as the diseased ear, we know from 
what has been stated of paralysis of the facial nerve that there is an impli- 
cation of the chorda tympani. Since it is certain that in these diseases 
of the middle ear disturbances of taste eventually occur in the posterior 
third of the tongue and in the palate, they may be attributed to an impli- 
cation of the tympanic plexus and of the branches of the glosso-pharyngeal 
nerve contained therein. Furthermore, during therapeutic manipulations 
within the tympanic cavity, an abnormal secretion of saliva has been ob- 
served; if, as is certain, fibers of the chorda tympani supply the sublingual 
and submaxillary glands, the parotid must obtain its secretory fibers from 
the glosso-pharyngeal nerve, and Urbantschitsch reports an interesting man- 
ipulation of this kind in the middle ear, in which case the saliva was seen 
to exude directly from Steno's duct. 

Little can be said of the prognosis and treatment. If in disease of the ear 
there is a syphilitic affection or a functional neurosis (hysteria), the prognosis 
is relatively favorable, and under appropriate treatment the malady will dis- 
appear. But there is less hope of cure when these conditions are due to dis- 
ease of the medulla oblongata, or to tumors at the base of the brain, etc. Here 
also potassium iodid and electro-therapeutic treatment may be tried; tempo- 
rary improvement may be brought about even though the disease is not cured. 



PARALYSIS OF THE PNEUMOGASTRIC NERVE 683 

4. PARALYSIS OF THE PNEUMOGASTRIC NERVE 

Paralysis of the vagus forms a particularly interesting chapter in the study 
of paralyses of peripheral nerves. In its extensive course the nerve is sub- 
jected to various deleterious influences; as, for instance, at the base of the 
skull, where it may be affected by inflammatory processes of the cerebral mem- 
branes, by neoplasms or similar processes in the bones, or by hemorrhages and 
aneur} r sm. 

Injuries from blows, or incised and gun-shot wounds of the nerve in the 
neck are no longer rare occurrences ; the nerve is also frequently injured during 
surgical operations upon the neck (extirpation of tumors, tying of the carotid 
artery, etc.). A lesion of the trunk of the vagus, particularly its main branch, 
the recurrent laryngeal, has repeatedly been attributed to tuberculous, scrofu- 
lous, or carcinomatous tumors in the neck, in the mediastinum, and in the 
surroundings of the bronchi. A primary neoplasm of the nerve is rare. More 
frequently it succumbs to pressure from induration after chronic pneumonia 
and pleurisy, or, what is particularly interesting, it is compressed in the course 
of pericarditis, as well as by great dilatation of the left auricle (for example, 
in mitral stenosis). 

Degenerative inflammations of this nerve develop in the course or as 
sequels of infectious diseases (enteric fever, diphtheria), as well as in metallic 
poisoning (lead, arsenic) or in chronic alcoholism. That the nerve may be 
damaged by diseases at its point of origin is apparent. Thus we find the 
region of the pneumogastric implicated in various affections of the pons and 
of the medulla oblongata, in acute or chronic lesions in this area, as in tabes, 
in syringomyelia, in multiple sclerosis, in progressive bulbar paralysis and 
from tumors; sometimes also in functional diseases of the nervous system, as 
in hysteria, in which the symptoms point to a special implication of the vagus. 

Before proceeding to the symptomatology of paralysis of the vagus I must 
briefly mention two important points: First, the fact revealed by the careful 
investigations of Navratil, Onodi, Grabower and others that all laryngeal 
nerves originate from the pneumogastric, and that the spinal accessory is not 
a laryngeal nerve. According to these researches, the latter nerve is purely 
a spinal nerve ; there is no cerebral accessory nerve. Secondly, the important 
factor that, on account of the close association of the pneumogastric and the 
spinal accessory in the jugular foramen, these nerves may be simultaneously 
involved by pathologic processes, and that with injuries to the vagus in its 
upper course or disease of the nerve at the base of the skull, the ninth, the 
eleventh, or even the twelfth cerebral nerve is also frequently implicated. It 
is well to bear these possibilities in mind so that in the differentiation of these 
cases, which is not very easy, the physician may more readily comprehend the 
conditions. 

Symptomatology. — Although the symptoms of paralysis of the larynx are 
most frequently due to lesions of the vagus or of its main branch, the recurrent 
laryngeal, no explicit presentation of the symptomatology can be attempted 
here; first, because the subject is too extensive for the scope of this article, 
secondly, because this subject is discussed in a special chapter by a competent 
authority. I shall only emphasize that paralyses of the vocal cords are due 
not only to lesions of the recurrent laryngeal, but are also observed in injuries 



684 PARALYSES OF THE PERIPHERAL NERVES 

to the trunk of the pneumogastric ; if sensibility of the larynx is retained, we 
are justified in concluding that the nerve is injured below the point at which 
it gives off sensory fibers to the superior laryngeal nerve which supplies the 
upper portion of the larynx. 

In injuries to the vagus in its upper course, to the pharyngeal nerves, 
or to the pharyngeal plexus which passes from the plexus gangliformis to 
the pharynx, deglutition is seriously impeded. The bolus remains upon the 
affected side of the pharynx, does not pass into the esophagus but into the 
larynx, and there causes cough and suffocative attacks. 

It is well known that after severing both vagi of young animals they 
perish, and in older animals pneumonic processes follow. Some authors 
believe these to be deglutition pneumonia, others assume that, in consequence 
of injury to the vagus, the lungs are more vulnerable, and a neuro-paralytic 
congestion occurs. This is hardly the place at which to discuss these still 
mooted questions; but I should like merely to state that in deciding upon 
the cause of death after injuries to the pneumogastric, the investigations of 
Eichhorst, Elias and Hoffmann should be considered, these authors having 
proven the heart muscle and the endocardium to be severely damaged after 
such lesions. But the question which most interests us is the following: 
What are the symptoms on the part of the respiratory and circulatory appara- 
tus which follow lesions of the pneumogastric in man? Different observers, 
principally surgeons, have given various answers. That a bilateral injury is 
invariably to be regarded as dangerous to life is self-evident; but, in spite 
of the objections of some surgeons, I believe that unilateral injuries of the 
vagus are never to be regarded as slight, since very serious phenomena often 
appear, and, in any case, the function of the laryngeal muscles is decidedly 
impaired. 

Some surgeons (Deibel, Weidner), in analyzing critically the results of a 
number of unilateral vagotomies, found no marked symptoms on the part 
of the heart; others demonstrated an undoubted increase in cardiac activity. 
On the other hand, prominent clinicians report in disease of the pneumogastric 
an increased pulse rate occurring as the sequel of infectious diseases (for in- 
stance, enteric fever and diphtheria), in alcoholic neuritis, in beri-beri, or 
in cases in which, post mortem, the vagus is found embedded in a neoplasm, 
for instance, a mediastinal tumor, or in which it is compressed by caseous 
bronchial glands. 

It is known that, besides the severing of the pneumogastric, paralysis of 
this nerve may increase cardiac pulsation, and that its mechanical, chemical, 
or electric irritation may decrease the number of heart beats and that the 
heart may even be arrested in diastole. I mention this physiological fact 
because several authors (Czermak, Oerhardt, Quincke, Dubois and others) 
have resorted to compression of the pneumogastric nerve in the neck in the 
treatment of paroxysmal tachycardia. I must also state here that tachycardia 
due to actual disease of the vagus may occur, and, so long as the nerve is 
not destroyed, especially by the irritation of a neoplasm (carcinomata of the 
mediastinal glands), may be preceded by a bradycardia, a slowing of the heart 
beat to 50 or 40 per minute; this condition, however, has been only infre- 
quently observed. 

Moreover, we know that the pneumogastric nerve is concerned in the 



PARALYSIS OF THE PNEUMOGASTRIC NERVE 685 

innervation of the stomach and intestines. Except for the previously men- 
tioned difficulty in swallowing, authors have not reported as due to traumatic 
lesions of the vagus in the neck any marked disturbance in the activity of 
the stomach and intestines; nevertheless, here and there, abnormal nausea, a 
decreased motility of the stomach, and an inordinate bulimia have been 
reported; the gastric crises which are not uncommon in tabes (paroxysmal 
retching, and vomiting accompanied by severe pain in the gastric region), 
as well as the rarer esophageal crisis described by Oppenheim, Courmont, 
Moreira, may probably be attributed to changes in the nerve at its point of 
origin in the medulla oblongata or in the structure of the nerve itself. I must 
also state that irritation of the vagus decreases the amount of urine, and 
would refer to the classic experiments of Claude Bernard that piqiire (injury 
to the floor of the fourth ventricle between the origin of the acousticus and 
the vagus) produces melituria and that a similar condition results from 
irritation of the center of the vagus, also by cutting the vagi; when I state 
that these conditions as the result of disease of the pneumogastric are cer- 
tainly very rare in man, if indeed they occur at all, the symptomatology of 
disease of the vagus, so far as physiologic investigation goes, is complete, and 
a few words only can be added in regard to the diagnosis, prognosis, and 
treatment. 

Diagnosis. — The diagnosis of paralysis of the pneumogastric may some- 
times be easy but is usually quite difficult. If, after injury to, or operation 
upon, the neck, symptoms appear which point to an impairment of the 
function of the larynx, of the respiratory organs, of cardiac activity, or of 
the function of the stomach, we at once recognize a lesion of the vagus. If, 
however, there is no trauma of these organs, we must elicit a careful history 
in order to determine whether there has been a previous infectious disease 
(enteric fever, diphtheria, influenza), must examine the neck for the presence 
of tumors, and the chest for evidence of pathological changes in the thoracic 
organs (the lungs, the pleura, the pericardium, the heart, the arch of the 
aorta, etc.). It must also be borne in mind that the vagus is implicated in 
the course of many acute and chronic diseases of the prolonged cord (hemor- 
rhage, softening, or neoplasm in this region, also tabes, syringomyelia, mul- 
tiple sclerosis, and progressive bulbar paralysis), and in every case which 
indicates a vagus lesion we must seek for proof of the existence of one of 
these affections. Only in the utter absence of any sign pointing to actual 
lesion of a nerve, whether at its point of origin or in its peripheral course, 
and on considering the variability of the symptoms and the temperament 
of the patient, are we warranted in diagnosticating a neurosis (neurasthenia, 
hypochondriasis, most often hysteria). 

Although the preceding has shown that disease of the vagus may produce 
changes in phonation, in respiration, in the circulation and in digestion, we 
know that these symptoms do not necessarily appear in every case. Fre- 
quently the muscles of the larynx alone are paralyzed, and this is usually 
due to a lesion of the recurrent laryngeal nerves. If paralysis of the laryngeal 
muscles is accompanied by disturbance of heart action and by a change in 
the rhythm of the respiration, there is every reason to apprehend an affection 
of the pneumogastric nerve. Finally, if we bear in mind that disturbance 
of the normal function of the vagus may be produced reflexly by disease 



686 PARALYSES OF THE PERIPHERAL NERVES 

of distant organs (the stomach, the intestines, the liver, the kidneys, the 
uterus) we shall have considered in the given case every factor that is of 
importance in the diagnosis of an affection of the pneumogastric nerve. 

Prognosis. — The prognosis is always doubtful. If the affection is due 
to disease of the central organ (medulla oblongata) the outcome depends 
chiefly on the importance of the underlying affection. Disturbances of heart 
action and of the respiration after infectious diseases, which may be attrib- 
uted to implication of the vagus, are always to be regarded as serious com- 
plications, and just as serious are those disturbances of the pneumogastric 
which are caused by disease of the intrathoracic organs, while, as we have 
seen, unilateral destruction of the nerve (due either to the presence of a 
tumor or to extirpation of neoplasms by operation) offers a much more 
favorable prospect of recovery, and even of life. 

The prognosis in paralysis of the muscles of the larynx naturally depends 
upon the underlying cause; paralysis of the openers of the glottis (the pos- 
terior crico-arytenoid muscles) are the most threatening to life, particularly 
if the affection is bilateral; here imminent danger can only be averted by 
tracheotomy speedily performed. 

Treatment. — Leaving out of consideration the immediate and great risk 
of tracheotomy in a bilateral paralysis of the posterior crico-arytenoid muscles, 
paralysis of the other muscles of the larynx is best treated by electricity with 
the galvanic as well as with the faradic current. On account of the difficulty 
of intralaryngeal electrization, and because the possibility of stimulating the 
nerves and muscles of the larynx by the external application of electrodes 
to the neck has been demonstrated, the latter method is preferable. Massage 
of the larynx and similar methodical exercises utilized as auxiliary measures 
are especially adapted to the treatment of hysterical paralysis of the vocal 
cords. 

If we detect syphilis, scrofula or tuberculosis in a given case of disease 
of the vagus, appropriate treatment for these underlying conditions should 
first be considered. If we find acute or chronic disease of the medulla oblon- 
gata, this condition must be treated. Disturbances of the respiration and 
circulation, especially after infectious diseases which produce disease of the 
pneumogastric, must be relieved by stimulants and a suitable diet to strengthen 
and tone up the patient. If there are tumors of the neck which interfere 
with the normal function of the nerve, operation is indicated; for we know 
from previous experience that a unilateral lesion of the vagus may be borne 
by the patient without great difficulty, but the removal of malignant tumors 
is naturally necessary for entirely different reasons. 

Neuroses of the pneumogastric such as occur in nervous, hysterical or 
neurasthenic persons are treated according to the principles on which is 
based the treatment of the underlying affection. In conclusion, if we have 
reason to believe that the symptoms indicating an affection of the vagus are 
attributable to disease of distant organs, the therapy must be directed to the 
latter; frequently, after the administration of an emetic or laxative, to 
mention only one of the possibilities, apparently dangerous symptoms may 
rapidly disappear. 



PARALYSIS OF THE SPINAL ACCESSORY NERVE 687 

5. PARALYSIS OF THE SPINAL ACCESSORY NERVE . 
(THE NERVE OF WILLIS) 

Before entering upon the symptoms of an uncommon affection, paralysis 
of the eleventh cerebral nerve, the spinal accessory, a few remarks are in place 
concerning some recent anatomical and embryologic investigations of this 
subject. While an internal and external branch of the nerve were formerly 
mentioned, and it was believed that the internal gave off branches for the 
heart and the larynx, we have come to the conclusion that the spinal accessory 
has nothing to do with the innervation of these organs ; that, on the contrary, 
the lowest roots of the vagus control these structures, and the spinal accessory 
supplies motor fibers to only two muscles, -the sterno-cleido-mastoid and the 
trapezius. It must be added that the trapezius is also innervated by the upper 
cervical nerves and, according to Sternberg's investigations in monkeys, the 
junction of the second cervical nerve with the spinal accessory resembles that 
of a sensory nerve, except that the spinal accessory joins the motor fibers 
just before it enters the muscle. 

When we consider how close the origin between the fibers of the spinal 
accessory and those of the vagus, how frequently both nerves are damaged 
by the same cause, also the close proximity of the hypoglossal to the two 
previously mentioned nerves, we may readily understand that paralysis of 
the spinal accessory frequently accompanies that of the pneumogastric, per- 
haps also of the hypoglossal nerve. 

Etiology and Symptoms. — In the etiology of paralysis of the spinal acces- 
sory, we must first enumerate wounds of different varieties at the back of the 
head and neck; secondly, operations upon the back of the neck, also in the 
vicinity of the sterno-cleido-mastoid and trapezius; thirdly, pathological 
changes in the interior of the upper cervical vertebras and injuries of the 
same which may lead to disease of this nerve. 

If the upper portion of the cervical cord is diseased, as is the case in 
tabes, in syringomyelia, in syphilitic affections of this region, or in tumor, 
symptoms appear which distinctly indicate the implication of the spinal 
accessory. We know, too, that the musculature of the neck and the back of 
the neck is frequently implicated in progressive and juvenile muscular atrophy, 
and this is emphasized as a guide to exact diagnosis. 

We shall first consider unilateral paralysis of the external branch of the 
spinal accessory, which produces paralysis of the sterno-cleido-mastoid and 
the trapezius. Unilateral paralysis of the sterno-cleido-mastoid causes the 
patient to hold his head obliquely toward the affected side; at the same time 
the activity of the normal muscle of the other side slightly raises the chin. 
By the action of the deep muscles at the back of the neck the head may 
readily be rotated toward the sound side. If the paralysis persists for a long 
time, the corresponding normal muscle frequently contracts, and atrophy of 
the paralyzed muscle may ensue, so that instead of the normal contour of the 
muscle it appears as a thin band; the diseased side of the neck is smooth 
or even depressed. Bilateral paralysis makes it very difficult to rotate the 
head while it is elevated; the muscles of the neck on both sides appear to be 
flattened. 

The trapezius muscle has actually three different parts and functions : The 



688 PARALYSES OF THE PERIPHERAL NERVES 

clavicular portion which moves with respiration and at the same time raises 
the shoulder ; the middle part, the true elevator of the shoulder-blade ; and the 
lower part which causes the shoulder-blades to approximate; this is usually 
implicated simultaneously with the middle portion. If the entire muscle is 
paralyzed, the shoulder-blade is depressed and appears as if it had slipped 
down. The internal border of the scapula is oblique, slanting from below 
inward and from above outward. Upon attempting to raise the arm the 
internal border of the scapula adheres to the thorax and does not rise. In 
spite of the fact that the shoulder on the diseased side may be raised through 
the action of the levator scapulae, this elevation is not as complete as upon the 
normal side. According to the recent investigations of Mollier, elevation of 
the arm anteriorly to the horizontal position is prevented by paralysis of the 
trapezius; the influence of the muscle may readily be dispensed with; but 
on weighting the arm thus raised a very light weight will more readily pro- 
duce fatigue and pain than in the normal arm. While a purely lateral raising 
of the extended arm to the horizontal position is impossible, the full uplift of 
the arm is almost perfect; the few degrees lacking upon the diseased side 
may be concealed by a greater curvature of the vertebral column. It is very 
difficult to draw the scapula upon the diseased side toward the rnedian line; 
the contours of the rhomboidei and of the levator scapula? become very 
distinct, and put the shoulder-blade into an oblique position (action of the 
rhomboid muscles). 

The rocking position (position ou mouvement de bascule) of the shoulder- 
blade, described by Duchenne, in which the acromial portion of the scapula 
is depressed, the lower angle deflected toward the median line, and the internal 
border oblique from above and outward to down and inward is observed only 
when the middle portion attached to the acromion and the spine of the scapula 
is implicated in the paralysis. 

Although the finding is not the same in all cases, it appears from the 
investigations of Eemak that the rocking position of the scapula does not 
appear in a pure, uncomplicated paralysis of the spinal accessory. It occurs 
only when, for example, after an operation, the deeper fibers which are fur- 
nished by the cervical nerves and which enter the trapezius at the anterior 
border of the muscle, are injured. 

If there is bilateral paralysis of the external branch, the back is abnor- 
mally arched, the thorax is deepened; the clavicles are very prominent, the 
supraclavicular fossae form deep cavities. Eotary movements of the head 
are especially difficult when the chin is raised; the normal outlines of the 
sterno-cleido-mastoid muscles in the neck have disappeared. 

To the symptoms of paralysis of the spinal accessory which have been 
described, and which affect chiefly the muscles of the neck and the nape of 
the neck, in numerous cases symptoms are added which were formerly attrib- 
uted to damage of the so-called internal branch, but which to-day we know 
to be due to a lesion of the pneumogastric ; namely, disturbances in the func- 
tion of the velum palati, the pharynx, the muscles of the larynx, and anom- 
alies in the beat of the heart. 

In unilateral paralysis of the velum of the palate the diseased portion, 
which appears to be broader than the normal half, is lower, in phonation 
only the sound half contracts while the paralyzed half remains flaccid, and 



PARALYSIS OF THE SPINAL ACCESSORY NERVE 689 

on this side there is no power to close the pharyngo-nasal isthmus. Thus 
the speech becomes nasal, and fluids regurgitate from the nostril on the 
diseased side. Unilateral paralysis of the muscles of the pharynx forces the 
patient to incline the head toward the paralyzed side. The same difficulty 
appears in deglutition, for particles of food which pass into the larynx 
readily produce cough. When the laryngeal branches are implicated the 
speech is hoarse and low, and hard coughing is impossible. 

Laryngoscopic examination reveals (according to whether there is uni- 
lateral or bilateral paralysis of the vocal cord, and according to the law 
emphasized by Semon which may be observed in many cases) that the pos- 
terior crico-arytenoids are the muscles which first show the signs of paresis. 
In some cases an alteration in the beat of the heart has been observed. Pulse 
frequency (even in rest) is permanently increased. 

The symptoms vary according to the extent of the destructive process and 
its seat in the individual case; there may be unilateral or bilateral paralyses 
of the sterno-cleido-mastoid and the trapezius, symptoms which indicate an 
implication of the so-called internal branch, may be absent or unilateral or 
bilateral, and may vary according to the involvement of the fibers for the 
velum of the palate, larynx, etc. 

Little can be said in regard to disturbances of sensation and nutrition 
following paralysis of the spinal accessory. These are very insignificant. 
If the implication is severe, the paralyzed muscles may atrophy decidedly; 
thinness of the vocal cord has also been observed, and electrically the different 
varieties may show altered contractility. 

After what has been stated it can scarcely be difficult to recognize the 
influence of paralysis of one or both of the spinal accessory nerves upon the 
normal function of the sterno-cleido-mastoid and trapezius. Perplexity can 
here be occasioned only by the fact that in neuritic processes, in trauma, and 
in progressive muscular paralysis, the movements of the shoulder-blade and 
other important muscles are impeded, and thus the true picture of paralysis 
of the spinal accessory may be masked so far as external muscles are concerned. 

Considering the possible implication of the so-called internal branch which 
supplies the muscles of the palate, the lar}rax and the pharynx, these struc- 
tures must be investigated in each individual case, and we should note the 
condition of the velum of the palate, the muscles of the larynx, etc., as well 
as test the pulse. Only when the peripheral lesion (for example, after oper- 
ations upon the neck) is far distant from the intravertebral origin of the 
nerve, in the middle or posterior third of the neck, may these tests be 
dispensed with. 

If, in a given case, we are assured of the implication of the cervical 
sympathetic or the hypoglossal nerve, we must seek for a pathologic cause 
in the interior of the cervical vertebral canal. If this cannot be determined, 
a high lesion of this nerve in the neck may be excluded. An affection of the 
base of the brain is suggested if, besides the spinal accessory, the sympathetic 
and the pneumogastric, paralysis affects the nerves situated more anteriorly, 
such as the auditory, the facial, or the abducens. The history must always 
be carefully elicited, and we must bear in mind that, in the given case, we 
may be dealing with progressive muscular atrophy or with an affection 
of the central nervous system (tabes, syringomyelia). If syphilis can be 
45 



690 PARALYSES OF THE PERIPHERAL NERVES 

excluded, we must consider whether there is not a tumor developing slowly 
in the posterior part of the skull or in the cervical vertebral canal. 

Various observers have reported unilateral or bilateral congenital absence 
of the trapezii and of the sterno-cleido-mastoid muscles. If the history be 
carefully taken this fact may prevent errors in diagnosis. 

In disease of the spinal cord, when there are tumors in the vertebral 
canal, and in progressive muscular atrophy the prognosis is naturally very 
unfavorable; it is brighter when the lesion is of syphilitic character. Im- 
provement or recovery may follow even inflammatory or traumatic affections 
of the nerve, although the time when this occurs cannot be stated with 
certainty. 

Treatment must be directed to the underlying affection. If a causal 
therapy, as in syphilis or in disease of the vetrebras, is possible, if neoplasms 
can be successfully removed, of course therapeutic measures in this direction 
are indicated. If there are paralyses due to trauma or neuritic processes, 
electro-therapeutic treatment should be instituted, and this may be assisted 
by orthopedic measures. 

6. PARALYSIS OF THE HYPOGLOSSAL NERVE; PARALYSIS 
OF THE TONGUE; GLOSSOPLEGIA 

Paralysis of the hypoglossal nerve, in comparison with that of the facial, 
especially the purely peripheral, is rare. The former is usually caused by 
gunshot wounds of the nerve, by incised wounds with a knife (attempts at 
suicide), by glandular tumors and operative wounds of the neck. Paralysis 
of the tongue is found in a number of pathologic processes at the base of the 
skull in which the hypoglossal nerve, usually in combination with neighboring 
nerves (the vagus and spinal accessory) is affected. Here we are prone to 
find tuberculous, carious, carcinomatous, or syphilitic diseases at the base 
of the skull, neoplasms in this region or in the occipital foramen, and also 
in the upper portion of the cervical canal. Not a few cases have been 
reported of paralysis of the tongue which developed in consequence of disease 
of the upper cervical vertebras (caries, necrosis, syphilis). In another series 
of cases the authors were apparently unable to find any etiologic factor for 
the unilateral lingual paralysis which they observed. Recent reports of these 
interesting cases make it appear likely that neuritic processes generated by 
inflammatory changes in the mucous membrane of the pharynx are the 
cause of these remarkable paralyses which have perhaps been brought about 
by glandular disease which implicated the hypoglossal nerve. 

The frequent coincidence of such unilateral atrophy of the tongue with 
unilateral atrophy of the face, the latter being the consequence of infectious 
disease, particularly of tonsillitis, is significant. Just as in facial paralysis, 
congenital (usually unilateral) atrophies take place in the tongue but are 
more rare; and, as in congenital facial paralysis, there is usually a defective 
formation of other cerebral nerves and anomalies of structure in other parts 
of the body. 

That unilateral atrophy of the tongue is also observed, but more rarely, 
in scleroderma and in acromegalia is mentioned here for the sake of com- 
pleteness. In comparison with the rare, purely peripheral paralyses of the 



PARALYSIS OF THE HYPOGLOSSAL NERVE 691 

hypoglossal nerve, implication of this nerve from various pathologic changes 
affecting the brain is relatively frequent. We find this in central hemi- 
plegias due to supranuclear lesions, in progressive paralysis, and most often 
in all acute, subacute or chronic processes which affect the normal structure 
of the medulla oblongata. Among these processes I must mention, above all, 
bulbar paralysis, progressive muscular atrophy, hemorrhages, softening of the 
medulla oblongata, and syphilitic processes. Some spinal cord affections are 
occasionally combined with unilateral atrophy of the tongue, and also, it is 
reported, with tabes and syringomyelia. Isolated cases are found in litera- 
ture which prove that multiple sclerosis may be associated with atrophy of 
the tongue. 

Symptoms. — In unilateral paralysis of the tongue the diseased half, com- 
pared with the healthy, firm side, is much softer, more flaccid, collapsed, 
fissured, and furrowed; frequently there are fibrillary contractions. 

In the majority of cases of unilateral paralysis, the tongue on being 
protruded deviates toward the paralyzed side, obeying an impulse from the 
non-paralyzed genioglossus muscle. The concavity of the raphe, too, is di- 
rected toward the paralyzed side. If the tongue lies at rest on the floor of 
the mouth, its tip may deviate toward the normal side, but several cases 
have been described of unilateral partial lesions of the hypoglossus in which 
the protruded tongue deviated toward the paralyzed side. Apparently there 
are variations in the symptoms which depend, perhaps, upon the more or 
less intense implication of this or that bundle of fibers in the muscles of the 
tongue. 

A fact especially important is the remarkably slight subjective difficulty 
experienced in unilateral paralysis of the tongue. Although in some cases 
motion of the tongue may be difficult, and effort is necessary to enunciate 
certain sounds, the majority of investigators express astonishment over the 
undisturbed power of speech, of mastication, and of deglutition. In some 
cases of unilateral atrophy of the tongue the patients are aware of this only 
because their attention is called to it by the physician. As to disturbances 
of sensation in unilateral paralysis of the tongue, most authors unanimously 
agree that these are absent or insignificant. This is true not only of sensa- 
tions of contact, of pain, and of temperature, but also of the sense of taste 
which, in the main, is found to be unimpaired. 

The less essential observation of some authors that the tongue is more 
thickly coated on the paralyzed side than on the normal side may be men- 
tioned; this probably is explained by its decreased power of motion on the 
paralyzed side. A curious fact, which so far as I know has been but once 
reported, is that in paralysis of the hypoglossal nerve severe trophic disturb- 
ances and gangrene of the paralyzed side may be observed as well as the 
previously mentioned atrophy of the paralyzed muscles. 

It is well known that the descending branch of the hypoglossus forms a 
loop with branches coming from the root of the second and third cervical, 
and thus innervates some of the muscles of the hyoid bone, such as the sterno- 
hyoid, sternothyroid, the lower belly of the omohyoid, and perhaps of the 
thyrohyoid. If these muscles are implicated in the paralysis, as has been 
observed in a few cases, a flattening of the thyroid cartilage is noted upon 
the affected side; this is most prominent in the neck upon deglutition, the 



692 PARALYSES OF THE PERIPHERAL NERVES 

larynx deviates laterally, and the electric contractility of the paralyzed mus- 
cles reveals characteristic changes. 

According to the severity of the lesion, this electric contractility of the 
muscles of the hyoid bone, as well as of the tongue itself, may denote a mild, 
medium, or severe degree of paralysis, a fact with which we are familiar, 
and which need not be minutely discussed after all that has been stated of 
the phenomena of contractility in peripheral paralyses. 

Various observers have demonstrated the galvanic movements on deglu- 
tition which were especially studied by Erb; these are produced reflexly by 
stroking the external half of the larynx with the cathode even when there 
is complete unilateral paralysis of the tongue, and notwithstanding atrophy. 

The diagnosis of a unilateral atrophy of the tongue in itself presents no 
difficulty. If there is a trauma of the neck, the paralysis of the tongue is 
easily understood; if, besides the hypoglossus, other nerves situated in the 
posterior fossae of the skull are implicated in the paralysis and atrophy, 
syphilis is suggested, disease of the base of the skull and the upper cervical 
vertebras are thought of, and, as has been previously stated, the existence 
of tabes or syringomyelia must be considered. If there is unilateral atrophy 
of the tongue as well as hemiatrophia facialis, the coexistence of these con- 
ditions is readily apparent. When I state that in cases of encephalitis pontis 
a unilateral paralysis with facial paralysis upon the same side developing 
during childhood has very rarely been observed, that softening of the medulla 
oblongata may occur due to disease of the arteries which is also rare and 
which appears clinically as an alternating hemiplegia (unilateral paralysis 
of the tongue and atrophy of the tongue with paralysis of the opposite half of 
the body), I have mentioned all that, in a given case, can aid us in the 
diagnosis. I may add that if the muscles of the hyoid bone are found to be 
implicated in the paralysis, we must look for the seat of the lesion in the 
trunk of the hypoglossal nerve above the point of its anastomosis with the 
upper cervical nerves. According to Dinkier, the symptoms of a trunk or 
root paralysis differ only in degree, and the difference between total and 
partial paralysis is only quantitative. A paralysis of the root is likely when 
there is partial atrophy with DeR, and when other symptoms denoting 
disease of the root are simultaneously present. 

Before concluding our discussion, bilateral paralysis of the tongue must 
be referred to. Peripheral lesions of the nerves of the tongue causing paral- 
ysis have, so far as I know, never been described. But we know that these 
conditions belong to the symptomatology of acute, subacute, and chronic 
bulbar paralysis and pseudobulbar paralysis. In these conditions, contrasted 
with unilateral paralysis of the tongue, speech, deglutition and mastication 
are impeded to a high degree. The tongue itself is wrinkled and flaccid, it 
presents fibrillary contractions, its movements are imperfect, the bolus of 
insufficiently salivated food can scarcely be rolled to the pharynx and saliva 
collects in the mouth or persistently dribbles from the partially closed mouth. 
In spite of this extreme disturbance of motility, sensation and taste remain 
unimpaired or suffer only because the food which is imperfectly masticated 
no longer comes sufficiently into contact with the surface of the tongue. 
Electric examination reveals a diminution of contractility or partial DeR. 

In a given case it is not difficult to diagnosticate bilateral paralysis of 



MULTIPLE CEREBRAL NERVE PARALYSIS 693 

the tongue, and it is usually attributable to an affection of the medulla 
oblongata. We must bear in mind that peripheral bilateral paralysis of the 
tongue is as yet unknown. The implication of the facial nerve, especially 
of its labial branches, and atrophic conditions in the muscles of the body as 
well as bilateral paralysis and atrophy of the tongue, must be called to mind; 
a review of the history will show that bilateral paralysis of the tongue has 
perhaps only lately been added to progressive muscular atrophy which had 
existed for a considerable time, after which we may readily diagnosticate 
bilateral paralysis of the tongue and recognize the pathologico-anatomical 
disturbance which causes it. We would digress too far to discuss at this 
point the differential diagnosis of paralysis of the tongue due to unilateral 
or bilateral lesions of the cerebrum (pseudo-bulbar paralysis). 

The prognosis in peripheral paralysis of the hypoglossal nerve is much 
more favorable than in those forms produced by diseases of the brain, of the 
medulla oblongata, and of the spinal cord. There is more hope of recovery 
in paralyses due to syphilis and disease of the cervical vertebrae than in those 
due to central lesions. 

The treatment of paralyses of the tongue which follow an affection of 
the cerebrum, of the medulla oblongata, or of the spinal cord must be directed 
to the underlying affection. Appropriate surgical or orthopedic treatment 
may be instituted in paralysis of the tongue caused by disease of the vertebras, 
and antisyphilitic treatment when the affection is attributable to syphilis. 
Electricity is always beneficial in central paralysis as well as in that due to 
purely peripheral conditions, either because the muscles of the tongue or, 
indirectly, the nerves are stimulated by faradism or galvanism, and because 
the galvanic current is applied to the brain, the medulla or spinal cord. 

7. MULTIPLE CEREBRAL NERVE PARALYSIS 

After the consideration of paralysis due to lesions of individual cerebral 
nerves, we must describe those not infrequent paralyses in which the lesion 
cannot be located definitely in this or that nerve but in which a number of 
cerebral nerves are implicated. I refer to the so-called paralysis of multiple 
cerebral nerves. In the preceding description I have frequently referred to 
the fact that, besides one special cerebral nerve, occasionally this or that 
neighboring nerve or even several may be implicated in the process. 

In the etiology of such cases we find a history of injuries to the head, 
the neck, and the back of the neck, and of fractures of the skull, especially 
at the base. In addition, tuberculous and syphilitic changes at the base of 
the skull and tumors in this region play an important role. Even if the 
cerebral membranes at the base alone are implicated, or, as in the case of 
tumors (sarcomata, carcinomata) if the bones also are injured, several cranial 
nerves, as a rule, are affected, although this depends upon the seat of the 
lesion. Congenital conditions (insufficient development?) very rarely affect 
several of the cerebral nerves ; nevertheless there are a few reports of this 
in literature ( Schapringer, Schmidt). 

It may here be in place to call attention again to the symptomatology 
of paralysis of the individual cerebral nerves which has been heretofore men- 
tioned. 



694 PARALYSES OF THE PERIPHERAL NERVES 

In the majority of cases multiple cerebral nerve paralysis is unilateral, 
and implicates three or four, often even more of the nerves ; in syphilitic affec- 
tions and in tumor the pathologic conditions may implicate either one side 
or both. 

While disease of the olfactory nerve is rarely mentioned, inflammation 
of the optic nerve and a consequent impairment of the sight plays a far 
more important role. Ophthalmoscopically we usually find neuritis of this 
nerve, and only when there is direct pressure upon the chiasm or the optic 
nerves such as is produced by tumors of the pituitary body, do we observe 
white atrophy of the papillae without preceding inflammation. While, not- 
withstanding existing inflammation of the nerve, the sense of sight may be 
more or less retained, direct pressure of a tumor in the region of the hypophy- 
sis producing atrophy causes a well-defined bi-temporal hemianopsia, and the 
subsequent implication of the optic nerves may occasionally aid us in the 
diagnosis. 

It must be borne in mind that inflammation of the optic nerve as well as 
the other symptoms of the patient (headache, etc.) are the general expression 
of a limitation of space within the skull, and they have a local diagnostic 
value only when the inflammation is unilateral and corresponds to the 
symptoms upon the affected side produced by injury to the other cerebral 
nerves. 

If a neoplasm ruptures the anterior cranial fossa, exophthalmos and 
complete paralysis of all the muscles of the eye may follow. If the lesion is 
in the middle of the cranial fossa it causes a conspicuous paralysis of the 
muscles of the eye (especially of the oculomotor, or the trochlear nerve, more 
rarely of the abducens). Moreover, symptoms attributable to the damaged 
trigeminal nerve appear (rarely paralysis of the muscles of mastication), 
particularly as paresthesias, anesthesias, pain in the facial branches of this 
nerve, to which naturally a neuroparalytic inflammation of the corresponding 
eye may be added. If the pathologic processes at the base involving the facial 
and the acoustic are very extensive, the last four cerebral nerves (glosso- 
pharyngeal, vagus, spinal accessory, and hypoglossal) may sometimes be im- 
plicated. If, however, these nerves are affected while the nerves of the ocular 
muscles and the fifth nerve remain undamaged, we may regard it as certain 
that the lesion is situated in the posterior cranial fossa. 

We have now enumerated the fundamental laws upon which to base a 
diagnosis, and it is unnecessary to recapitulate the individual points. 

As to the nature of the affection which leads to multiple cerebral nerve 
paralysis, it may be stated that young persons who are predisposed to tuber- 
culosis, or who suffer from tuberculous affections of the bones or joints, are 
prone to tuberculous disease of the cerebral membranes or of the bones at 
the base of the skull. 

The probability of syphilitic affection is more frequently assumed in later 
life than in the young; the history combined with other characteristic symp- 
toms of syphilis will justify a suspicion of this disease at the basis cranii. 
The question, often difficult to decide, whether in a given case we are dealing 
with syphilis or a neoplasm, is frequently determined by the results of anti- 
syphilitic treatment. Nevertheless, this cannot be depended upon with cer- 
tainty, for cases have been reported in which, in spite of antisyphilitic treat- 



PARALYSIS OF THE DIAPHRAGM 695 

ment, the subsequent autopsy revealed the presence of a syphilitic process at 
the base of the brain. 

If there has been a trauma of the skull, particularly a fracture at the 
base, or an operative wound in the upper part of the neck, there can hardly 
be a doubt as to the diagnosis. 

In the diagnosis of multiple cerebral nerve paralysis, preexisting disease 
of the spinal cord must be considered (tabes, progressive paralysis, syringo- 
myelia), in which case numerous cerebral nerves may be paralyzed simultane- 
ously; when I state that in so-called nuclear paralysis of the muscles of the 
eye other cerebral nerves, chiefly the facial and the trigeminal, may be impli- 
cated, I have enumerated all the points which are necessary in a given case 
for the diagnosis of multiple cerebral nerve paralysis. 

In conclusion I must briefly remark that a paralysis which involves sev- 
eral cerebral nerves situated in the posterior cranial fossa, and which develops 
gradually, may occasionally be confounded with Duchenne's progressive bul- 
bar paralysis. Another fact is that, especially in pathologic processes in the 
posterior cranial fossa, besides paralysis of the nerves of this region, polydipsia 
and polyuria may appear, and may confirm the diagnosis of a pathologic proc- 
ess in this area. 

In the prognosis and treatment of multiple cerebral nerve paralysis, it 
may be said that the cases due to syphilis or external injuries have a much 
better chance of recovery than those due to tuberculosis or to tumors at the 
base of the skull. Xotwithstanding the oft quoted failure of antisyphilitic 
treatment, it is wise to institute this, for even in some cases of undoubted 
neoplasms a period of improvement, at least, was thus brought about. Only 
an unfavorable prognosis can be given when multiple cerebral nerve paralysis 
occurs in the course of chronic diseases of the spinal cord, and here the treat- 
ment must be based upon the underlying affection. In cases in which the 
implication of the trigeminal nerve produces severe neuralgic pains, narcotics 
should be administered to the patients. 

8. PARALYSIS OF THE DIAPHRAGM 

Of the paralyses affecting the region of the cervical plexus the practitioner 
is interested only in paralysis of the phrenic nerve, paralysis of the diaphragm, 
which is seldom observed. The nerve of the diaphragm extending from the 
region of the third and fourth cervical nerves may be injured in its course 
through the neck and within the thorax by trauma or neoplasms (aneurysms) 
but this is more rarely the case than its degeneration after some infectious 
disease, such as diphtheria and scarlatina, or by polyneuritis, occasionally 
from exposure to cold, more frequently from various poisons (alcohol, lead, 
carbonic oxid, opium, etc.). 

The branches distributed to the diaphragm may be affected by inflamma- 
tion of the pleural or peritoneal lining of the same, and in injuries of the 
upper cervical vertebrae and of the upper cervical cord this chief nerve of 
respiration may be damaged by various pathologic processes as well as in 
chronic diseases of the cervical cord (bulbar processes, progressive spinal 
muscular atrophy) and in acute inflammatory affections of this region. In 
rare cases, the nerves has been found implicated in tabes ; functional (hysteri- 



696 PARALYSES OF THE PERIPHERAL NERVES 

cal) paralyses also occur. In very rare cases there is congenital absence or 
partial absence of the diaphragm. 

The symptoms of paralysis of the phrenic nerve depend upon the absence 
of contractions of the diaphragm in inspiration. The thorax is dilated by 
the contraction of the intercostal muscles and the scaleni, but while it ex- 
pands the hypochondriac and epigastric regions retract. The respiration, 
hardly increased during rest, becomes very rapid on exertion and dyspnea 
and a sense of suffocation follow. The patient avoids deep respiration because 
it often causes pain and a feeling as if the abdominal viscera were rising 
into the thorax. Since the abdominal cavity is enlarged by the high position 
of the diaphragm and by paralysis of the muscle, the strength of the abdom- 
inal press (prelum abdominale) is decidedly decreased, and all expiratory acts 
(cough, evacuation of the intestines, etc.) are accomplished with difficulty. 

Litten's Diaphragm Phenomenon. — I must here call attention to a 
phenomenon discovered by Litten which may be demonstrated in every normal 
person; it consists of an undulatory movement which, beginning bilaterally 
at the height of the sixth intercostal space and extending as a straight line 
or slight furrow with deep inspiration descends several intercostal spaces, 
then rises to the same height during expiration. This phenomenon is best 
seen in a person who assumes a horizontal position with his back toward the 
light; the sign is most clear upon the right side of the thorax anteriorly, 
less well marked upon the left side and upon the back. In children and 
women the phenomenon is less distinct than in men. Litten's phenomenon 
has already been employed diagnostically ; for example, in the hemiplegia of 
adults as well as in that of children, the phenomenon is less well developed 
upon the paralyzed than upon the normal side. 

The electric contractility of the normal phrenic nerve is the same as that 
of all other motor nerves. In asphyxia (chloroform or charcoal) the electric 
contractility is greatly diminished; the same is true of all paralyses of this 
nerve due to neuritic processes, particularly in cases of alcoholic neuritis. 

If we bear in mind the points just stated in regard to paralysis of the 
diaphragm, symptoms arising after injury or other disease of the upper 
cervical vertebrae may correctly be referred to a lesion of the phrenic nerve. 
Similar conditions arise in neuritic affections of the nerve, in the case of 
alcoholics, or in persons who have just recovered from severe infectious diseases. 
In chronic affections of the cervical cord and the medulla oblongata, especially 
in their terminal stages, the action of the diaphragm may be paralyzed, and 
it has already been mentioned that this may occasionally occur in tabes and in 
poisoning from lead and gas. We should always consider a more or less 
severe implication of the diaphragm when we are dealing with a hysterical 
person, in lead poisoning, or if respiratory difficulty appears in the course of 
peritonitis or pleurisy. 

In the rare unilateral paralyses of the phrenic nerve in consequence of 
tumors in or injury to the neck, Litten's phenomenon, the decreased or almost 
inaudible inspiratory murmurs, the anxiety and dyspnea of the patient, his 
low speech, and the absence of laryngeal stridor may assist in the diagnosis. 

The prognosis of paralysis of the diaphragm depends upon the underlying 
condition, therefore in injuries to the vertebrae of the neck and in acute or 
chronic diseases of the cervical cord and the medulla it will always be dubious. 



PARALYSIS OF THE DIAPHRAGM 697 

All diseases of the phrenic nerve must be regarded as serious. Although, 
while the patient remains quiet, the difficulty in respiration is not decided, 
this may at once become dangerous if the slightest affection of the bronchi 
or lungs is superadded. Aside from the form occurring in diphtheria, alcoholic 
paralyses of the phrenic nerve are the most serious; at all events, they neces- 
sitate more care and are less favorable than those which occasionally appear 
in persons suffering from lead poisoning or syphilis. 

We should not be too sanguine in the prognosis of paralysis occurring 
in the course of hysteria, for we must bear in mind the not infrequent experi- 
ence that death may occur suddenly. 

I must admit that in a recent article two American authors, Schroder 
and Green, have attempted to prove that the cases described in literature 
of injury to the phrenic nerve with fatal consequences depended upon errone- 
ous observations, that severe injury of important organs was usually found, 
and that the unfavorable outcome could not be ascribed solely to a lesion of 
the phrenic nerve. Their own investigations and the study of the literature 
of the subject led these authors to the conclusion that a unilateral injury 
of the phrenic nerve is not always followed by a fatal termination or even 
serious consequences. 

Treatment. — Paralyses of the diaphragm due to wounds of the neck or 
to trauma of the cervical vertebrae naturally require careful surgical treat- 
ment. Paralysis due to syphilis, to lead poisoning, etc., must be combated by 
treating the underlying condition witli the measures now to be described. 
Paralysis of the phrenic nerve on a psychic basis (hysteria) should be treated 
by suitable physical and psychical therapy. If we are dealing with asphyxia, 
as in the new-born, in poisoning with toxic gases, in chloroform narcosis, or 
that which occurs in severe hysterical attacks, the use of electricity, especially 
the faradic irritation of the phrenic, will sometimes be very beneficial even 
in apparently desperate cases. 

The electrodes, which should not be too small, are placed on both sides 
of the neck at the external border of the sterno-cleido-mastoid, some pressure 
being exerted from without inward. (This method of treatment has been 
evolved by v. Ziemssen.) 

The current should be moderately strong; we need not fear to stimulate 
other inspiratory muscles; on the contrary this is rather desirable. The 
shoulders, the upper arm, and the head are to be fixed; after a stimulation 
of one or two seconds in duration, expiration should be practised by making 
pressure upon the abdominal wall, both laterally and from below upward. 
From time to time we suspend the electricity, and note whether respiration is 
taking place. It must be remembered that a long time may often pass before 
respiration occurs spontaneously. There are reports in literature which make 
it appear that, often after hours during which there was apparently no reac- 
tion to electric irritation, contractility returned and the patient was saved 
from imminent death. 

Finally, another caution. Deficient diaphragm action, which is not rarely 
seen in alcoholics, and especially in patients convalescing from diphtheria, 
requires exceedingly careful treatment. Here the patient must refrain from 
all exertion, and his strength must be maintained by nutritious food and the 
administration of wine or other stimulant. This condition of debility and 
4G 



698 PARALYSES OF THE PERIPHERAL NERVES 






paralysis of the most important nerves of respiration may be combated also 
by subcutaneous injections of strychnin, by cautious galvanic treatment of 
the neck, and by systematic faradization of the phrenic nerves. 

9. PARALYSIS OF THE SUPRASCAPULAR AND POSTSCAPULAR 

NERVES 

Paralyses rarely isolated are those of the suprascapular and postscapular 
nerves. 

Hardly a dozen cases have been observed of paralysis of the first mentioned 
nerve, which innervates the supraspinatus and infraspinatus. Among the 
etiologic factors we recognize the pressure of heavy loads upon the shoulder, 
shock to the shoulder from a fall, torsion of the nerves of the brachial plexus, 
inflammation of nerves (neuritic conditions) in consequence of exposure to 
cold and, in a curious case observed by Bettmann, a congenital absence of 
these muscles. 

The supraspinatus is less used in rotating the arm outwardly than the 
infraspinatus; as Duchenne emphasized, the muscle is an active ligament of 
the humeral joint, and when paralyzed the arm droops, hence throws addi- 
tional labor on the actual lifter of the arm, the deltoid, which is most 
active in raising the arm. This produces a certain weakness of which the 
patient complains when he attempts to raise his arm. After it has been 
raised to a horizontal position, a visible effort as though to overcome some 
obstruction seems necessary before it can be further elevated to a vertical 
position. 

The function of the infraspinatus which rotates the arm outwardly is 
more clearly developed; if the muscle is paralyzed this rotation ' becomes 
difficult but is never impossible. 

Here the teres minor and the posterior portion of the deltoid may per- 
haps render assistance. Duchenne reported that paralysis of the infraspinatus 
prevented the movements for writing, embroidering, the drawing of long 
threads in sewing; I once observed this in a man who, although he experi- 
enced no difficulty in writing, was unable to draw a needle outward in 
sewing. 

Symptoms. — Besides the functional disturbances just enumerated under 
the symptomatology of paralysis of the suprascapular nerve, atrophy in both 
grooves of the shoulder-Made, particularly the lower, is evident. This atrophy 
is less readily detected in the supraspinatus because of the intact trapezius; 
however, the infraspinatus fossa may look as if excavated and the bone may 
be directly palpated. 

Among the sensory disturbances which may be experienced, pain is rarely 
absent at the onset of the disease and is usually of most importance, while 
objectively demonstrable disturbances of sensation are either insignificant or 
wholly absent. 

The electric contractility of the paralyzed muscles is more or less decreased 
according to the severity of the affection. This is readily determined in the 
infraspinatus for the muscle may be directly irritated, and it may also be 
directly caused to contract from Erb's point; in severe lesions of the nerves, 
this reaction is, of course, entirely absent. 



PARALYSIS OF THE SUPRASCAPULAR AND POSTSCAPULAR NERVES 699 

In the diagnosis of paralysis of the suprascapularis not only is pain in 
the region of the shoulder-blade significant but inspection of the shoulder- 
blades is also important, for here the excavation of the infraspinatus fossa 
may be at once discovered. Limitation of power to raise the arm freely, the 
difficulty of outward rotation of the arm, and the intactness of the other 
muscles of the shoulder-girdle facilitate the diagnosis of an isolated paralysis 
of the suprascapular nerve. 

Although we have stated that isolated paralyses of the muscles of the 
shoulder-girdle are rare, it must nevertheless be noted that this form of paral- 
ysis is not infrequently combined with paralysis of other muscles of the 
shoulder. These muscles, as we shall see later, may be implicated in Du- 
chenne-Erb's plexus paralysis, in the so-called obstetric paralyses of children, 
in neuritic processes which affect the nerve areas of the shoulder and arm, 
and in the so-called humero-scapular form of spinal progressive muscular 
atrophy; finally, a fact which Hoffmann has clearly demonstrated, these mus- 
cles are also implicated in syringomyelia, and this must be borne in mind 
(also the characteristic sensory disturbances of this affection) to protect us 
from error. 

No severe disturbance is produced by paralysis of the nerves which inner- 
vate these muscles, and the patient of Bettmann's mentioned above was even 
said to be an athlete; nevertheless, the difficulty in raising the arm and the 
possible prevention of its outward rotation will make the patient seek pro- 
fessional aid in order to rid himself as soon as possible of these disabilities. 

Treatment. — I shall not here discuss the treatment of a possible neuritis 
which causes such paralysis as we have been considering; the sequels of neu- 
ritis or trauma, producing paralysis of the muscles of the shoulder-girdle, are 
treated according to the fundamental principles of electro-therapy, with the 
addition of all procedures, such as massage, etc., which in similar cases have 
an abortive or curative influence on the disease. 

Still more rare than paralysis of the suprascapular nerve is isolated paral- 
ysis of the nervus dorsalis scapula?. The nerve supplies the levator scapulae, 
the rhomboideus and the superior serratus. Of these muscles the posterior 
serratus draws the upper ribs upward and posteriorly, and during respiration 
assists the thorax to expand. The levator scapulae, as its name indicates, raises 
the shoulder-blade; this acts simultaneously with the upper portion of the 
trapezius and the rhomboideus, the latter muscle drawing the shoulder-blade 
upward, backward, and inward toward the vertebral column. 

Aside from the occasional implication of these muscles in the paralysis 
and wasting of progressive muscular atrophy, these muscles, or, rather, the 
dorsalis scapular nerve, is implicated in neuritic processes which attack the 
cervical nerves after infectious diseases, syphilis, etc. A paralysis of the ser- 
ratus posterior can scarcely be determined externally. In paralysis of the 
levator scapulae the shoulder can be only partially raised; if the rhomboidei 
are implicated, the maximal approximation of the shoulder-blade to the verte- 
bral column is incomplete and, as Mollier has shown, the power of dropping 
the arm with force is reduced by the insufficient fixation of the scapula. 

When the arm is at rest, paralysis of this nerve is denoted by the 
low position of the shoulder-blade and its remoteness from the vertebral 
column. 



700 PARALYSES OF THE PERIPHERAL NERVES 

The treatment of such paralysis does not differ from that of other forms ; 
considering its etiologic factors (syphilis), electro-therapy should first be 
tried. 

10. PARALYSIS OF THE ANTERIOR THORACIC AND 
SUBSCAPULAR NERVES 

Paralysis of these nerves alone is exceedingly rare. 

The first mentioned nerves innervate the pectoralis major and minor, 
as well as the clavicular portion of the deltoid ; these muscles are occasionally 
implicated in progressive muscular atrophy, in unilateral paralysis of a cere- 
bral nature, and in injuries of the brachial plexus. 

If they are paralyzed, particularly the pectoralis major, the arm cannot 
be thrust forward or pronated; and it is either difficult or impossible to place 
the hand upon the opposite shoulder. In atrophy which follows severe paral- 
ysis of the muscles in question, the infraclavicular fossa becomes so deep that 
the upper intercostal spaces may be seen and palpated. 

We know that various muscles of the body may be congenitally absent. 
This fact has been previously mentioned in discussing paralysis of the tra- 
pezius. Absence of some of the muscles of the chest has also been noted. 

An interesting fact which occasions surprise is that most authors who 
have seen these conditions emphasize the very slight motor disturbance pro- 
duced by this congenital absence. The persons affected have developed into 
fine gymnasts and swimmers, which is perhaps accounted for by the vicarious 
function of muscles that have hypertrophied, such as the deltoid, the sub- 
clavius, and others. In the differential diagnosis, when deciding whether in 
the given case we are dealing with a congenital defect or with progressive 
muscular atrophy, the absence of muscles in other parts of the body, the 
absence or distortion of individual members (curvature of the fingers, absence 
of individual phalanges, the union of the fingers, webbed fingers, etc.) may 
be a guide as well as a carefully elicited history which may prove that the 
malformation existed from birth. 

Without minutely discussing this condition, I must mention that com- 
petent authorities have expressed the opinion that besides the so-called con- 
genital defects of individual muscles there is a rudimentary form of pro- 
gressive muscular dystrophy. 

Like the anterior thoracic, the subscapular nerves are rarely paralyzed 
alone. When this occurs, we have paralysis of the subscapular muscles, the 
teres major, the latissimus dorsi, and the inferior serratus. 

Nearly all of these muscles rotate the arm inwardly; the latissimus dorsi 
also draws the arm backward, and lowers it after it has been raised. Paralysis 
of the previously mentioned muscles, by the action of their antagonists, espe- 
cially the infraspinatus and teres minor, produces an outward rotation of the 
arm which decidedly hinders all those movements in which the arm is rotated 
inwardly. 

An important etiologic factor of paralysis of the muscles under consider- 
ation is progressive muscular atrophy (perhaps also excessive use), which 
causes abnormal distention or torsion of the latissimus dorsi. 

In paralysis of the latissimus dorsi alone, only a few cases of which have 



PARALYSIS OF THE THORACICUS LONGUS NERVE 701 

been observed, we know from what we have learned regarding the function 
of the muscle that the arm can only with difficulty be thrown backward or 
lowered. As the upper border of this muscle covers the angle of the scapula 
and presses it upon the thorax, it is apparent that when paralyzed the lower 
angle of the shoulder-blade will escape from this fixed position and be drawn 
downward, a movement which is assisted by the action of the coracobrachialis, 
the pectoralis minor, and the short head of the biceps, all of which draw 
the shoulder-blade forward from the coracoid process. 

11. PARALYSIS OF THE THORACICUS LONGUS NERVE 
(PARALYSIS OF THE SERRATUS) 

Among the most interesting paralyses of the upper extremities are those 
of the long thoracic nerve which innervates the serratus anticus major (ser- 
ratus magnus). 

In the study of paralysis of these muscles, our views have lately been con- 
siderably modified and our knowledge supplemented by the investigations of 
many authors, among whom I must mention Mollier and Steinhausen. For 
instance, we know to-day that there may be not only complete paralysis of 
this muscle but paresis, that is, partial paralysis; the upper portion of the 
serratus is of unique interest inasmuch as it frequently does not become 
paralyzed. The upper fibers of the nerve most often escape the injury which 
damages those extending down to the supraclavicular fossa. In the discussion 
of the etiology this will be more minutely considered. 

The muscle draws the shoulder-blade outward and forward, thus pressing 
it against the thorax, and draws the inner median border of the scapula away 
from the vertebral column. Here we observe (and this is evident in normal 
persons) that the serratus contracts long before the arm reaches a horizontal 
plane; the elevation of the arm is not due, as was formerly assumed, to the 
combined and equal action of the deltoid and serratus, but the latter com- 
bined with the trapezius supplies only about one-third of the necessary force, 
the other two-thirds being conveyed by the abductors of the arm. 

The etiologic factors may be any injury which can possibly affect the long 
thoracic nerve in its course through the supraclavicular fossa. Besides those 
due to gross trauma (such as incised wounds, direct blows, gunshot injuries) 
the cases are interesting in which compression of the nerve occurs from the 
pressure of heavy loads carried upon the shoulder, or in which the nerve is 
unilaterally damaged by the continuous movement of the arm in some definite 
occupation (as in shoemakers, carpenters, farm hands, seamstresses, etc.) 
and by the contraction of the scalenus medius through which the nerve passes. 

The stretching and torsion to which the nerve is subjected in some move- 
ments (as lifting down of heavy objects from a considerable height, torsion 
during gymnastic exercises, sudden spasmodic action of the muscle when a 
threatened fall is prevented by extending and bracing the arms) may occa- 
sionally produce paralysis of the serratus. 

Other cases are known in which paralysis immediately followed wetting 
or chilling, infectious diseases (enteric fever, diphtheria, influenza, and in the 
puerperium), and severe rheumatic affections of the shoulder- joint. Paralysis 
of the nerve not due to wounds in the supraclavicular fossa but to trauma 



702 PARALYSES OF THE PERIPHERAL NERVES 

affecting other regions (incised wounds of the axillary cavity or extirpation 
of diseased lymph-glands in this area) is rare. Besides other muscles of the 
shoulder-girdle, the serratus magnus is implicated in progressive muscular 
atrophy and in infantile spinal paralysis; paralysis of the serratus occasion- 
ally occurs in hysteria. 

Symptoms. — In the cases of progressive muscular atrophy just mentioned, 
whether of spinal origin or occurring in the course of dystrophia muscularis 
progressiva, the paralysis of the muscles is usually bilateral; on the other 
hand, peripheral bilateral paralysis of the serratus seldom occurs. 

In the majority of cases paralysis of the serratus causes no conspicuous 
disturbance of sensation; but at the onset of those paralyses which appear as 
neuritic inflammation, and also after so-called rheumatic damage, severe 
pain may arise from the implication of other sensory nerves belonging 
to the cervical plexus, and may subsequently radiate upward and down- 
ward. 

There was a time when authors were not unanimous in regard to the 
position of the shoulder-blade during rest with the arm hanging down at the 
side of the body. The most diverse opinions were held as to the possibility 
of an oblique position of the scapula during rest; Berger was of the opinion 
that the medial border of the shoulder-blade extended obliquely from above, 
and outward, downward, and inward; other authors quite as distinguished 
maintained that the medial border was parallel with the vertebral column. 
According to the latest investigations these conditions alternate. A slight 
rocking motion is observed in quite isolated paralyses of the serratus; in any 
case, they fail to prove a special implication of the lower portion of the tra- 
pezius, which in fact is frequently affected simultaneously by the same dam- 
aging factors. 

The condition is different when the position of the scapula is changed 
(with an intact trapezius) ; this approximates it to the vertebral column, 
and shows it to be higher than that of the normal side; even in rest the 
spinal border of the shoulder-blade, especially its lower angle, ranges some- 
what posteriorly. The antagonists of the serratus, the rhomboidei, the tra- 
pezius, and the levator anguli scapulas, are the ones which produce this higher 
position and the approximation to the vertebral column; the muscles which 
are inserted into the coracoid process, the pectoralis minor, the coraco- 
brachialis and the biceps, draw the shoulder-blade forward and lift it out 
of the encircling upper border of the latissimus. 

The conditions in paralysis of the serratus to some extent resemble those 
of paralysis of the facial nerve. When the patient is quiet some physicians 
are unable to discern anything abnormal, but they perceive the anomaly as 
soon as the patient makes any movement. I saw a young seamstress who, 
a few weeks before she presented herself at the Clinic, suffered from very 
severe pain in the right shoulder region and was soon afterward unable to 
raise her arm, or, when I requested her to do so, she could merely raise it 
to a horizontal plane. Instead of removing the internal border of the shoul- 
der-blade from the vertebral column, as on the normal side, it was, on the 
contrary, approximated, and displaced by the rhomboideus and the trapezius. 
When I told her to move the arm from the frontal to the sagittal plane, she 
raised the internal border of the scapula and the lower angle from the thorax, 



PARALYSIS OF THE THORACICUS LONGUS NERVE 703 

and the shoulder-blade projected posteriorly like a wing. The inner surface 
of the scapula could readily be palpated. 

This position of the shoulder-blade in the sagittal plane of the horizontally 
raised arm is a pathognomonic sign of serratus paralysis. The muscle which, 
in normally performing this movement, holds the shoulder-blade to the thorax 
during the contraction of the deltoid, that is, the serratus, cannot perform 
its function because the antagonists alone, those muscles arising from the 
coracoid process as well as from the vertebral column, act upon the shoulder- 
blade. 

Such conspicuous conditions as in the case just detailed are not always 
found; we know to-day that the arm may be raised above the horizontal, 
even to a vertical position. Here the muscles which act vicariously are usu- 
ally hypertrophied, especially the middle and lower portions of the trapezius, 
also the infraspinatus and the posterior portions oi the deltoid which com- 
pensate for the deficient or imperfect function of the serratus; at other 
times there is only partial paralysis of the serratus alone; a condition which 
Steinhausen observed in soldiers, and which may be present for years with- 
out the individuals in question or even the examining physician suspecting 
its existence. 

How important the function of the serratus is for the complete elevation 
of the arm is shown by the fact that when the shoulder-blade is pressed against 
the thorax and simultaneously shoved downward and outward the raising 
of the arm, previously impossible, is found to be very easy. 

That derangements of motion from serratus paralysis are disabling for 
the person in question is shown by the following: Upon raising the arm it 
cannot be projected forward with any power, hence the disease must in many 
cases be regarded as severe, and greatly reduces the patient's capacity for 
labor. 

If neuritic pains, to which I have already called attention, have preceded, 
no characteristic disturbance of sensation is subsequently noted in serratus 
paralysis. In the peripheral paralyses which run their course with complete 
DeR (aside from the cases due to progressive muscular atrophy) we fre- 
quently perceive an atrophy of individual areas of the serratus on the dis- 
eased side which is absent in milder cases. 

As in all peripheral paralyses, mild, medium and severe forms occur in 
which the electric contractility may be readily determined by an electro-diag- 
nostic examination, and this should never be omitted. 

A point in the diagnosis is the peculiar change in the position of the 
shoulder-blade on attempting to raise the arm to the frontal plane; this is 
even more marked in the sagittal. Contrasted with this, the change in the 
position of the scapula when the arm is at rest is difficult to recognize and 
unimportant. Since it has been recently demonstrated that mild and also 
partial paralyses of the serratus certainly occur, the patient's chest must 
be bared during the examination if we wish to avoid error. 

We know that in partial paralysis, just as in normal conditions, the arm 
may be raised far above the horizontal plane, and we know, too, that when 
the function of the serratus is absent the middle and lower portions of the 
trapezius, the supra- and infraspinatus, and the deltoid vicariously perform 
these functions; hence, if the patient does not uncover his chest and shoul- 



704 PARALYSES OF THE PERIPHERAL NERVES 

ders, the actual condition may be readily overlooked. We must bear in mind 
that paralysis of the serratus is often associated with a similar condition 
in the middle and lower areas of the trapezius, because the injury which 
affects the long thoracic nerve may also implicate the neighboring branches 
belonging to the cervical plexus. The history and a minute inspection of 
the affected thorax will soon show whether the paralysis of the serratus is 
merely an accompanying process affecting other muscles (as in progressive 
muscular atrophy from various causes) or whether we are in fact dealing 
with a paralysis of this nerve and muscle alone. It must be observed that 
this form of paralysis rarely occurs in children, it is much more frequent in 
men than in women, the working classes are particularly prone to be affected, 
and the right side, as will readily be understood, is more often implicated 
than the left. 

Serratus paralysis can never be regarded as a mild affection, for it may 
seriously impair the function of the arm. We have stated that the vicarious 
action of other muscles of the shoulder may compensate for the loss of power 
in the serratus, and although the prognosis is by no means favorable, yet 
when electric examination shows only a slight or moderate degree of paralysis, 
recovery may occur in the near future. 

Of course the prognosis is more unfavorable when electric examination 
reveals a profound paralysis or when the paralysis is an accompanying symp- 
tom of advancing muscular or spinal disease. 

Treatment. — In the prophylaxis we must prohibit the immoderate use of 
the arm in any special way, also all exercises which cause prolonged tension 
of the nerves of the neck (gymnastic exercises). If, as often happens, there 
is pain in the early stages, this may be combated by hydrotherapy or anti- 
neuralgic remedies. In any case electro-therapeutic treatment should be insti- 
tuted by the daily application for two or three minutes of the galvanic cur- 
rent (5 to 6 milliamperes, the cathode in the supraclavicular fossa, the anode 
to the nape of the neck) and by labile stroking of the muscles in the axillary 
region with the cathode; finally the attempted stimulation of the nerves and 
muscles by the faradic current. 

In bilateral paralysis of the muscles of the shoulder-girdle due to pro- 
gressive muscular atrophy, the question whether operative procedures such 
as were once attempted by a prominent surgeon (who sewed together the 
shoulder-blades) will find their way into practice must be decided in the 
future. 

In the course of this description I have several times referred to Mol- 
lier's 1 investigations of the statics and -mechanics of the shoulder-girdle ; for 
obvious reasons I must refrain from describing minutely the results which 
this investigator obtained. Yet I trust enough has been said to enable the 
practitioner in a given case to diagnosticate correctly a paralysis of the tra- 
pezius and serratus. Very important work has been done in this field. Mol- 
lier's original book is not of easy access to all, but there are more or less 
detailed references to it which give a synopsis of the most important points. 

i S. Mollier, Jena, 1899, Fischer. 



PARALYSIS OF THE AXILLARY (CIRCUMFLEX) NERVE 705 

12. PARALYSIS OF THE AXILLARY (CIRCUMFLEX) NERVE 
(DELTOID PARALYSIS) 

Isolated paralyses in the course of the circumflex brachialis or axillary 
nerve are not so frequent as paralysis of the radial nerve, nevertheless they 
are not rare. Thus, paresis and disturbance of function in that muscle most 
involved in paralysis of the axillary nerve, that is, the deltoid muscle, are the 
most frequent accompaniments or sequels of rheumatic or traumatic inflam- 
mation of the shoulder-joint. As in all paralyses of peripheral nerves, so 
also in those of the axillary nerve, injuries form the most important etiologic 
factor, whether the muscle be directly injured by a fall, a contusion or a 
blow, or whether the immediate cause be a blow, a gunshot wound or stab of 
the nerve itself, compression from fractures of the upper arm, or luxations 
of the upper arm. Like other peripheral nerves, the circumflexus brachii is 
attacked by neuritic processes after infectious diseases, intoxications, or con- 
stitutional diseases; for example, after enteric fever, influenza, lead poison- 
ing, gout or diabetes. It may be readily understood that tumors in the axil- 
lary region or in the supraclavicular fossa may occasionally damage the nerve 
either alone or in association with other nerves of the arm. 

Occasionally unilateral, more frequently bilateral, paralyses of the muscle 
occur after prolonged operations under anaesthesia during which one or both 
arms have for a long time been hvpercxtended and abducted; paralyses have 
occurred during sleep in consequence of one or both arms being extended 
above the head, or from the direct pressure of the body upon this muscle 
during sleep or in following an occupation (for instance, that of miners who 
lie upon the left side continuously while loosening ore, Seeligmiiller). 

I must also mention paralysis of the deltoid as an accompanying symptom 
of other neuritic or central processes (progressive muscular atrophy, syringo- 
myelia, etc.) and the implication of this muscle in paralysis of the plexus or 
lead paralysis, in hemiplegia and paralysis of hysterical character. 

Symptoms. — The principal symptom of axillary paralysis is paralysis of 
the deltoid muscle. This nerve gives off branches to the teres minor and 
the capsule of the shoulder-joint, also main branches such as the posterior 
cutaneous brachial nerve, but the lesions of these branches are so insignificant 
in comparison with paralysis of the deltoid that they scarcely merit descrip- 
tion. In severe paralysis of the deltoid it is impossible to raise the arm 
or to lift it above the thorax, at least at first; the performance of this act 
necessitates many important movements of the arm which have become impos- 
sible. If the profound paralysis persists for a long time the muscle emaciates, 
the arm glides downward and is no longer fixed in the glenoid cavity. 

If paralysis of the nerve is due to neuritic processes, pain may be present 
from the first (and this occurs also in paresis of the deltoid due to inflamma- 
tion of the shoulder- joint). If an accurate examination be made in those 
cases in which paralysis is attributed to a lesion of the axillary nerve, we find 
a more or less developed hyperesthesia (or anesthesia) which has frequently 
been described; this consists in a cutaneous disturbance of sensation in the 
upper and posterior third of the upper arm. The patients themselves com- 
plain of it but little and the disturbance can only be detected by some effort. 

The anomalies of electric contractility in the damaged nerve and muscle 



706 PARALYSES OF THE PERIPHERAL NERVES 

are usually parallel with the severity of the lesion ; thus electro-diagnostically 
we sometimes find slight, sometimes moderate, at other times severe forms of 
paralysis. In electric examination, those pareses of the deltoid which follow 
inflammation of the shoulder- joint usually show only a quantitative diminu- 
tion in electric contractility. It must be remembered that even in severe 
paralysis the median bundles of this muscle often remain exempt because 
supplied by fibers of the anterior thoracic nerve. Two other facts must be 
mentioned which occasionally perplex those not familiar with them ; first, that 
in exceptional cases lead paralysis (which otherwise first affects the extensor 
muscles of the hands and fingers) may immediately attack the deltoid without 
this muscle showing any change in electric reaction. Secondly, what is per- 
haps more rarely observed, in lead paralysis the activity of the muscle is 
apparently unimpaired, nevertheless electro-diagnostic examination reveals the 
existence of marked reactions of degeneration. But these most interesting 
conditions cannot at this point be minutely discussed. 

When it is borne in mind that by a carefully elicited history and electric 
examination (even though only with the faradic current) deltoid paralysis 
has been diagnosticated in persons who applied for treatment of a joint in- 
flammation or trauma, we should also note whether pain has preceded the 
onset of paralysis; and on considering the occupation of the patient (a 
worker in lead, etc.) it can scarcely be difficult to recognize the cause of the 
deltoid paralysis and with the aid of the symptomatology to make a correct 
diagnosis. If infectious diseases have preceded the paralysis, if the exami- 
nation of the patient reveals symptoms of a chronic affection of the spinal 
cord (progressive muscular atrophy, syringomyelia, etc.), it is apparent in 
the given case that paralysis of the muscle is only one phenomenon of an 
extensive clinical picture. 

Finally we must remember that hysterical paralyses after trauma are not 
very rare, even in men. By the retention of electric contractility these may 
be readily differentiated from severe paralysis which characteristically impli- 
cates the entire arm and shows sensory disturbance. They are also often 
marked by a surprisingly rapid disappearance of all the symptoms, and this 
again distinguishes them from paralysis of other origin. 

The prognosis and treatment of deltoid paralysis vary according to the 
severity of the inflammation of the shoulder- joint or the trauma. The direct 
treatment of the nerve in muscular paralysis must be preceded by proper 
surgical measures. Only after inflammation has subsided, a dislocation has 
been reduced, or a fracture has been properly set, may we begin massage, 
baths, etc., combined with electro-therapeutic treatment. We need scarcely 
emphasize that any existing anemic conditions, diabetes, or lead poisoning, 
must not be overlooked in the treatment. 

In conclusion, if rest or support is to be given to an inflamed joint the 
patient may be ordered to wear a brace; in other cases of longer existing 
paralysis, it is sometimes advisable, as various authors have recently recom- 
mended, for the patient to practise systematic exercises which increase the 
activity of the humero-scapular muscles (the supra- and infraspinatus, per- 
haps also the pectoral muscles, the trapezius, the coracobrachialis, etc.) and 
thus compensate for the loss in muscular power due to paralysis of the del^ 
toid. Very satisfactory results have been obtained in this way. 



PARALYSIS OF THE MUSCULOCUTANEOUS NERVE 707 

13. PARALYSIS OF THE MUSCULOCUTANEOUS NERVE 

Isolated paralyses of the musculocutaneous nerves (perf oralis Casserii) 
are rare occurrences. Fourteen cases are reported in literature. The causes 
of this paralysis are those with which we are already familiar: Compression 
of the upper arm, a fall upon the shoulder, tumors in the supraclavicular 
fossa, gunshot wounds, etc. In a few cases the cause may have been excessive 
use of the arm or a neuritis limited to this particular nerve. Everything pre- 
viously stated applies also to isolated paralyses of this nerve. In association 
with injury of other nerves of the upper extremity, the musculo-cutaneous 
nerve is also frequently damaged; as we shall see, it plays an important role 
in the symptomatology of the so-called Duchenne-Erb paralysis. 

Symptoms. — We shall first consider the symptoms due to motor loss, and 
here we find that in paralysis of this nerve the muscles which it innervates, 
the coracobrachialis, the biceps, and the brachialis internus, lose their func- 
tion. The flaccid lower arm cannot be supinated, and can be flexed upon the 
upper arm only by the vicarious action of the supinator longus. Of course 
the brachialis internus is not wholly innervated by the musculo-cutaneous 
nerve; the musculo-spiral also sends it branches. In regard to the paralytic 
implication of the coracobrachialis, which is also supplied by this nerve, its 
intact state is particularly emphasized in the majority of the reports which 
mention it at all. 

As the sensory portion of the perforans Casserii supplies the skin on the 
radial side and the internal and external surface of the forearm as far as 
the ball of the thumb and the palm of the hand with sensory fibers, injury 
of the nerve causes a subjective sense of numbness in these areas; in isolated 
cases the volar side of the thumb is also implicated. 

If the damage to the nerve is severe, this is manifest by changes in the 
electric contractility; of course, these may be either simple, quantitative 
diminutions of contractility, moderate degrees, or well developed reactions of 
degeneration. It has been repeatedly observed that the portion of the brachi- 
alis internus innervated by the musculo-spiral nerve may react normally. 

The atrophy of the affected muscles which always appears in severe paral- 
ysis gives to the upper arm of the patient a characteristic appearance : From 
the normal deltoid to the supinator longus the upper arm is emaciated owing 
to atrophy of the biceps and a large portion of the brachialis internus. 

After what has been said of the disturbance of motion and sensation in 
these cases, the diagnosis of isolated musculo-cutaneous paralysis cannot be 
difficult. Even a superficial examination reveals to us whether the paralysis 
is independent and isolated or is a part of other processes; I refer particu- 
larly to the combined paralyses of shoulder and arm of the Duchenne-Erb 
type, which will later be described. Of course, neuritic processes may affect 
this nerve simultaneously with others in the upper extremities; and by a 
fracture of the humerus or luxation of this bone it may be injured simul- 
taneously with other nerves. 

I must call attention to the possibility that isolated paralysis of this nerve 
may be confounded with the muscular atrophy which accompanies Erb's 
juvenile dystrophia and particularly affects the flexors of the forearm. To 
avoid error we must bear in mind that in Erb's disease there is no history 



708 PARALYSES OF THE PERIPHERAL NERVES 

of trauma; but, on the contrary, the affection exists from early youth, or at 
least for many years. The careful examination of the patient, the compari- 
son of the atrophic muscles with normal or hypertrophied muscles in the 
same member, and the absence of sensory disturbances, etc., will soon reveal 
the true condition. 

The prognosis of an isolated paralysis of the musculo-cutaneous nerve 
depends upon the severity of the original injury and the degree of electrical 
reaction; the treatment should be chiefly electric, and with perseverance on 
the part of the physician and the patient this will often lead to satisfactory 
results. 

14. MUSCULOSPIRAL (RADIAL) PARALYSIS 

In my experience the most frequent form of paralysis in the upper extrem- 
ity is musculo-spiral paralysis. A common, if not the most common, cause is 
the pressure to which the arm is subjected during sleep because the head or 
the whole body of the sleeper rests upon the exit of the musculo-spiral or the 
area a little above this point while the arm rests on a hard surface. In rare 
cases this pressure acts lower down. The clinical picture may be best illus- 
trated by quoting the history of a young workman who was attacked by right- 
sided musculo-spiral paralysis which occurred in the manner described. 

When I directed the patient to extend both hands and at the same time the fingers 
he succeeded in accomplishing this only on the left side ; the right hand as well as 
the fingers hung down in volar flexion. Neither hand nor fingers could be raised to 
a horizontal position, to say nothing of dorsal flexion ; the thumb was flexed on its 
phalanges under the other fingers and could neither be abducted nor extended. On 
account of paralysis of the extensor carpi radialis longus and brevis and the extensor 
carpi ulnaris there Avas difficulty in abducting and adducting the hand, these move- 
ments being normally performed by the muscles just referred to with the combined 
action of the corresponding flexors. Here I must mention a fact which in my experi- 
ence is frequently overlooked; namely, that the inability to extend the fingers is actu- 
ally true only of the basal, the proximal phalanges, for when I passively extended 
them the patient at once extended the middle and distal phalanges, the interossei 
muscles which are supplied by the ulnar nerve and which control this movement not 
being implicated in the process. 

The musculo-spiral nerve supplies both spinators as well as the extensors of the 
hand and fingers. When I told the patient to supinate the extended lower arm on 
the upper arm he could not perform this movement on account of paralysis of the 
supinator brevis. 

That the supinator longus is involved in the paralysis is most evident when the 
patient flexes the arm and tries with all hjs might to keep it flexed while the physician 
attempts to extend it. During this procedure the contours of the supinator longus 
become very prominent in the normal arm of the patient, but not on the paralyzed 
side; we there feel only the flaccid belly of the muscle. 

We must now investigate the function of another extensor of the arm which is 
innervated by the musculo-spiral nerve, the triceps. In the case under discussion this 
muscle escaped paralysis, and the patient could extend both the raised arm and the 
flexed lower arm. This is not surprising when we remember that the causative factor, 
pressure, was exerted below the point where branches are given off for the triceps, as 
is usually the case in radial paralysis. If, as sometimes occurs, there is compression 
on the trunk of the nerve in the axillary cavity, for example, from an insufficiently 
padded crutch, from gunshot wounds or stabs in the axillary cavity, or from high 
fractures or dislocations of the humerus, the triceps as well as other muscles inner- 
vated by the musculo-spiral nerve may be paralyzed; in rare cases the triceps may be 
the only muscle involved. 



MUSCULOSPIRAL (RADIAL) PARALYSIS 709 

Before considering the history of this patient further, I must state that to-day 
even less than ever do we ascribe to exposure to cold a predominant role in the etiology 
of radial paralyses. The majority of those afflicted with radial paralysis belong to 
the working classes, and besides the etiologic factor of pressure upon the nerve a 
tendency to the abuse of alcohol here comes into question, the nerves of these persons 
being more susceptible to external damage than the nerves of non-alcoholics. Besides 
from pressure on the musculo-spiral nerve during sleep and its injury from fractures 
and dislocations of the upper arm, from blows, from gunshot wounds or stabs, pressure 
paralysis occurs in nurslings from their being too closely wrapped in clothes, in the 
arms of persons who are tightly held by the police when arresting them, from tight 
bandages after surgical operations, from too great constriction of the arm by an 
Esmarch bandage, and in some occupations in which the axillary cavity of the work- 
man is subjected to too prolonged or abnormally great pressure. In this category 
probably belong a number of so-called anesthesia paralyses of the radial nerve which 
occur during prolonged anesthesia from pressure of the head of the abducted and 
hyperextended humerus ; the nerve near its exit may be pressed against the hard sur- 
face of the operating table; or it may occur in other ways. We must also include 
among these musculo-spiral paralyses those cases which are caused by suspension, as 
in Motschukowski's method of treating tabes dorsalis. Here I must mention that in 
the course of tabes, apparently without external cause and spontaneously, paralysis. of 
various nerves, among them the musculo-spiral, may occur, and these like other periph- 
eral paralyses may improve while the underlying affection, tabes, persists. 

We have already spoken of radial paralyses from various injuries of the upper 
arm, and we must now consider those which follow a fracture of the bones of the lower 
arm, especially of the head of the radius in adults, as well as that occasionally 
observed in the new-born. 

It was noticed in my patient, as well as in many others, that radial paral- 
ysis due to pressure during sleep is only partial, for, as I have stated, the 
triceps is always exempt. This muscle is implicated in the paralysis only 
in the case of trauma of the axillary cavity or in injuries of the entire plexus. 

In a patient who suffered from lead paralysis some of the muscles innervated by 
the musculo-spiral nerve characteristically escaped paralysis. Before giving his his- 
tory, I shall briefly refer to the not very rare radial paralyses due to the subcutaneous 
injection of ether in the forearm. These paralyses in which the supinators and 
extensors of the hand are usually exempt are due to the deleterious effect of ether upon 
the nerve substance ; we should therefore be extremely careful when injecting this sub- 
stance into areas where there are superficial nerves which may be easily damaged. 

Other poisons as well as lead may attack the musculo-spiral nerve either 
alone or conjointly with other nerves; among these I must mention arsenic, 
alcohol, carbonic oxid, carbon bisulphid, etc. Xeuritis and polyneuritis, which 
frequently occur after various infectious diseases, such as enteric fever and 
pneumonia, also attack the musculo-spiral nerve, and abnormal distention of 
the nerve, as when one falls with the hand in volar flexion or when the nerve 
is pressed against the humerus by the forcible contraction of the triceps after 
an abnormal and sudden movement, may result in radial paralysis. 

We must still consider the sensory disturbances in the case of the first 
patient, which also occurred in other patients attacked by the same disease. 
Usually these are very slight. They affect the area of the superficial radial 
nerve, and are manifested by abnormal sensations, numbness, formication, 
etc., rather than by demonstrable hyperesthesia; severe anesthesia occurs only 
in exceptional cases. Some of the skin on the ball of the thumb, the skin 
on the radial side of the root of the hand, and the middle hand as far as 



710 PARALYSES OF THE PERIPHERAL NERVES 

the third metacarpal bone, also the skin over the proximal phalanges of the 
thumb and first two fingers and perhaps of the third on the radial side is 
implicated. In high lesions of the nerve, the disturbances of sensation are 
observed in the skin of the posterior aspect of the upper arm, and possibly 
down to the wrist on the extensor side of the forearm (the superficial cutane- 
ous nerve, external branch). 

It should be remembered that, even in severe musculo-spiral paralysis, 
there is sometimes no sensory disturbance, a condition which may perhaps 
be attributed to the extensive compensatory innervation, particularly in the 
nerves of the hands and fingers. 

There are no marked trophic disturbances except a decided emaciation of 
the extensor muscles on the posterior surface of the lower arm in severe cases. 

Among these we should perhaps reckon the swelling of the tendon sheaths 
of the extensors of the fingers on the back of the hand, which was first de- 
scribed by Gubler; this was conspicuous in the patient with lead poisoning 
whose history is soon to be related. Whether these are really trophic disturb- 
ances or whether the swelling is due to torsion of the tendons and their 
sheaths from the drooping of the paralyzed hand cannot be positively deter- 
mined. 

Before considering the electric reactions of musculo-spiral paralysis, 
which, with certain modifications, are also observed in all peripheral paralyses, 
I will refer to the history of a painter who was attacked by lead paralysis 
(paralysis saturnina) . 

Although the motility of the left hand of this patient was not quite intact, yet 
the right hand was chiefly affected. When I told him to flex dorsally the hand which 
was made into a fist he could do so but the hand deviated to the ulnar side. Both 
the radial extensors of the hand were at least paretic. In extending the fingers the 
second and third drooped below the horizontal plane, and the index and little fingers 
barely reached this line. On the other hand, the movements of the thumb were quite 
free. In regard to the active movements of the triceps and both supinators, it was 
observed that in this patient these muscles moved normally. 

When we considered that this man was a painter ( often working with lead paints ) , 
that he had not suffered from trauma, that the paralysis came on during the day 
while he was at work and not after sleep, that he was pale and cachectic with a lead 
line on his gums, even without an electric examination we readily diagnosticated a 
case of radial paralysis due to lead poisoning. 

It is well known that radial paraljsis is rarely the first symptom of lead poisoning. 
In the majority of such cases it is a later symptom, it attacks the patient years 
after he begins to work with lead, and other signs of lead intoxication (attacks of 
colic, pains in the limbs, especially in the joints, tremor, etc.) have usually preceded. 
Besides the occupation of the patient, we find a guide to the diagnosis in the almost 
invariable exemption of the supinators and the triceps, and particularly in the bilateral 
nature of the radial paralysis and the results of electric examination. Naturally, the 
bilateral character rarely develops to such an extent that the sides are affected with 
equal severity; as a rule, the extremity most used is the one most severely implicated. 

When we examined diagnostically the electric contractility of the paralyzed radial 
region in the patients whose histories have been given, a marked difference in reaction 
could be perceived. In the patient with pressure paralysis from sleeping on his arm 
I obtained a good reaction with both currents at the point where the musculo-spiral 
nerve winds around the bone. Contractility, direct as well as indirect, was retained 
for both currents. 

This was true also below the point of pressure; the conditions above this point 
will be described after discussing the electric contractility in the patient with lead 
poisoning. 



MUSCULOSPIRAL (RADIAL) PARALYSIS 711 

Here it was soon apparent, both by direct and indirect irritation with the faradic 
current, that only those muscles could be stimulated which could still be actively 
moved, the paralyzed ones not responding at all. The indirect galvanic contractility 
of the paralyzed muscles had been lost, but, when directly stimulated by the galvanic 
current, they responded with a slow, sluggish contraction, especially marked to the 
anode, and soon showed the reaction of degeneration. 

In the case of lead paralysis the electric contractility of the supinators and the 
triceps had not suffered. 

I must now call attention to some very interesting points of this examination 
which are of differential-diagnostic significance. 

All paralyses due to pressure on the musculo-spiral nerve during sleep are not 
slight; they may be very serious, although more rarely than from other severe trau- 
mata. It is easily understood that the electrical conditions then resemble those in 
severe paralysis of other nerve regions. When, therefore, as in the majority of cases 
of pressure paralyses occurring during sleep, contractility is retained at the point on 
the upper arm where the nerve curves around the bone, this is due to the fact that 
pressure has been operative upon the nerve above this point. If, as in rare cases, the 
lesion is below this point, even with mild paralysis no reaction is obtained. In both 
of these patients I demonstrated this by faradically irritating Erb's point in the 
supraclavicular fossa ( this will subsequently be considered more minutely ) . In the 
patient with lead paralysis it then became evident that the supinator longus which 
had been exempt from paralysis, together with other muscles which need not now be 
mentioned, contracted powerfully while this did not occur in the patient with pressure 
paralysis. 

Diagnosis. — After the foregoing it can scarcely be difficult to diagnosticate 
musculo-spiral paralysis. Paralyses due to trauma, to pressure during sleep, 
and to injections of ether, may readily be recognized from the history; the 
occupation of the patient (a worker in lead in its broadest sense), the impli- 
cation of both radial areas, the results of electric exploration, the absence of 
paralysis in definite muscle groups, so characteristic of paralysis saturnina, 
will protect us from error. It must, however, be remembered that other 
metallic poisons such as arsenic and silver produce pathologic pictures which 
resemble lead poisoning, although more rarely; and in the given case there 
need be no difficulty in determining whether we are dealing with a musculo- 
spiral paralysis which is not isolated but is a part phenomenon of polyneuritis 
or of cerebral or spinal paralysis if we always make it a point to examine the 
patient systematically and thoroughly. 

The prognosis of radial paralysis depends upon the cause and the possi- 
bility of removing the deleterious factor. This is evidently most true of 
injuries to the nerve from trauma. 

Most paralyses from pressure during sleep prove to be mild, and to a cer- 
tain extent are self-limited. They are somewhat relieved by electro-therapy; 
the better the electric contractility the more favorable, as a rule, the prog- 
nosis, though to this there are, of course, exceptions. But lead palsy is among 
the most severe forms of paralysis, and yields only slowly to treatment. 

Musculo-spiral paralysis from pressure usually terminates in recovery 
without treatment, but the return of active motility may be ^decidedly has- 
tened by proper electro-therapy, and if only a week be gained in restoring 
to health one who maintains his family by manual labor, this is important 
and well worth striving for. According to Remak, the activity of the nerve 
may be stimulated by stabile treatment with a not too strong faradic current, 
the cathode being at the point of pressure, the other electrode being used 



712 PARALYSES OF THE PERIPHERAL NERVES 

to stroke the muscles in the paralyzed areas. I have several times treated 
in this way paralysis of the musculo-spiral nerve which had existed for sev- 
eral weeks, and recovery followed in a relatively short time. 

Unyielding paralyses (in an electro-diagnostic sense) as well as lead 
paralysis, require such galvanic treatment for a long time, often for months. 
If possible the patient with lead poisoning should be removed from the 
deleterious influence of the poison, the alcoholic should be warned to break 
up his evil habits, etc. 

If the paralysis is due to trauma, we must endeavor to remove by surgical 
means any obstruction caused by the fracture, dislocation, or compression of 
the nerve, etc., before beginning electrical treatment. If the nerve has been 
completely severed various methods, even nerve suture, must be resorted to. 

I should like to call attention here to two points: First, that if nerve 
suture has been performed late, electrical treatment should not be too early 
abandoned ; that is, the operation is not to be regarded as fruitless if motility 
is not restored as soon as was expected. Others, as well as I, have known 
active motility to return a year, and even more, after the patient was dis- 
charged from the hospital as incurable. Secondly, from my own experience 
and that of others, I think we must beware of performing a bloody operation 
too soon in musculo-spiral paralysis caused by a fracture of the humerus. 
Several of my patients for whom operation had been advised have recovered 
under a systematic and prolonged galvanic treatment. 

I must also mention that in addition to neurotomy, primary and secondary 
nerve suture, etc., surgeons have lately added transplantation of tendons to 
their operations in paralysis, and have thereby increased the possibilities of 
cure even in apparently desperate cases. For various reasons, I cannot allow 
myself to discuss this interesting theme; I will merely state that sometimes 
by uniting the tendons of a normal and strong muscle, even although it be 
an antagonist, with the elongated or more frequently shortened tendon of a 
paralyzed muscle, satisfactory functional activity has been restored after all 
other remedies had proven fruitless. [Nerve transplantation promises better 
results in such cases. — Ed.] 

In conclusion, I will add that a special prosthetic apparatus has been 
invented (Heussner, Goldscheider) to improve or compensate for deficient 
extension of the fingers, with what results I am unable to state. 

15. PARALYSIS OF THE ULNAR NERVE 

Etiology. — Although not as frequent as musculo-spiral paralysis, paralysis 
of the ulnar nerve is by no means rare. Pressure or compression of the nerve 
during sleep is not so frequently a cause as in radial paralysis. Because of 
the protected position of the ulnar nerve in comparison with other nerves 
some authors question the possibility of its paralysis by direct pressure. On 
the contrary, they attribute it to the pressure of the head of the humerus on 
the nerve in the axillary cavity during sleep with the arms extended upward 
and backward. If during anesthesia this position of the arm is long main- 
tained, anesthesia paralysis of the ulnar nerve may readily occur; this has 
been pointed out by Braun. 

Duchenne has called attention to occupation paralysis in the course of the 



PARALYSIS OF THE ULNAR NERVE 713 

ulnar nerve; namely, paralysis of the nerve occurring in persons whose occu- 
pations force them to use their elbows as a brace while working upon a hard 
under surface. Many authors, of whom I shall mention only Leudet, Ballet, 
Bruns, Menz, Gessler, Salomonson, have observed this in glass-workers, wood- 
carvers, telegraphers, and diamond-cutters, also in bakers, who knead the 
dough for bread with the ulnar side of the right hand and forearm, and thus 
constantly exert pressure upon a hard surface (Huet and Guillain). This 
has also been reported by the earlier authors (Coster and Remak, as well as 
by myself) who noted, particularly in laundresses, cigar makers, etc., that 
the small muscles of the hand became weak and atrophic. In these cases the 
etiologic factor of occupation neurosis was probably the direct pressure ex- 
erted upon the small muscles or upon the nerve. 

In a certain sense we may perhaps add to these the not infrequent paral- 
yses of the ulnar nerve on the left side which are seen in bicyclers, and are 
caused by exposure of the ulnar nerve at the wrist-joint to pressure between 
the handle of the bicycle and the pisiform bone; this has been described by 
Destot and myself. 

Another and less common cause of paralysis of the nerve of the elbow is 
compression from the use of an Esmarch bandage, from handcuffs, or from 
the pressure of a crutch in the axillary cavities. More rarely tumors seated 
at or near the nerve, or syphilitic affections, will produce this paralysis. 
Among the most common causes are injuries to the bones of the shoulder 
and the elbow-joint. Here I must refer to conditions long known but 
which have .only recently attracted special attention ; namely, ulnar paral- 
ysis which occurred years afer a preceding injury to the elbow, and also 
the so-called luxation of the ulnar nerve which may be either congenital or 
acquired, this nerve being normally protected (in its groove between the 
internal condyle and the olecranon) from the danger to which the elbow- 
joint, being without this natural protection, is exposed. 

This nerve is often injured by blows, stabs, or cuts at the wrist, more 
rarely by isolated injuries of the brachial plexus ; I once saw paralysis develop 
after a subcutaneous injection of ether clumsily given in the area of this nerve 
on the inner side of the upper arm. 

Inflammatory degeneration in the region of the ulnar nerve is not rare 
after infectious diseases, especially after enteric fever; at the same time I 
must state that the peripheral paralyses occurring during the puerperium 
frequently implicate the nerves of the hand, usually the ulnar nerve. When 
I state that ulnar paralysis is observed in leprosy, and that the small muscles 
of the hand are implicated in many cases of lead poisoning, and, as I have 
seen, even without a primary radial paralysis, all of the important factors 
which may lead to ulnar paralysis have been enumerated, and it need only 
be added that paralysis in the course of this nerve, especially in the small 
muscles of the hand, is also met with in tabes, in syringomyelia, and very 
often in progressive muscular atrophy. 

Symptomatology. — In complete paralysis of the ulnar nerve the use of 
the hand for the execution of finer movements, as in writing, etc., is mark- 
edly impaired. Flexion of the wrist and the last three fingers is incomplete. 
The little ringer is motionless. The interossei muscle, the separators of the 
ringers, the muscles which draw them toward each other, and the adductor 



714 PARALYSES OF THE PERIPHERAL NERVES 

pollicis are innervated by the ulnar nerve; since the proximal phalanges of 
the fingers are flexed by the interossei, the middle and terminal phalanges, 
however, being extended, in injury of the nerve above the wrist- joint (a fre- 
quent occurrence), and with a normal condition of the upper portion of the 
ulnar which supplies the branches for the long flexors of the finger, the char- 
acteristic claw or talon hand (main en griff e) develops, a peculiar position 
of the affected hand and fingers with which we are familiar from Duchenne's 
description. By paralysis of the interossei which flex the basal phalanx and 
extend the middle and terminal phalanges, and with intact flexors of the 
middle and terminal phalanges, as well as the extensors of the basal finger- 
joints (the extensor communis digitorum which is innervated by the radial 
nerve) this resemblance of the hand to the claw of a bird is particularly 
marked in extreme atrophy of the interossei muscles. There is decided dis- 
turbance of sensation in the hollow of the hand, most marked in the little 
finger, especially in the ball of it, as well as in those parts of the third finger 
and the palm of the hand which are situated on the ulnar side of an imag- 
inary line drawn through the middle of the ring finger. 

On the dorsum of the hand the entire little finger is implicated, the 
ulnar side of the third, the proximal phalanges of the second and third 
upon the ulnar side, and finally the region of the fourth and fifth metacarpal 
bones. 

Among trophic disturbances I have already mentioned atrophy of the 
interossei and hypothenar muscles which is often extreme and gives a sunken 
appearance to the metacarpal spaces. With or without the formation of vesi- 
cles ulceration appears in the cutaneous areas more or less deprived of sensa- 
tion, particularly by the action of a low temperature, and in a few cases con- 
tracture in the palmar fascia (Dupuytren's contracture) has been regarded 
as a trophic disturbance. In a few cases, hyperplasia of the subcutaneous cel- 
lular tissue of the palm of the hand has been noted (Lowenfeld), and has 
been regarded as a trophic disturbance in the course of the damaged ulnar 
nerve. 

In ulnar paralysis elective contractility reveals nothing special; according 
to the severity of the lesion we are dealing with a slight, a moderate, or a 
severe form of peripheral paralysis. 

As to prognosis, little can be stated except that the more severe the trauma 
which has affected the nerve the more difficult the cure. If the nerve is com- 
pressed, and it is possible to relieve this by a surgical operation and restore 
its normal function, the prognosis is favorable; the outlook is least promis- 
ing when we are dealing not with a peripheral lesion but with the implication 
of the nerve in a pathologic central process, which is the case in progressive 
muscular atrophy and in sj^ringomyelia. 

Considering the deformity which it produces the diagnosis of paralysis of 
the ulnar nerve occasions no perplexity. If there are signs which point to 
a possible trauma (fracture of bones, dislocations, wounds, etc.), the cause 
of the affection is readily recognized, and appropriate treatment should be 
instituted. If these conditions are not present, a carefully elicited history 
may enable us to determine in the given case whether any infectious disease 
has preceded or whether we are dealing with an occupation paralysis, and a 
thorough examination of the patient will perhaps reveal that the paralysis 



PARALYSIS OF THE MEDIAN NERVE 715 

is but the symptom of a grave derangement of the nervous system, for exam- 
ple, syringomyelia or progressive muscular atrophy. 

In regard to treatment, paralyses due to the fracture of bones, disloca- 
tion, etc., indicate surgical measures by which the damaged nerve may be 
released from the pressure of any bone, foreign body or cicatrix; later, even 
when the nerve has been severed by trauma and reunited by suture, electricity 
may be employed, and this treatment is also applicable in those cases in which 
the paralysis may be attributed to a neuritic affection, no matter what the 
antecedent cause. 



16. PARALYSIS OF THE MEDIAN NERVE 

Before discussing the pathology of paralysis of the median nerve, which 
is less frequent than radial paralysis yet by no means rare, I desire to give 
the history of two patients. 

Case 1. — A girl, who had fallen upon glass a few weeks prior to her appearance 
in the Clinic, received a wound two or three fingerbreadths above the right wrist- 
joint and several centimeters in length. This severed the median nerve; the ulnar 
nerve was uninjured. When the hand was examined it was remarked that the index 
and middle fingers were smooth, glistening, and cool to the touch; in contrast with 
the other fingers they never perspired. This smooth, glistening appearance is an illus- 
tration of what is known as " glossy skin." The cicatrix and the areas above it were 
insensitive to pressure; but pressure upon the nerve below the cicatrix caused a painful 
sensation in the palm of the hand at the base of the first and second fingers. The 
sensory disturbances were then investigated. Test with a needle showed profound 
anesthesia on the volar side of the middle and distal phalanges of the first and second 
fingers and upon the radial side of the third finger as well as in the distal phalanx 
of the thumb; marked hyperesthesia was found in the basal phalanges of the thumb 
and first two fingers and on the radial side of the third, and over the ball of the 
thumb and the adjacent areas of the palm of the hand. At the cushion of the middle 
finger a cicatrix from a burn was noted; and this injury had when received not been 
felt by the patient, but was only perceived later. 

The disturbances of sensation upon the back of the hand are extremely interesting, 
and still not sufficiently understood. Absolute anesthesia was found over the entire 
distal and middle phalanges of the first and second fingers, and on the radial side of 
the third; the sensibility of the skin over the basal phalanges on the dorsal side of 
these fingers was but little altered; the thumb and back of the hand were in this 
respect absolutely normal. 

It was surprising to find that but little disturbance of motility could be deter- 
mined. The first, second and third fingers as well as the distal phalanx of the thumb 
could be flexed and the hand clenched into a fist. Careful investigation revealed a 
slight flattening of the ball of the thumb in the region of the abductor pollicis brevis; 
there was no marked atrophy of the muscles of the ball of the thumb. 

All movements of the thumb were free; it could be brought into apposition with 
all the fingers without flexing the distal phalanx. Articles could be held between the 
distal phalanges of the thumb and index finger, although not with as firm a grasp as 
upon the normal left side. 

The results of the electrical examination formed an apparently remarkable contrast 
to the generally well-retained motility. Unlike the conditions on the normal left side, 
no effect could be produced upon the affected right side even with the strongest faradic 
currents, neither with direct nor with indirect irritation; this was true also of indirect 
galvanic irritation. The musculature of the ball of the right thumb, however, reacted 
slowly and sluggishly to direct irritation with a current which upon the normal side 
had no effect whatsoever, the anodal closure contractions being equal to the cathodal, 
contrary to the normal, 



716 PARALYSES OF THE PERIPHERAL NERVES 

Case 2. — I will now relate the case of a patient who had been married six years, 
had borne three children, and, according to report, during every labor had complained 
of tearing pain in the shoulder. After the last labor, during which the patient suf- 
fered from pain in the right shoulder, she noticed a gradually increasing emaciation 
of the right hand. This affected the interosseous spaces (especially the first) and the 
ball of the thumb; along the entire ulnar border of the right lower arm and in the 
little finger there was numbness. The power to spread the fingers and draw them 
together again was decidedly limited, as well as movements of the thumb. Electric 
examination showed a moderate reaction of degeneration in the area of both nerves. 

The history of these two patients discloses the etiological factors which 
most frequently produce paralysis of the median nerve — trauma and degen- 
erative neuritis. In my experience, incised wounds of the nerve immediately 
above the wrist-joint, most often by a fall upon glass, etc., are the common 
causes of paralysis either of the median nerve alone or combined with the 
ulnar nerve. Paralysis also occurs after gunshot wounds, stabs, or operations 
on the upper arm, and somewhat more frequently in consequence of fractures 
and dislocations. 

While tumors of the nerve have several times been described as causes of 
paralysis of the median, paralysis of the nerve from the pressure of a crutch, 
pressure during sleep, or after anesthesia is a rare occurrence. Paralysis of 
the median nerve after dislocation of the wrist- joint (Growers) has been 
only occasionally reported; E. Weber published the case of a young man in 
whom the sensory branches of the median nerve were affected while dancing 
because of a marked hyperflexion and pronation of the hand and lower arm. 
As with other nerves of the arm, the pressure of an Esmarch bandage or any 
other compression may paralyze this nerve either alone or combined with 
other nerves of the arm; for instance, when handcuffs are applied just above 
the wrist-joint. 

The second case belongs to the form not infrequently seen after infectious 
diseases; alone or in association with the ulnar nerve the median nerve may 
be affected during the puerperium, as was first stated by Kast and subsequently 
emphasized by Mobius and other authors ; such cases have been observed after 
variola and enteric fever, and lately by Eulenburg and myself after in- 
fluenza. 

A degenerative neuritis is probably the cause or consequence of over- 
exertion in an occupation; the so-called professional or occupation paralysis 
occurs in laundresses, carpet beaters, carpenters, locksmiths, cigar and cigar- 
ette makers, perhaps in dentists (in filling teeth) and in those persons who 
milk many cows (milker's paralysis). 

I must also call attention to the fact that paralysis of the median nerve 
may be an accompanying symptom of a widely distributed neuritis in paralysis 
due to lead, to arsenic, or occasionally to phosphorus and carbon bisulphid. 

I omitted to mention among the so-called occupation paralyses a few cases 
of paralysis of the thumb in drummers. Bruns first called attention to this 
isolated affection of the flexor pollicis longus. Later investigations (v. Zan- 
ders, Diims, Steidel) have proven that in such cases the extensor pollicis 
longus is just as frequently implicated, and that the condition is often not 
one of nerve or muscle paralysis but is due to a laceration of the tendons of 
these muscles. 

In conclusion I must state that atrophy and paresis of the small muscles 



PARALYSIS OF THE MEDIAN NERVE 717 

of the hand, therefore also in the distribution of the median nerve, are ob- 
served in spinal progressive muscular atrophy and in syringomyelia. 

The symptomatology of paralysis of the median nerve varies according to 
the seat of the lesion. If, as is often the case, the nerve is injured above 
the wrist-joint, and provided the tendons of the long flexors of the fingers 
are not also injured, the muscles of the ball of the thumb only are paralyzed. 
Flexion and apposition of the thumb to the fingers becomes impossible. The 
extended thumb, adducted from the fingers and with its normal position of 
apposition lost, shows its volar side anteriorly. The hand loses the charac- 
teristics of the human hand, becomes flat, and resembles the claw or paw of 
the lower animals, for instance the monkey. 

When the injury to the median nerve is higher up, the conditions differ. 
It is well known that, besides supplying both pronators, this nerve also inner- 
vates the palmaris longus, flexor digitorum sublimis, the radial portions of 
the profundus, the flexor pollicis longus, the three first lumbricales, and, 
except the abductor pollicis which is innervated by the ulnar nerve, it supplies 
all the other small muscles of the ball of the thumb. 

It is evident, therefore, that if the lesion is high, pronation of the hand 
is imperfect or impossible, flexion of the hand also is incomplete, and when 
attempted the hand is drawn medially by the flexor carpi ulnaris which acts 
alone. As the flexor digitorum sublimus flexes the middle phalanges of all 
the fingers, the digital phalanges of the second and index fingers, and the 
flexor digitorum profundus which is also innervated by this nerve, these 
movements are impossible if the lesion is high. Of the altered movements of 
the first three lumbricales after injuries to the median nerve we have little 
clinical knowledge. Flexion of the basal phalanges of the four fingers (not 
the thumb) is produced by the activity of the interossei. Flexion of the distal 
phalanges of the last two fingers depends upon the flexor digitorum pro- 
fundus, which here receives its innervation through the ulnar nerve. If we 
add to these disturbances the lost power of apposition in the thumb, further 
details of the loss of power in the hand and fingers from paralysis of the 
median nerve are unnecessary. 

Disturbances in sensation following lesions of the median nerve were re- 
ferred to in the history of the first patient; it was also emphasized that the 
dorsum of the hand, the nail, the middle phalanx of the index and second 
fingers, and the radial side of the same members and of the third finger 
receive sensory fibers from the median nerve. In some persons the dorsal 
side of the distal phalanx of the thumb appears to receive its sensory fibers 
from the median nerve. The distribution of the sensory fibers is more easily 
traced in the palm of the hand : The most lateral parts of the ball of the 
thumb receive their sensory innervation from branches of the superficial 
radial and musculo-cutaneous nerves ; the bulk of the skin covering the thenar 
eminence and the vola manus is innervated by sensory fibers from the 
median, as well as the volar side of the thumb, of the index and second 
fingers, and the radial side of the third finger. 

These fibers are not implicated to the same extent in all cases of injury 
to the median nerve, for the lesion may be slight; the disturbance of sensa- 
tion just described is more or less extensive according to the severity of the 
injury. 



718 PARALYSES OF THE PERIPHERAL NERVES 

Among the trophic and vasomotor disturbances which occur in almost all 
severe peripheral paralyses we here find a flattening of the ball of the thumb, 
and in lesions higher up a flattening of the radial side of the forearm. Hand 
and fingers are cool to the touch and often show a livid discoloration; the 
skin is readily injured and reacts even to slight injuries by the formation of 
vesicles and ulcers. As was seen in one of my patients the skin of the finger 
is smooth, glistening and insensitive; upon it herpetic and pemphigus-like 
vesicles readily appear. Marked trophic disturbances in the nails are not 
uncommon; these become irregular, curved or fissured, but, so far as I have 
been able to observe, their growth is but little affected. 

The changes in electric contractility vary according to the severity of the 
injury; they may be slight, moderate or extensive. To avoid repetition, I 
shall not enumerate these conditions, but I must call attention to one peculiar- 
ity observed very frequently in paralysis of the median nerve, and which was 
clearly demonstrated in the case of the first patient. 

While in peripheral paralysis with a severe implication of the motor nerve 
fibers the sensory fibers are but slightly or not at all affected, I have observed 
(and I note from literature that other authors also have noted) that in 
median paralysis these relations are often reversed. Contrary to the usual 
condition very decided sensory disturbances may appear after an apparently 
slight implication of active motility; this was evident from the history of the 
first patient. This remarkable condition may be explained by the anastomoses 
(which have been understood for some time but have recently been more 
clearly demonstrated by Frohse) which take place between the median and 
the deep branches of the ulnar in the hand. At all events we may assume 
that this anastomosis does not take place in all persons, at least not to the 
same extent, so that in the majority of cases median paralysis runs its course 
according to the ordinary diagram of peripheral paralysis. 

After the foregoing it can hardly be difficult to diagnosticate median 
paralysis, especially if in the examination of the patient we elicit a history 
of trauma. It need only be premised that paralysis of the muscles supplied 
by the median nerve may be an accompanying condition of cerebral, and even 
more frequently of spinal, disease; I need merely refer to syringomyelia, to 
progressive spinal muscular atrophy, and to similar processes. The prognosis 
depends upon the severity of the lesion. Compression is much more favorable 
than complete severance. Electro-diagnostic examination will clear the prog- 
nosis. 

The treatment should include the use of electricity, but it must be remem- 
bered that, as in all injuries produced by complete severance of the nerve, 
this should be employed only after the divided nerve-ends have been united 
by suture. 

17. COMBINED PARALYSES OF THE NERVES OF THE 
SHOULDER AND ARM 

Having studied paralysis of the individual nerves of the arm, we must 
still discuss those paralyses which attack several nerves of the upper extrem- 
ity simultaneously. These combined paralyses of the nerves of the shoulder 
and arm develop from different causes. Usually they are due to injuries 



COMBINED PARALYSES OF THE NERVES OF SHOULDER AND ARM 719 

of the bones or joints which produce a lesion of the nerve: Fracture of the 
head of the humerus, of the shaft of the bone, of the clavicle, or dislocation 
of the head of the humerus. Xext we consider injuries made by abnormal 
pressure in the axillary cavity (pressure of the crutch) or by a too great 
constriction of the arm during operations (with Esmarch's bandage), or by 
constriction from ropes around the upper arm. Paralysis which occurs after 
prolonged anesthesia is specially interesting. Later we shall more minutely 
discuss the forms of paralysis designated by Braun anesthesia paralysis; we 
may here state that such paralysis need by no means implicate all the nerves 
of the upper extremity, but that besides complete paralysis there may be every 
possible degree according to the injury which the nerves have sustained. 

An excellent example of combined paralysis of the shoulder and arm occurred in 
the case of a man, aged 28, who sustained a fall, his right shoulder striking the ground. 
He at once observed that he could not move his arm. It was rotated inwardly, could 
be extended at the elbow-joint, but could not be elevated to the frontal plane and only 
a few degrees in the sagittal plane; outward rotation of the arm was impossible. 
With the lower arm supine the patient could not flex it toward the upper arm ; actual 
supination of the lower arm was impossible. The right shoulder appeared flattened, 
as well as the supra- and infrascapular fossae. On the anterior and outer side of the 
deltoid muscle there was an area 4 to 5 centimeters long and 2 centimeters in breadth 
which, like the posterior surface of the thumb and the extensor surface of the lower 
arm along the radius, was somewhat less sensitive to touch than the corresponding 
region of the left lower arm. 

The electric contractility showed a moderate reaction of degeneration, for mus- 
cular contraction occurred quite readily as compared with the left side on direct and 
indirect faradic irritation with but a slightly increased current ; muscular contraction 
was also sharp and lightning-like with indirect galvanic irritation, while in the biceps 
and the posterior and external bundle of the deltoid, with direct galvanic irritation 
the contractions were sluggish and the CaOC which is otherwise difficult to demon- 
strate could be readily produced. Before entering upon the pathology of the form 
of paralysis, of which this patient furnished an almost classic example, I must call 
attention to a symptom which in this case was readily demonstrated. I performed the 
following little experiment: I passively flexed the lower arm of the patient upon 
the upper arm, and asked him to keep the arm flexed while I attempted to extend it; 
it was evident that I could easily do this; not only did the true flexors of the lower 
arm, the biceps and the brachialis internus, fail to contract, but during this process 
the supinator longus on the right side remained flaccid while in the left normal arm 
it contracted distinctly and energetically. Was it not remarkable then that the patient 
could flex his arm? It was noted that he slightly pronated the lower arm while 
extending the hand upward ; if the flexor and ulnar side of the right lower arm was 
grasped with the fingers a strong contraction of the muscles from the internal condyle, 
especially of the pronator teres, the flexor carpi radialis and the ulnaris, was dis- 
tinctly felt; and the tendon of the palmaris longus became very tense. If pressure 
was made with the fingers alongside the quite flaccid supinator longus, this was fol- 
lowed by contraction of the extensor carpi radialis. It was observed, too, that it was 
unnecessary to flex the hand dorsally in order to flex the loAver arm, for this could 
easily be done with the hand completely flexed. 

Such vicarious functions of the flexors of the hand and fingers (more 
rarely of the extensors) are not always so evident in paralysis of the flexors 
of the lower arm, hence I took occasion to mention this case which I had 
observed years ago. To complete the history of this patient it is only neces- 
sary to add that the oculo-pupillary fibers (of the sympathetic) were intact 
as well as the regions supplied by the median and ulnar nerves; also that 
with the exception of the supinators the radial region was normal, as was also 



720 PARALYSES OF THE PERIPHERAL NERVES 

evident from the ease and promptness with which movements of these areas 
were executed. 

This form of paralysis, although known before Erb described it, was, 
nevertheless, only then fully appreciated. In these paralyses the deltoid, the 
biceps, the brachialis internus, the supinator longus (sometimes also the 
supinator brevis), also, although not in all cases, the supra- and infra- 
spinatus are simultaneously paralyzed while other nerve regions are usually 
exempt. 

The question arises, Where must the damaging factor fall to produce 
such a plexus paralysis'? Naturally, at that point in the plexus where the 
motor tracts for the involved muscles have not yet divided into the different 
nerve trunks. Erb showed that the fifth and sixth cervical nerves in par- 
ticular assist in the formation of the branches of the brachial plexus here 
under consideration, and he indicated the supraclavicular fossa as the point 
in which, under normal conditions, the paralyzed muscles may be induced 
to contract (Erb's point). 

According to Hodemaker this point is found by drawing a line from the 
sterno-clavicular joint to the spinous process of the seventh cervical vertebra, 
somewhat anteriorly to and 1.5 cm. from the border of the trapezius muscle. 

Some points in the etiology have already been mentioned: In addition to 
trauma of the supraclavicular fossa, a fall upon the shoulder, operative 
wounds in this region, blows, gunshot wounds, or stab wounds, this form of 
paralysis may be caused by tumors or neuritic processes in this area as in the 
case of tumors of the cervical vertebrae. 

Following Duchenne, Erb and still later many other observers called atten- 
tion to the paralyses which not infrequently occur in the new-born, paralyses 
which, according to Duchenne, are designated as " paralysies obstetricales 
infantiles due membre superieur sans complication " ; our knowledge of these 
forms of paralysis has been added to and elucidated by the investigations of 
more recent authors. The following is the history of such a case: 

A child, aged 5 weeks, delivered without instruments. The labor was tedious 
owing to the non-appearance of the shoulders, and paralysis was noted immediately 
after birth and still existed at the time the infant was examined. It was evident that 
the left upper extremity rotated internally (from paralysis of the supra- and infra- 
spinatus ) , being extended upon the thorax. In contrast with the activity of the right 
upper extremity, the left as far as the arm and forearm were concerned was immotile, 
only the hand and fingers moving. Testing the electric contractility in such small 
children is, as is well known, extremely difficult, but in this case it was enormously 
diminished, especially in the deltoid, the flexors of the lower arm, and the supinator 
longus. 

It is exceedingly interesting to review the various theories which have 
been proposed to explain this Duchenne-Erb paralysis of the plexus of the 
arm. The etiology of these paralyses in the adult is easy to understand if 
trauma has affected the supraclavicular region. But concerning those which 
follow a blow upon the shoulder, the opinions of authors are wide asunder. 
While some maintain that the clavicle is raised and approximates the verte- 
bral column in consequence of the forcible adduction of the shoulder which 
so presses upon the cervical vertebral column as to affect the middle portion of 
the transverse processes of the sixth and seventh cervical vertebrae an<J to 



COMBINED PARALYSES OF THE NERVES OF SHOULDER AND ARM 721 

injure the nerves located there, others reject this explanation, and believe that 
the injury is done between the clavicle and the first rib, while still others 
take a middle ground and admit both possibilities. 

What I have previously stated is particularly true of the traumatic paral- 
yses of adults during anesthesia. 

In regard to such paralysis in the new-born, Erb first called attention 
to the fact that in Prague, according to custom, the fingers of the obstetrician 
encircle the neck of the child and pull with such force that this region of , 
the supraclavicular fossa is subjected to great pressure. At other times the 
fingers of the obstetrician hooked in the axillary cavity of the child may pro- 
duce compression, or the condition may be due to torsion and pressure when in 
a breech presentation the arms of the child are forcibly pulled. Recent inves- 
tigations made upon the cadavers of children, indicate that it is not so much 
the direct pressure as torsion of the fifth and sixth nerve roots which causes 
this paralysis, and also ascribe to this etiologic factor so-called anesthesia 
paralysis. 

The majority of paralyses occurring intra partum are of the Duchenne-Erb type; 
the internal rotation of the arm is invariably attributed to paralysis of the outward 
rotators, particularly of the infraspinatus. 

Aside from the immediate damage to the nerves by injuries to the bone or joint, 
we occasionally find paralyses which implicate the median and ulnar nerves yet differ 
from those ordinarily observed. 

There may be profound paralysis which implicates the oculo-pupillary fibers. It 
is characteristic, however, that in a case of paralysis of the new-born recently described 
by Jolly in which there was a bilateral paralysis of the pectoralis major, of the latis- 
simus dorsi, and the triceps, and in which nearly all of the muscles of the forearm 
and of the hands were implicated, the function of both deltoids and biceps was retained. 
Both arms were raised, the forearms strongly flexed ; the hands drooped and. as Jolly 
expresses it, were in a pump-handle position. In this case torsion had occurred between 
the sixth and seventh cervical vertebras and had damaged the point of exit of the sixth 
and seventh pairs of roots. 

Before passing to another equally important and interesting form of 
plexus paralysis, I must call attention to the fact that during labor, besides 
injury to the nerve, other lesions may be sustained which occasionally result in 
the paralysis of one or several nerves in the arm of the child. I refer to 
injuries of the bones and joints such as are not infrequent in difficult deliv- 
eries. Paralyses of this kind must receive the same consideration as trau- 
matic paralyses of adults, for they are due to similar causes. 

We will now discuss those paralyses which affect in a t/ypical manner only 
one or another of the muscles ordinarily paralyzed in common with other 
muscles. It has been observed that sometimes, in trauma or neuritic affections 
of the plexus, not all but merely a few of the muscles implicated are paralyzed, 
or even only one, yet minute investigation has shown the point of origin of 
this apparently isolated paralysis to be in the plexus. I mention this solely 
for the sake of completeness, since such peculiar conditions as this are rarely 
met with. 

The bilateral paralyses of the Duclienne-Erb type need more minute inves- 
tigation. These occur bilaterally in anesthesia if the arms are drawn upward 
and back for a considerable time during prolonged operations. Cases of this 
kind have been reported by Braun, Jolly, Mallv and others. 
47 



722 PARALYSES OF THE PERIPHERAL NERVES 

A number of neuritic plexus paralyses have become known in the course 
of years; here infectious diseases such as pneumonia and influenza, or other 
affections such as anemia, perityphlitis, tuberculosis, etc., play a causative 
role. Bilateral plexus paralysis has also been observed in persons whose occu- 
pations forced them to carry heavy loads on the shoulders (coal, stones), 
the supraclavicular fossa thus being subjected to heavy pressure for weeks 
or even years. 

For the sake of completeness I must mention that such paralysis is now 
and then produced by excessive use of the muscles of the upper extremities, 
also that torsion of the arm or gymnastic exercises which abnormally stretch 
the arms may be the cause. These paralyses have also been observed after 
hemorrhage in the supraclavicular fossa (in alcoholics) and in isolated cases 
from gout. A case reported by Raymond is unique from the fact that there was 
merely an affection of the sensory plexus roots without implication of the motor 
ones; this rare condition was probably due to a tuberculous pachymeningitis. 

So far we have considered only plexus paralysis of the upper roots. There 
are also forms which have long been known but have only lately been explained 
by the studies of Dejerine-Klumpke who considered these to be paralysis of 
the lower roots of the plexus. In Klumpke's paralysis the regions of the 
median and ulnar nerves are chiefly implicated; these show paralysis and 
atrophy of the musculature of the ball of the thumb and the little fingers, 
also of the interossei; accompanying this are sensory, especially oculo-pupil- 
lary, disturbances, manifested by the narrowing of the palpebral fissure of the 
affected eye, the retraction of the eyeball, and the contraction of the corre- 
sponding pupil (myosis). These signs of disturbance in the function of the 
sympathetic indicate an injury to the communicating branch of the first 
dorsal nerve, as Klumpke proved by experiments upon animals, and Oppen- 
heim in man by an extremely interesting case. 

Etiologic factors for this paralysis may be wounds in the affected region, 
but it is usually caused by tumors which, starting from the lungs or from the 
vertebrae, damage the lower cervical and upper dorsal vertebrae and the nerve 
roots between them. 

Upper or lower plexus paralysis of this kind may not always exhibit from 
the onset a characteristic type. A paralysis which immediately affects all the 
fibers of the plexus may yield in the course of weeks or months so that all 
that remains of the once complete paralysis is the affection of the upper or 
lower plexus. This is true not only of the traumatic cases, which are at once 
recognized, but also of those which occur intra partum ; we know to-day from 
a series of observations, Heubner's, for instance, that plexus paralysis of 
Klumpke's type is seen in children and is due to caries of the vertebrae or 
tumors of the vertebrae. 

There are, of course, exceptional eases which deviate from the types here 
described. Plexus paralyses implicate the median and ulnar regions without 
paralytic phenomena on the part of the sympathetic; cases have been seen 
in which the symptoms of Erb's paralysis were combined with those of sym- 
pathetic involvement; these, however, may be due to the fact that the tumor 
which compresses the upper roots presses also upon a branch of the sympathetic 
in the neck ; and here and there are other deviations which, in the given case, 
must be especially investigated. 



COMBINED PARALYSES OF THE NERVES OF SHOULDER AND ARM 723 

I shall not touch upon the disturbances of sensation, nor the vasomotor 
and trophic symptoms in paralysis of this kind, nor shall I describe the elec- 
trical reactions which vary according to the severity of the individual case, 
since these do not differ from those ordinarily observed in isolated paralysis 
of this nature. 

The diagnosis of these cases usually occasions no perplexity if there is a 
demonstrable trauma, if close inspection reveals a tumor in the upper or 
lower part of the supraclavicular fossa, or if there are caries of the upper or 
lower cervical vertebrae. If infectious disease has preceded the appearance 
of plexus paralysis, or there has been severe pain, neuritis must be considered. 
We can easily decide from the history whether in the given case the paralysis 
was obstetrical. 

We must be particularly cautious in the diagnosis of paralyses of this 
plexus which occur early in life. I have previously referred to those pseudo- 
paralyses which follow injuries to the bones or joints in instrumental deliv- 
eries and which have no direct association with a damage to the nerve. I 
must also refer to the epiphyseal loosening which is observed in children 
with congenital syphilis and which may resemble paralysis (pseudo-paralysis, 
Parrot). 

Further de Peters has called attention to a form of paralysis which he 
regards as a spinal cord affection; this occurs in children with congenital 
syphilis and, particularly if the radial region is involved, gives a peculiar 
appearance to the hand and fingers. The wrist- joint and the pronated fore- 
arm are so flexed and abducted that a singular position results which resem- 
bles the paddles of a seal, and was called by de Peters the " paddle position." 

Moreover, we observe in children affections of the spinal cord which are 
more or less familiar to us as spinal infantile paralysis. 

This acute poliomyelitis of children may closely resemble the Erb-Duchenne 
paralysis, the same muscles are paralyzed, in the same combination, and with 
the same electric reactions. In comparing these with paralyses occurring intra 
partum we have as differential factors the history, the more advanced age of 
the child, preceding infectious diseases or a febrile condition, and the absence 
of marked disturbance of sensation. 

I must admit that Remak has recently described an amyotrophic plexus 
neuritis in children which may very readily be confounded with poliomyelitis ; 
its differential diagnosis from circumscribed poliomyelitis may under some 
circumstances be exceedingly difficult, and perhaps only by its more favorable 
outcome can amyotrophic plexus neuritis be distinguished. 

In adults we may find an acute, subacute, or chronic spinal paralysis 
which is described in literature as acute poliomyelitis or chronic poliomyelitis 
of adults; if, in the given case, we suspect lead poisoning, we must ascertain 
whether any of the symptoms can be referred to this etiologic factor. 

Finally, we may observe paralyses resembling those described in persons 
predisposed to nervous derangements after trauma. These local liystero-trau- 
matic paralyses may be differentiated from those due to a peripheral nerve 
injury by the peculiarly distributed disturbances in sensation almost invaria- 
bly present, by the retention of electric contractility, and by the other symp- 
toms of hysteria. 

The prognosis of plexus paralysis depends upon the underlying affection. 



724 PARALYSES OF THE PERIPHERAL NERVES 

Tumors of the vertebrae or carious processes in the cervical vertebrae which 
produce plexus paralysis are more serious than slight injuries of the plexus 
itself. In electric contractility we have a test which rarely fails to reveal 
the severity of the attack and its probable duration. Cases of obstetrical 
paralysis usually improve with time, nevertheless recovery is incomplete; in 
any case it is wise, in a prophylactic sense, to avoid during labor all unneces- 
sary pulling or tension upon the limbs and the vertebral column of the child. 
During prolonged anesthesia, too, we should not hold the arms of the patient 
too firmly nor abduct them too strongly. It need hardly be emphasized that 
in gymnastic exercises and in certain occupations there should be intervals 
of rest to prevent abnormal tension, torsion and strain. If operation is indi- 
cated this should first be performed before we proceed to treat the paralysis 
by means of baths, massage, exercises and electrotherapy. 

Parrot's pseudo-paralyses, as well as de Peters paralyses due to syphilis, 
should be treated with antisyphilitic remedies which are usually very bene- 
ficial. 

18. PARALYSIS OF THE MUSCLES OF THE NECK, BACK 

AND ABDOMEN 

Before describing paralysis of the lower extremity, we must briefly con- 
sider those pathological conditions due to paralysis of the muscles of the neck, 
back and abdomen, which rarely occur singly but are partial phenomena of 
other pathologic processes. 

Paralysis of the muscles of the neck is noted in progressive muscular atro- 
phy, in infantile spinal paralysis, in asthenic bulbar paralysis, in syringo- 
myelia, in polyneuritic processes, and in caries of the vertebrae of the neck. 
The head droops upon the chest, and can be raised only by the aid of the 
hand. In the muscles of the neck, which are usually atrophic, electric con- 
tractility is either markedly decreased or is lacking. 

Isolated paralysis limited to the deep anterior muscles of the neck has 
been observed only once, in a case of hysteria, the patient's head being bent 
backward, and he was unable to bring it forward without the aid of his 
fingers introduced within the mouth. 

Paralysis of the auxiliary muscles of respiration may follow lesions in the 
upper part of the spinal cord ; this has been seen also in hemiplegic conditions. 

If the muscles of the back, especially the extensors of the vertebral column, 
are paralyzed, it is impossible to raise the trunk, that is, to extend it. To 
maintain the equilibrium of the body the upper part must be bent far back, 
the lumbar vertebral region showing lordosis. The center of gravity for the 
body is behind t'he promontory of the sacrum. If the patient bends too far 
forward he falls. The actions of these patients on rising from a recumbent 
posture are especially characteristic : They place both hands upon the knee and 
slowly draw them up the thighs, and, as it were, climb up upon themselves; 
then with the help of their arms and shoulders they raise the trunk. In sit- 
ting, the weak extensors of the back no longer hold upright the lumbar verte- 
bral column which is bent backward, and shows kyphosis. In walking the 
trunk waddles, for the weak extensors of the back no longer hold it firm. 
When the patient is in the recumbent posture the kyphotic curvature of 



PARALYSIS OF THE MUSCLES OF THE NECK, BACK AND ABDOMEN 725 

the vertebral column no longer appears. It is easy, therefore, to see that 
unilateral paralysis of the muscles of the back causes lateral displacement 
of the vertebral column (scoliosis). 

This description is especially applicable to a disease described by Du- 
chenne, pseudohypertrophy of the muscles; of course, the symptom-complex 
will be identical even if other pathologic processes have caused the same 
weakness of the muscles of the back. The condition is found in infantile 
spinal paralysis, after enteric fever or diphtheria, in severe lesions of the 
spinal cord, also when the back has been injured or is diseased by trauma or 
rheumatic processes. It is well known that paresis of the muscles of the back 
occurs in weak anemic children, even without a conspicuous nervous affection, 
and gives rise to curvatures of the vertebral column (scoliosis, kyphosis). 

Paralysis of the muscles of the abdomen may generally be referred to the 
same etiologic factors as the forms of paralysis just described. In the course 
of enteric fever the abdominal muscles are often severely damaged. x\s 
the vertebral column and the trunk are bent forward by the muscles of the 
abdomen (also the ilio-psoas muscle), when there is loss of function in these 
muscles the patients readily fall backward on slight extension of the trunk. 

According to Duchenne, such patients markedly flex the pelvis anteriorly 
so as to support the trunk by the extensors of the vertebral column which 
have remained normal. With the consequent curvature of the vertebral 
column, the point of equilibrium falls in front of the promontory of the 
sacrum. 

If the abdominal muscles are paretic or paralyzed, expiration and all the 
functions dependent upon it (coughing, sneezing, expectoration, etc.) suffer, 
above all intra-abdominal pressure (difficulty in emptying the bladder and 
the rectum). 

The abdomen is distended; the patients cannot rise from a recumbent 
posture without aid. 

To complete the symptomatology I must state that upon tapping the sur- 
face of the abdomen various reflexes (Rosenbach's, also Geigel-Dinkler-Bech- 
terew's reflex) are absent; in the extremely uncommon unilateral paralyses 
with forced expiration the paralyzed half of the abdomen is drawn toward the 
sound side. 

A few researches into the possible change in the electric contractility of 
the paralyzed muscles have revealed the same changes which occur in diseases 
of other muscle regions. 

The points upon which to base the diagnosis of these conditions were 
enumerated in the description of the symptoms. 

When we remember that these paralyses rarely occur singly, a most care- 
ful examination of the patient's entire condition is necessary in order to deter- 
mine the relation of the symptoms which I have described to an affection of 
the spinal cord, of the muscles, etc. 

That diseases of the spinal cord, progressive muscular atrophy or pseudo- 
hypertrophy of the muscles, may be followed by paralysis of the muscles of 
the neck, the back and the abdomen, and that these do not permit a favorable 
prognosis is obvious; it is also apparent that paralysis of the muscles which 
facilitate expectoration may seriously threaten life. 

In the treatment of these forms the underlying condition must receive 



726 PARALYSES OF THE PERIPHERAL NERVES 

full attention; baths, strengthening diet, massage, electro-therapeutic treat- 
ment and, finally, rational orthopedics will in many cases produce good results. 

19. PARALYSIS OF THE CRURAL NERVE 

Paralysis of the peripheral nerves of the lower extremity is much less com- 
mon than that of the upper extremity. Paralyses affecting the lumbar plexus 
were described with paralyses affecting the region of the sciatic nerve. Of 
these we are concerned only with paralyses of the crural nerve; those in the 
course of the obturator and external cutaneous femoral nerve are more rare, 
and will merely be mentioned here. 

Wounds which affect the nerve or its surroundings (a blow, a stab, or a 
gunshot wound, fractures of the thigh, of the pelvis, etc.) are important etio- 
logical factors of crural paralysis, just as of paralysis of other peripheral 
nerves. 

Diseases of the vertebrae, particularly of the lower thoracic and upper 
lumbar vertebrae, must be considered as well as inflammation of the pelvis 
(or tumors in this region), of the femur, or of the ilio-psoas muscle. 

That neuritic processes are particularly prone to affect the crural nerve 
has been demonstrated in the last few years; I shall merely refer to post- 
typhoid and post-diphtheritic nerve degenerations and those sequential to 
chronic alcoholism or diabetes which may cause inflammation or paralysis of 
this nerve. 

Crural paralysis has been observed in the course of osteomalacia, gout, 
tabes and (usually of benign course) immediately after labor, especially in 
primiparae; in a few rather obscure cases this has also been observed after 
anesthesia. 

The region of the crural nerve is of course implicated in various cere- 
bral and spinal affections. I refer not only to progressive muscular atrophy 
but to infantile spinal paralysis and to progressive neurotic or neuritic 
atrophy. 

Finally, I desire to call special attention to the accompanying atrophy 
and debility which also appear after acute and chronic inflammations of the 
knee-joint; these pareses of the extensors of the lower leg differ in various 
ways (as we shall see when discussing the diagnosis) from other paralytic 
conditions which attack the crural nerve. 

The symptomatology of crural paralysis may be easily understood if we 
consider the function of the muscles innervated by this nerve. First, the 
motor branches of the crural nerve are distributed to the following muscles: 
The internal iliac, the psoas, the quadriceps femoris, the sartorius and, together 
with the obturator nerve, the pectineus. Paralysis of these muscles prevents 
flexion of the thigh at the hip-joint; when the thigh is flexed and the trunk 
is in the recumbent posture the patient cannot bend forward, nor can he raise 
the whole leg or extend the lower leg. Under these circumstances we can at 
once understand that the lower extremities cannot be used (as in standing, 
walking, running, jumping) if a bilateral affection is present, and even when 
the paralysis is unilateral the patients are compelled to drag the paralyzed leg 
with a pendulum motion, because on the slightest flexion of the knee they are 
in danger of falling. Paralyses of the sartorius and pectineus cause but little 



PARALYSIS OF THE CRURAL NERVE 727 

disturbance; the former muscle assists in flexing the knee- and hip-joint; the 
latter adducts the thigh and to some extent flexes it. 

Sensation is somewhat diminished upon the inner surface of the thigh 
(internal cutaneous branch), in the middle of the anterior surface of the 
thigh (anterior internal cutaneous), also on the inner side of the knee, the 
inner and anterior surface of the leg, the inner side of the ankle, and the outer 
border of the foot as far as the great toe (the large saphenous nerve). If 
there are sensory disturbances in the skin over the pubic region, in the sexual 
organs, and in the thigh, outside of the fovea ovalis, the function of the sensory 
branches of the ilio-hypogastric, ilio-inguinal, genito-crural and lumbo-ingui- 
nal nerves is also impaired. 

"While marked vasomotor disturbances are absent in crural paralysis, we 
occasionally find decided atrophy in the paralyzed muscles which, when caused 
by inflammation of the nerve or a spinal affection (as in spinal paralysis of 
infants or adults) may be combined with changes in electric contractility in 
contrast with the atrophy before mentioned which occurs in acute or chronic 
affections of the knee-joint, and in which there may also be a decided, 
quantitative, diminution of electric contractility, but in which no DeR is 
observed. 

Diagnosis. — After this description it can scarcely be difficult to diagnosti- 
cate paralysis of the crural nerve. If we note the etiologic factor trauma, the 
diagnosis is comparatively easy, as it is also when there are injuries to the 
vertebral column (fractures, dislocations), a tumor of the pelvis, or inflam- 
matory processes which affect the ilio-psoas muscle. Diagnosis becomes more 
difficult when trauma must be excluded. Here, especially if pain has preceded 
or accompanies the paralysis, we must consider neuritis in the crural region, 
such as occurs in alcoholism, in diabetes, after prolonged suppuration (I have 
seen bilateral crural paralysis in hepatic abscess which developed while in the 
tropics), in beri-beri, or after diphtheria. The examination of the urine is 
necessary, particularly if, besides the crural, the obturator and sciatic nerves 
are also implicated, for this is characteristic of diabetic neuritis. In some 
cases it may remain doubtful whether the affection is purely peripheral or 
whether the paralysis is merely an accompaniment of acute atrophic spinal 
paralysis. 

It must be remembered that individual muscles may be peculiarly affected 
in peripheral as well as in spinal processes ; for example, the sartorius may be 
exempt from paralysis, a peculiarity which I must note here without attempt- 
ing in any way to explain it. 

"We should never fail to determine the presence or absence of the patella 
tendon reflex. In the paralysis of this nerve, due to cerebral disturbance, the 
patella reflex is usually markedly increased. If the tendon reflex is absent 
the existence of a peripheral neuritic or a poliomyelitic affection is suggested: 
an accurate history and the consideration of all the auxiliary conditions will 
reveal the actual underlying affection. Remak demonstrates by careful inves- 
tigations that an increase of tendon reflexes in neuritis is always exceptional ; 
as a rule, they are absent, and when recovery ensues they remain absent much 
longer than the oralvano-muscular reactions of defeneration . 

An especially important fact is that, even without actual crural paralysis, 
without demonstrable reactions, such as occur in severe peripheral or polio- 



728 PARALYSES OF THE PERIPHERAL NERVES 

myelitic paralysis, the knee-jerk may be absent; this has been observed after 
diphtheria, and repeatedly in the case of diabetes. 

While, however, in the majority of cases of tabes (it is well known that 
in this disease the absence of the knee-jerk, the so-called Westphal's sign, is 
one of the first and most constant symptoms) the knee-jerk which has disap- 
peared may exceptionally return; and in anterior poliomyelitis it usually 
remains absent, it may happen that after diphtheria or diabetes the knee- 
jerk which has been lost may in the course of weeks or months be restored with 
an improvement in the general condition. 

Furthermore, on firm pressure above the internal femoral condyle between 
the sartorius and the vastus internus, the testicle on this side rises (cremaster 
reflex, Jastrowitz). The large saphenous nerve and the external spermatic 
nerve which innervate the cremaster muscle are branches of the crural nerve ; 
in hemiplegia this reflex is absent on the paralyzed side; whether it is also 
absent in peripheral paralysis of this nerve will be disclosed by investigation 
of such cases. 

As an extreme abnormality I must mention congenital absence of the ex- 
tensor of the leg; whether this is due to persistent or beginning muscular dys- 
trophy is as yet unknown. 

If paralysis of the crural nerve is produced by trauma of the vertebral 
column, or by tumors in this region or in the pelvis, the prognosis is usually 
unfavorable. In cerebral or spinal pathological processes its curability is 
more unlikely than when due to a peripheral lesion. When electrical exami- 
nation reveals conditions deviating but little from the normal, the more favor- 
able is the prognosis. Neuritic paralysis after diphtheria, in diabetes, or from 
chronic alcoholism affords a more hopeful prognosis because good results may 
be attained by means of tonics and general strengthening measures, and some- 
times by prohibiting injurious elements (such as alcohol). 

In many cases, at least in the first stages, the treatment should be purely 
surgical. This is especially true when the paralysis is due to injuries of the 
thigh, the vertebrae, the bones of the pelvis, or to abscesses, tumors, or other 
affections of the hip- and knee-joint. Atrophy of the thigh which is often so 
extreme in affections of the knee-joint, is favorably influenced by orthopedic 
treatment, by massage, and particularly by electro-therapy which cannot be 
too strenuously advocated. 

Atrophic paralysis which frequently persists after severe poliomyelitis and 
resists all attempts at cure, and which as yet has been treated with very little 
success, has lately in a few cases yielded to careful surgical treatment. 

Without entering into details, I may state that in a case of severe infantile 
paralysis in which the quadriceps femoris was almost wholly atrophic, F. 
Krause by a successful operation transplanted the flexors of the leg into the 
anterior side of the thigh, and thus almost wholly compensated for the loss 
of muscle by atrophy. 

20. PARALYSIS OF THE OBTURATOR NERVE 

As a supplement I mention paralysis in the course of the obturator nerve. 
Of course this nerve may occasionally be injured by trauma; more frequently, 
although rarely, it is damaged by a difficult labor or by a hernia (cases of 






PARALYSIS OF THE .EXTERNAL CUTANEOUS FEMORAL NERVE 729 

this have been mentioned in literature) or by a lesion of the lower thoracic 
or upper lumbar vertebrae. 

The symptomatology is soon apparent if the divisions of the nerve and its 
branches in the muscles of the thigh are considered. 

This nerve gives off branches to the adductors of the thigh as well as to 
the rotators ; it also supplies the pectineus and gracilis. ' The adductor magnus 
receives a branch from the sciatic nerve, the pectineus one from the crural 
nerve, and the obturator nerve not only supplies the external obturator muscle 
but, at least in part, the internal obturator also. 

In paralysis of the adductors, it becomes impossible to draw the legs 
together or to throw one leg over the other; walking is fatiguing as well as 
horseback riding, because firm pressure of the thighs can no longer be exerted. 
Some sensory disturbances appear upon the inner surface of the thigh; if a 
hernia becomes strangulated there is sometimes burning pain in this area- 
It is impossible to satisfactorily explain vasomotor and trophic disturb- 
ances, either from my own experience or from the reports in literature, nor 
can I throw much light upon the electric reactions; nevertheless we may 
assume that electric anomalies occur here as in other peripheral paralyses. 

The prognosis and treatment of paralysis of the obturator nerve is the 
same as that explicitly described for paralysis of the crural nerve. 

21. PARALYSIS OF THE EXTERNAL CUTANEOUS FEMORAL 

NERVE 

Symptoms. — Before concluding the discussion of paralyses in the region 
of the crural nerve we must briefly consider a peculiar symptom-complex due 
to an affection of the external cutaneous femoral nerve, which has only lately 
attracted the attention of physicians. 

This nerve, which carries sensory fibers to supply the skin of the anterior 
and external surfaces of the thigh, is subject to disturbances which cause those 
who are affected to complain of abnormal sensations in the region it supplies; 
for instance, of numbness which, upon objective testing of sensation, fre- 
quently shows a diminution in the various qualities of sensation. 

Actual pain, particularly when the patients sit or lie down, there is none; 
but on standing or walking about the feeling of numbness is often increased 
to seemingly actual pain. This affection which was first described by me and 
immediately after by Roth, occurs in both sexes but in women much less fre- 
quently than in men. Roth gave to this affection the name meralgia paraes- 
thetica; it is usually unilateral but may be bilateral, and among its causes we 
may enumerate the effect of cold in this region of the body, infectious diseases, 
lead poisoning, trauma, syphilis, chronic alcoholism, gout, pregnancy, too firm 
pressure around the brim of the pelvis (from tight-lacing or pressure of the 
belt which supports the trousers). The anatomical course of the nerve in the 
fibrous canal of the fascia lata perhaps explains the abnormal tension and 
high degree of pressure upon the nerve, which, in the few cases in which 
autopsy has been performed, has been found thickened and spindle-shaped with 
the characteristic signs of neuritis and perineuritis. 

It is remarkable that this paresthesia, limited to the external cutaneous 
femoral nerve, should occur so frequentlv in phvsicians; a number of descrip- 
48 



730 PARALYSES OF THE PERIPHERAL NERVES 

tions of this peculiar affection have been written by colleagues who have 
suffered from it. Pal recently called attention to the fact that a certain 
proportion of those who suffer from this affection also suffer from obesity 
and flat-foot, due to a displacement of the static relations of the leg, and 
probably to an over-distention of the hip- joint. 

The prognosis is generally favorable. In the majority of cases we are 
dealing with an affection of only one nerve, and the symptoms of which the 
patients complain and which they regard as indicating a severe or progressive 
nervous disease, may be relieved. 

It is always wise to examine the patient carefully since there are cases 
reported which show that the disease may occur in tabes, in hematomyelia, 
in malignant affections in the cavity of the pelvis, and in paralysis. 

In treatment, prophylaxis is of the greatest importance. Tight lacing 
must be prohibited, and also the use of cold douches ; persons whose occupa- 
tions force them to stand for a long time (I have noted the affection par- 
ticularly in waiters and the police) should be advised to take periodic rest; 
if there is flat-foot, measures should be taken to relieve this affection, appa- 
ratus being manufactured from firmly resistant material, and this must 
extend from the heel to the tip of the great toe. 

Operation (resection of the nerve) is advisable only when very severe 
pain makes walking impossible. This will stop the pain; naturally, it is 
succeeded by complete anesthesia in the region of the resected nerve. But in 
the 'reports of such operations in literature we note that amelioration is not 
always immediate, and that relapses occur. 

Of course, if there is a severe affection of the spinal cord, no operation 
can be undertaken. Besides treating the probable underlying affection (gout, 
rheumatism, alcoholism, etc.), inunctions, lukewarm salt baths, massage and 
treatment with a faradic brush are to be recommended. 

Often the disturbances are so slight that the affection is only accidentally 
discovered by the physician. 

22. PARALYSIS OF THE SUPERIOR AND INFERIOR GLUTEAL 

NERVES AND OF THE POSTERIOR CUTANEOUS 

FEMORAL NERVES 

Before describing paralyses of the sciatic plexus and of the most impor- 
tant nerve of this plexus, the sciatic and its branches, I must briefly refer to 
those paralyses in the course of the three upper nerves belonging to this 
plexus; namely, the superior gluteus, the inferior gluteus, and the posterior 
cutaneous femoral nerves. The inferior gluteal nerve supplies the gluteus 
maximus ; the superior innervates the gluteus medius and minimus, the tensor 
fascia lata, and the pyriformis; while the posterior cutaneous femoral is 
chiefly a cutaneous nerve which is distributed to the skin over the buttock, the 
internal and upper portions of the thigh, and over its posterior surface down 
to the popliteal space. 

An isolated paralysis of this nerve can scarcely occur ; but it is implicated 
in lesions of the conus of the spinal cord or of the cauda equina if these are 
due to injuries of the lowest portions of the back or the upper lumbar verte- 
brae, or to a fall upon the buttocks. These lesions, as well as direct injury 



PARALYSIS OF THE SCIATIC NERVE 731 

to the sacrum or tumors of this region, also lead to paralysis of the muscles 
supplied by the gluteal nerves. While, however, isolated paralysis in these 
nerve and muscular regions is very rare, paresis, particularly of the muscles 
of the buttock, occurs in progressive muscular atrophy, especially in the 
affection known as pseudo-hypertrophy. If the gluteus maximus, which draws 
the thigh backward and outward and when the thigh is fixed extends the 
trunk, is paralyzed, climbing steps and rising from a sitting or recumbent 
posture become exceedingly difficult. The " climbing up upon himself " which 
is so characteristic of pseudo-hypertrophy, is also seen when these patients rise 
from a recumbent posture, and is due to paralysis of the large muscles of 
the buttock, combined, of course, with a co-existing weakness of the muscles 
of the back. If the gluteus medius and minimus which abduct the thigh are 
paralyzed, and simultaneously the pyriformis (the gluteus medius with the 
posterior portion of its fibers), these movements are weak or cannot be made 
at all. In paralysis of the gluteus medius and gluteus minimus the abduc- 
tion of the leg and its rotation inwardly is defective ; and as the tensor fascia 
lata assists the ilio-psoas in flexing the hip and at the same time rotates the 
thigh a little inward 1 v, flexion of the thigh, if this muscle be paralyzed, will 
be less complete and in walking the leg will be rotated outwardly. 

Among the trophic disturbances we recognize an apparent increase in 
volume, and, upon tapping, a distinct tension of the muscles of the buttock 
which in progressive muscular atrophy become extremely emaciated; this 
may also be observed in severe sciatica and in chronic affections of the 
hip-joint. Aside from the previously mentioned pathologic conditions in the 
course of the posterior cutaneous femoral nerve, little is known of sensory 
disturbances of other nerves; on electric examination the muscles generally 
show a diminished power of contractility, which varies according to the sever- 
ity of the affection. With widely distributed muscular disease or a spinal 
lesion causing paralysis of the muscles of the buttock, the prognosis is less 
favorable than when these are due to sciatica or to a disease of the hip-joint 
which causes atrophy ; the treatment is the same as that described in paralysis 
of other peripheral nerves. 

23. PARALYSIS OF THE SCIATIC NERVE 

In describing paralysis of the sciatic nerve, I must mention at the outset 
that paralysis of the entire nerve trunk may occur, but that much more fre- 
quently the paralyses which come under professional treatment are due to 
damage of one of its main branches, either the peroneal or tibial nerve. 

The nerve trunk which traverses the pelvis may be injured by fractures 
of the lower vertebrae or of the sacrum, by injuries to the cauda equina, by 
tumors of the pelvis, particularly those of the uterus (in difficult labor by 
pressure of the child's head), which impair its integrity; or it may be dam- 
aged by injury to the femur or by tumors which have developed within it or 
in its vicinity. It is self-evident that it may also be injured by direct lesions 
(a blow, a stab, etc.). Among the chief etiologic factors of paralysis of an 
entire nerve or one of its branches are inflammations which may be purely 
rheumatic, neuritic, infectious, or septic, these affecting the nerve after vari- 
ous infectious diseases, in the puerperal period, in chronic suppuration, in the 



732 PARALYSES OF THE PERIPHERAL NERVES 

course of acute or chronic intoxication from alcohol, carbon dioxid, arsenic, 
and lead, and also in the course of diabetes. 

The paralyses of the peroneal nerve which appear early in tabes and, inde- 
pendent of the progress of the underlying affection, result in recovery are 
especially interesting. Similar conditions in progressive paralysis have been 
noted by different authors; lately reports have been published of paralysis 
which occurred (more readily than in the healthy) from the pressure of one 
leg being thrown over the other or from the arthritic affections which not so 
rarely occur in tabes (in these cases, naturally, those of the knee-joint). 
In the description of peroneal paralyses which we shall soon discuss other no 
less interesting etiologic factors will be mentioned. 

In describing paralysis of the two main branches of the sciatic nerve, the 
peroneal and the tibial, symptoms due to paralysis of the upper branches 
of the trunk must be enumerated, and here the outward rotators of the thigh 
and the flexors of the leg are particularly involved. 

The outward rotators are the quadratus femoris, the gemelli, the obturator 
internus. A lesion of the branches which innervate these muscles impairs 
the motion as previously described. If the flexors of the leg (the semi- 
membranosus, semitendinosus, and the biceps femoris) are paralyzed the 
leg cannot be flexed. By hyperextension in the knee-joint, the leg becomes 
stiff. 

Even if the peroneal nerve remains exempt in such a paralysis, the short 
head of the biceps femoris innervated by it may cause a weakened power of 
movement. 

(a) PARALYSIS OF THE PERONEAL NERVE 

Prior to a description of the symptomatology of peroneal paralysis, I 
must mention the etiologic factors which may induce such paralysis. It is 
prone to occur in women with a narrow pelvis after the expulsion of a very 
large child (maternal obstetric paralysis) and resembles that occurring in 
new-born infants who have been delivered by the feet. In rare cases subcu- 
taneous injections of ether in the posterior side of the thigh may cause paral- 
ysis of the peroneal nerve (analogous to radial paralysis). Those cases are 
particularly interesting which occur in persons who are much upon their knees, 
for instance, farm laborers, asphalt workers, plumbers, etc. The tibial nerve 
is, of course, sometimes implicated, but never to the same extent as the pero- 
neal nerve. Among these occupation pareses, so-called, we may include the 
paralyses occurring in workmen who hold the article upon which they work 
between the knees, which sustain a heavy pressure. 

Strong pressure upon a nerve during anesthesia is also a cause of paralysis ; 
isolated cases have been reported from sudden torsion of the leg while slipping 
manacles around it (analogous to paralysis of the radial or other nerves of 
the arm from handcuffs), in persons who walked upon stilts, and after severe 
injuries while playing football. I shall not describe the other rare and excep- 
tional paralyses of this nerve; for example, paralysis after chorea; more 
important are those which, like radial paralysis from pressure during sleep, 
attack the peroneal nerve after sleep, and those which Oppenheim and Stein 
have recently described as senile forms of multiple neuritis. Finally, I must 
acknowledge that in many cases no etiologic factor can be detected, provided 



PARALYSIS OF THE SCIATIC NERVE 733 

that in cases of this kind we do not regard pressure effects to be increased by 
chronic alcoholism. 

Certainly in some cases such a predisposition may be assumed; in other 
cases we must accept a report of exposure to cold, of rheumatism, etc. But 
in such cases we should never omit the examination of the urine for albumin 
and sugar ; a positive finding of either of these will often clear up a case eti- 
ologically obscure. 

The symptomatology of peroneal paralysis is readily understood if we con- 
sider which muscles are supplied by this nerve. The most conspicuous feature 
is the lost power of flexing the foot dorsally and of extending the basal pha- 
langes of the toes. The foot which hangs down flaccidly glides over the floor 
upon its tip; to obviate this the patient strongly flexes the thigh and the 
leg and foot are thrown forward. This gait is very characteristic; Charcot 
calls it the steppage gait (demarche des steppeurs), equine gait. It is due to 
paralysis of the following muscles : the tibialis anticus, the extensor communis 
digitorum, extensor hallucis longus, extensor digitorum et hallucis brevis, and 
of the first dorsal interosseus muscle. 

In complete paralysis of the nerve the peroneus longus and peroneus brevis 
are both inactive. The first named muscle flexes the foot toward the sole, 
abducts it, and at the same time lowers the anterior portion of the inner bor- 
der of the foot; it raises the external border of the foot, at the same time 
abducting it by the action of the peroneus brevis. 

Disturbances of sensation in peroneal paralysis are rarely marked; the 
patients complain of a feeling of cold and numbness; objective examination 
reveals hyperesthesia or anesthesia at the outer anterior side of the leg, upon the 
dorsum of the foot, and the posterior surface of the toes. Implicated in this 
are the cutaneous branches of the peroneus (the posterior, middle and external 
cutaneous crural, the internal and middle cutaneous dorsi pedis, and a few 
branches of the peroneus profundus). In profound paralysis the muscles chiefly 
implicated are more or less conspicuously atrophied,, and electric contractility 
reveals distinct reactions of degeneration ; of course, many cases are reported 
in literature of less severe paralysis with less marked electric alterations. 

Among vasomotor disturbances we may mention the bluish, livid discol- 
oration of the member which has been described in some cases, as well as 
the abnormal growth of the nails and hair, and the formation of bedsores in 
the areas subjected to great pressure (the heel, trochanter, etc.). But these 
symptoms are rare, and are observed only in consequence of severe traumatic 
lesions of the trunk of the sciatic nerve. 

An especially interesting and important fact is that traumatic paralysis 
of the peroneal nerve, and also that which is regarded as the sequel of neuritis, 
does not always implicate all of the fibers. The branches intended for the 
tibialis anticus are sometimes exempt, while in other cases these, also those 
supplying the other extensors, are likewise affected, the peroneal muscles re- 
maining intact. I shall revert to these conditions in considering the diagnosis. 

(b) PARALYSIS OF THE TIBIAL NERVE 

Isolated paralyses of the tibial nerve are much more rare than those of 
the peroneal. In paralysis of the tibial nerve the function of the muscles 



734 PARALYSES OF THE PERIPHERAL NERVES 

innervated by this nerve naturally suffers, particularly the triceps surae, that 
is, the gastrocnemius and the solius muscles. 

These extend the foot downward and abduct it. In paralysis of this nerve 
it is impossible to flex the toes or to stamp vigorously upon the floor. 
In paralysis affecting the long and short flexors of the toes these movements 
are likewise impossible, as well as individual movements of the large and 
small toes which depend upon the abductor hallucis, the adductor hallucis, 
the flexor brevis, the flexor digiti minimi, and the abductor digiti minimi. 
If the tibialis posticus is paralyzed the power to turn the internal border 
of the foot upward and to abduct the foot is lost. By paralysis of the interossei 
pedis muscles which (together with the lumbricales pedis) extend the middle 
and terminal phalanges and flex the basal phalanges, a deformity is produced, 
the "pied en griffe," which resembles "main en griffe," the basal phalanges 
being dorsally flexed, the other two phalanges showing marked plantar flexion. 

Disturbances of sensation caused by paralysis of the tibial, like those in 
paralysis of the peroneal nerve, affect the skin of the calves, the region of 
the ankle, the external border of the foot, and, finally, the under part of the 
toes. The nerves affected are the suralis, the cutaneous plantar branches, and 
the external and internal plantars. 

Before discussing the diagnosis, prognosis and treatment of these paral- 
yses I must state that, particularly in partial peripheral or spinal paralysis 
of the lower extremities, abnormal positions of the foot are assumed in con- 
sequence of loss of function of individual muscles ; these we cannot here com- 
prehensively describe, but I must mention a most significant fact. In partial 
paralysis (which is chiefly found in children as the consequence of infantile 
spinal paralysis) pes varo-equinus results from contracture of the plantar 
flexor. If the flexors of the foot are paralyzed, from contracture of the dorsal 
flexor, pes calcaneus follows; modifications are noted if the peroneus longus 
is implicated in the paralysis. These conditions, in themselves highly inter- 
esting, are especially so to the surgeon and the orthopedist, for reasons which 
cannot here be minutely given. This is particularly true in children in whom 
the conditions are chiefly observed as the result of spinal cord disease, for 
they not only suffer from muscular paralysis but the growth of their bones, 
etc., is impeded. 

The diagnosis of paralysis in the course of the sciatic nerve must be based 
upon the function of individual muscles, and this can hardly be difficult if 
there is a history of trauma. If external injury can be excluded, the eti- 
ologic factors before enumerated must be sought for, and we must consider 
in the given case whether we are dealing with paralysis following a difficult 
labor, or one that has occurred during the puerperal period, whether febrile 
infectious diseases have preceded, or intoxications from organic or inorganic 
poisons (alcohol, lead, arsenic, sugar, etc.). 

Especial significance must be attached to paralysis of the lower extremities 
which is so common in children in consequence of acute poliomyelitis. It is 
true that peripheral paralysis of the lower extremities also occurs in children, 
but here an accurate history will be a valuable aid. If no trauma can be 
detected, if other members are implicated, if individual muscles are exempt, 
if there is extreme atrophy of the affected limbs and decided diminution of 
electric contractility, the diagnosis of infantile spinal paralysis can cause no 



PARALYSIS OF THE SCIATIC NERVE 735 

perplexity. But I must mention a peculiarity often observed of late, that 
lead paralysis in the lower extremities is also observed in children, and that 
in children these members appear to be more frequently implicated than the 
upper extremities, while, as is well known, quite the reverse is true in adults. 

We must also bear in mind that in paralysis of the lower extremities from 
poliomyelitis individual muscles are frequently exempt (for example, the sar- 
torius in the region of the crural nerve, the tibialis anticus or the peroneus 
in the field of the sciatic) ; this peculiarity, however, is noted not only in 
spinal but also in peripheral paralysis. But this fact alone does not justify us 
in forming conclusions as to the seat of the lesion. 

Pathologic conditions are occasionally observed in the form of juvenile 
dystrophy or progressive neurotic (spinal neuritic) muscular atrophy. Here 
an accurate history, the occurrence of the disease in several members of the 
family, or heredity, the symmetry of the paralysis in the two sides of the 
body, and the greater advance of the pathologic process in the upper extrem- 
ities will prevent us from confounding conditions of this kind with periph- 
eral paralysis. 

If the vertebral column has been injured (shock, fractures, dislocations 
of the lower dorsal and upper lumbar vertebrae, etc.) we may regard paralysis 
of the muscles of the lower extremities as due to a lesion of the spinal cord 
or the cauda equina. At this point I cannot fully discuss the special diag- 
nosis; here our knowledge of other diseases of the spinal cord must be our 
guide or the reader is referred to other articles of this book. 

Before considering the prognosis and treatment of paralysis in the course 
of the sciatic I must mention a rare condition occasionally reported in litera- 
ture, appearing particularly in paralysis of the peroneal nerve, which is due 
neither to a lesion of the peripheral nerve nor to disease of the spinal cord, but 
is an affection of the upper portions of the central convolutions in the brain. 
Cerebral paralyses in the region of the peroneal nerve are characterized by 
the fact that they are usually of traumatic origin, that the paralysis although 
prominent is not the only symptom (weakness in the areas of the facial 
nerve and in the upper extremities will always appear), and that the electric 
reaction of the paralyzed muscles is absolutely retained. 

In regard to prognosis it may be stated that traumatic paralyses or those 
due to neuritic processes are less intractable than those attributable to disease 
of the brain or spinal cord, or which develop in the course of progressive 
muscular atrophy. 

In peripheral, traumatic and neuritic paralyses of the peroneal nerve the 
old law so frequently emphasized is still applicable, that when the electric 
contractility is retained or but slightly altered there is a better prospect of 
restoration to health than when it shows marked change. 

In the treatment, traumatic lesions of the nerve necessitate the same 
surgical procedures as are indicated in fractures, dislocations, impacted for- 
eign bodies, etc. If it is possible to reunite by suture a nerve which has been 
severed, this must, of course, be done, and. in fact, has been done several 
times. Surgical treatment is also necessary when the vertebral column is 
injured or when, as in rare cases, intravertebral tumors have formed. If we 
remember that many paralyses in the course of this nerve are due to neuritis 
from toxic causes, it becomes our duty to discover this cause, and to preserve 



736 PARALYSES OF THE PERIPHERAL NERVES 

the patient from its injurious effects (lead, arsenic, alcoholic intoxication, 
etc.) ; if there is diabetes appropriate treatment must be instituted. 

When all of these indications have been fulfilled, the electric current in 
its various forms offers a mode of treatment not only in peripheral but in 
spinal paralysis which if persevered in is sometimes very satisfactory. In 
spinal paralysis it is most necessary to combine peripheral with so-called cen- 
tral treatment, i. e., to subject the diseased spinal cord itself to the influence 
of the electric current. It is not my purpose to indicate here how this may 
be accomplished; text-books upon electro-therapy should be consulted. 

If, as unfortunately often happens in paralysis of the lower extremities, 
all our efforts are unavailing, the aid of the orthopedist must be invoked, and 
apparatus must substitute for the loss of function in the muscles. This idea 
of substitution has recently led surgeons to unite the muscles capable of func- 
tion with the diseased ones by a tendon transplantation, with remarkably good 
results. 

24. PARALYSIS IN THE COURSE OF THE PUDENDIC 
AND COCCYGEAL PLEXUSES 

Paralysis in the course of the pudendic and coccygeal plexuses and their 
nerves is of very rare occurrence, and in the majority of cases is very diffi- 
cult to differentiate from that of the cord in which they originate. 

In the etiology we usually find the history of an injury to the lowest por- 
tion of the vertebral column (the lower thoracic, lumbar and sacral verte- 
brae) sustained by a fall from a height which has produced either a fracture, 
a dislocation, or a severe contusion. These parts may also be directly injured by 
the falling upon them of heavy weights. Besides wounds, tuberculous and 
syphilitic diseases of the vertebrae and congenital anomalies (spina bifida) 
may be a cause. In the act casuistically described as " falling on the feet " 
there may be no demonstrable lesion of the vertebrae yet compression or hem- 
orrhage into the lumbar cord or its lowest portion, the conus medullaris, may 
have taken place. A purely inflammatory affection limited to the lower tho- 
racic, lumbar or sacral portions of the spinal cord is very rare. 

Syphilitic affections of the lowest portions of the cord and the membranes 
which envelop and compress the cauda equina are more common, above all, 
neoplasms in the sacral canal by which the nerves are compressed and ren- 
dered incapable of function. 

From a purely scientific standpoint as well as with a view to treatment, it 
is most necessary for the physician or surgeon to differentiate a lesion in 
the lowest part of the lumbar cord or the conus terminalis from one in the 
cauda equina. In the last twenty years authors have directed special atten- 
tion to this differentiation. 

The following points must be borne in mind: First, affections of the 
cauda are produced much less rapidly than those of the conus, as may be in- 
ferred from the preceding statements as to the cause of these diseases. In 
the overwhelming majority of cases a lesion of the conus produces no pain, 
while in an affection of the cauda, particularly at the onset, there is acute 
pain, localized in the sacrum, the bladder, the rectum and the buttocks, 
and this often radiates. Furthermore, in caudal affections motor irritative 



PARALYSIS IN THE PUDEXDIC AXD COCCYGEAL PLEXUSES 



737 



phenomena are absent (fibrillary contractions) while they are common in 
diseases of the conns. 

Symptoms. — The most constant disturbances in lesions of the lower por- 
tions of the spinal cord, as well as of nerves originating in this region, are 
those which affect the innervation of the bladder and the rectum, these organs 




being paralyzed and their normal functions suspended. This paralysis of the 
bladder and rectum is rarely found without a simultaneous disturbance of 
sensation along the course of the nerves originating from the pudendic and 
coccygeal plexuses. 

This disturbance in sensation affects the perineum, the posterior surface of 



738 PARALYSES OF THE PERIPHERAL NERVES 

the scrotum, the penis, or the labia majora in the female. There is also anes- 
thesia in the region of the anus, the anal groove, in the buttocks, and upon 
the posterior surface of the thigh to the upper border of the middle third. 
The mucous membrane of the genital organs and the rectum is also implicated 
in the anesthesia. 

Before describing the motor symptoms in these cases, I must call attention 
to the fact, most important in the differential diagnosis, that these disturb- 
ances of sensation may be distinguished by the time of their appearance and 
the degree of completeness (an important guide). If the conus is injured, 
anomalies of sensation soon develop without preceding pain. Furthermore, 
the disturbances of sensation in the majority of the pure cases present the 
characteristics of so-called dissociated sensory paralysis, i. e., tactile sensation 
is retained in contrast with the almost utter absence of pain and temperature 
sensibility. In opposition to this the sensory disturbances which appear grad- 
ually, and if there be disease of the cauda equina usually accompanied by very 
severe pain, are marked by complete anesthesia — facts which may occasion- 
ally be of the utmost significance in the differential diagnosis and in treat- 
ment. 

The sexual function is usually impaired; there may be complete impo- 
tence; but with retained libido cceundi only the property of ejaculation of the 
semen may be implicated, yet, in spite of existing libido and the power of 
erection of the member, impotentia generandi may be produced. 

If the lesion is above the point where the last three sacral roots branch 
off, therefore in the upper portion of the conus, in addition to the symptoms 
enumerated there is paralysis of the muscles of the buttock, the flexors of 
the leg (the muscles upon the posterior side of the thigh) and the entire 
musculature of the leg and foot. Then the anesthesia is more pronounced; 
the entire posterior side of the thigh is involved as well as the posterior and 
lateral portions of the leg, and the foot with the exception of a small portion 
on the internal border. 

The paralyzed muscles are atrophic, their electric contractility severely 
damaged. 

When the focus of disease is still higher (implication of the vertebral 
cord at the height of the twelfth thoracic or first lumbar vertebra) all the 
muscles of the lower extremity are paralyzed, are atrophic, and their electric 
contractility seriously impaired; the reflexes disappear, anesthesia extends to 
the mons Veneris. Marked trophic disturbances in the form of bed-sores upon 
the sacrum, the trochanters and the heels, are not uncommon. I have now 
described the symptomatology of these interesting paralyses as well as called 
attention to a few guiding points in the differential diagnosis between lesions 
of the conus and the cauda. I must also state that in the majority of cases 
this fine discrimination is impossible because the cord as well as the nerves 
of the cauda are often simultaneously affected by the injury or the disease. 
Fr. Schultze describes another important point : Whether we are dealing with 
disease of the conus or cauda the diagnosis in the given case depends not only 
upon the height of the vertebra injured, but also upon the transverse distribu- 
tion of the original disturbance. In a lesion of the lower portion of the lum- 
bar enlargement without implication of the caudal roots, a paralysis may 
appear which implicates only the sciatic distribution (seat of the lesion at the 



PARALYSIS IN THE PUDENDIC AND COCCYGEAL PLEXUSES 



739 



height of the twelfth thoracic and first lumbar vertebra) ; on the other hand, 
with a transverse lesion at the height of the second lumbar vertebra (for 
example, from tumors which fill the vertebral canal), there may be complete 
motor and sensory paralysis of all the nerve tracts of the lower extremities, 
so far as the roots of the crural and obturator nerves are here concerned. 






««tf., 




Fig. 192. 



These nerve tracts may remain unaffected after a complete transverse lesion 
extending the entire length of the vertebral canal if the affected roots have 
already emerged from the vertebral canal (when the lesion is below the third 
lumbar vertebra). 

In the prognosis of disease of the lower portions of the spinal cord and 
of the cauda equina we must always consider the severity of the damage. An 



740 



PARALYSES OF THE PERIPHERAL NERVES 



injury or disease of the cord is usually serious, although this is not invariably 
the case, for there are instances reported in literature which show that lesions 
of the cauda more often terminate seriously than those of the conus, although 
the latter are generally regarded as more dangerous. In not a few cases the 
local paralysis in certain areas of the lower extremities may in the course of 
weeks gradually disappear so that merely paralysis of the bladder and rectum 
and a disturbance of the sexual function remain. If the disease is due to 




Fig. 193. — Diagram of Spinal Sensation. (After Seiffer.) 



syphilis the prognosis is somewhat more favorable; when there is certainly a 
tumor of the cauda equina, there is some prospect of recovery if proper surgi- 
cal aid is at hand. 

Treatment. — In any case of injury to the vertebrae, surgical treatment is 
the first indication. In fracture or dislocation of the vertebras the surgeon 
must decide whether anything can be done to relieve the compression of the 
spinal cord and its lower roots. It is the duty of the physician to see that 
such patients are kept in a proper position and, above all, he must endeavor 



PARALYSIS IN THE PUDENDIC AND COCCYGEAL PLEXUSES 741 

to prevent bed-sores. In most cases catheterization must be resorted to on 
account of the paralysis of the bladder usually present, and one of the physi- 
cian's most important duties is to prevent infection of the mucous membrane 
of the bladder. 

If syphilitic infection has preceded, antisyphilitic treatment is, of course, 
in order. Lukewarm baths, suitable electric treatment, and subcutaneous 
injections of strychnin are useful in many cases. If preceding syphilis is 
excluded, and the affection progressively develops, the severe pain and other 
diagnostic factors which have been described will convince us that we are 
dealing with a slowly growing tumor in the lowest part of the spinal cord, 
and the necessity and feasibility of operation must be considered. 

Admirable results have several times been obtained by the combined skill 
of the neurologist and surgeon. 



NEURITIS AND POLYNEURITIS 

By R. CASSIRER, Berlin 

NEURITIS 

Case 1. — The patient under consideration, a laborer aged 30, states that two weeks 
ago he had an attack of influenza accompanied by fever, headache, lassitude, cough, 
and coryza ; he was confined to bed for three days. When he left his bed he noticed 
a numbness on the side of the right little finger with stabbing pain on the ulnar side 
of the forearm extending from the elbow to the hand, and soon after a feeling of 
weakness when performing certain movements with the right hand. The numbness 
and tingling were especially marked when the forearm rested on a hard surface. The 
patient has sustained no injury in the course of his occupation, does not use alcohol, 
and has not had syphilis. He reports that he several times suffered from lumbago. 
Examination reveals the following conditions: 

The right ulnar nerve is very painful when pressure is made upon the internal 
condyle, the pain radiating throughout the entire hand; the radial nerve also is some- 
what sensitive to pressure, but not the other nerves. The right ulnar nerve on com- 
parison with the left appears rather thicker. The right hand shows the so-called 
claw-hand (main en griff e) , the basal phalanges of the fingers being hyperextended, 
the middle and terminal phalanges flexed. The anomaly is more conspicuous in the fin- 
gers from the index to the little finger. The interosseous spaces are greatly depressed, 
especially the third and fourth. The region of the adductor pollicis is also depressed. 
The patient is unable to spread his fingers or to adduct them; neither can the thumb 
or little finger be abducted to any extent. The basal phalanges cannot be energetically 
flexed, nor can the terminal phalanges of the fingers be forcibly extended; this is most 
noticeable in the last three fingers. The terminal phalanges of the third and fourth 
fingers cannot be vigorously flexed. On the contrary, the apposition of the thumb is 
good, and the remaining muscles of the right arm are normally strong. On brush con- 
tact there is hypesthesia, and needle pricks in the areas innervated by the ulnar nerve 
(that is, in the hollow of the hand, the little finger, especially the ball of the little 
finger, and the ulnar surface of the third finger) are less painful than in normal areas. 
Upon the dorsum of the hand these disturbances of sensation are somewhat more 
extensive, for they partially implicate the radial portion of the third finger and the 
ulnar distribution of the second. The tendon reflexes of the arm are preserved. Elec- 
trical examination reveals contractility of the right ulnar nerve to the faradic as well 
as to the galvanic current, while in the left it is moderately decreased. In addition, 
the faradic contractility of the paretic muscles is diminished; stimulation of the mus- 
cles with the galvanic current reveals quantitatively normal conditions. The anodal 
contraction does not exceed the cathodal closure contraction, but no remarkable slug- 
gishness is observed. The patient is a strong healthy man.i 

This case is quite simple. As is evident from the history, the affection 
is strictly limited to the course of the right ulnar nerve, and presents the 
typical symptoms of neuritis occurring immediately after acute influenza. 

i This case, like the majority of those to be detailed, is from the Clinic of Professor 
Oppenheim, to whom I am greatly indebted for the privilege of using this material. 

742 



NEURITIS 743 

Accordingly the diagnosis is neuritis of the rigid ulnar nerve following influ- 
enza. In fact we here find all the symptoms which are requisite or important 
in neuritis. The patient first observed tingling, numbness, and pain. Our 
patients usually describe this neuritic pain as drawing, tearing, stabbing, or 
boring, or, at least in local neuritis, as limited to the definite distribution of 
the nerve. The pain is most severe in this region, but it is sometimes felt in 
one, sometimes in another area, and is subject to periodical variations; at 
the onset and at the height of the disturbance it is more or less constant, and 
this differentiates it from the lancinating pain of tabetics, as well as from 
true neuralgia. It is increased by all manipulations calculated to remove 
the nerve from the median position which is instinctively assumed by the 
patient. If, for example, I passively extend the elbow of the patient and 
hyper-extend his wrist, thus pulling upon the ulnar nerve, the pain is increased. 
Pressure upon the nerve also intensifies the spontaneous pain. This tender- 
ness of the nerve to pressure is another significant symptom, but one that 
must be utilized with care as we shall soon see. The pain is extreme when 
we press the nerve against a bony under-surface, such as the internal 
condyle; the sensation immediately over the wrist when we press the 
ulnar nerve against the styloid process is exceedingly disagreeable, and 
in many cases the affected nerve is sensitive to pressure throughout its 
entire course. The sensitiveness may be limited to the point of compres- 
sion; usually pressure produces pain peripherally over the course of the 
nerve, but it may often radiate centrally. Valuable as is this symptom for 
diagnosis, one point must not be overlooked. Even pressure upon a normal 
nerve will produce an unpleasant sensation; for instance, as we all know 
by experience, when we accidentally strike the internal condyle, and thus 
cause shock to the ulnar nerve. This sensitiveness of the nerve is frequently 
extreme in persons with neurasthenia and hysteria. In the peripheral nerves 
of neurotic individuals there is not rarely a pathological increase of tender- 
ness on pressure, appearing at one time in one, at another time in another 
nerve. 1 We cannot base our diagnosis of neuritis (as Franke 2 incorrectly 
assumed to be possible) on a few patients who have recovered from influenza. 
It has also been pointed out (especially by Remak 3 ) that when secondary 
nerve degeneration takes place — for example, after poliomyelitis — the nerve 
may be sensitive to pressure. Therefore, in my opinion, when differentiating 
neuritis from poliomyelitis too great value should not be attached to this 
symptom. 

It is difficult to decide whether we are justified in speaking of swelling 
of a peripheral nerve; when the affection is unilateral comparison with the 
normal side aids us. Little significance can be attached to slight differences, 
although the swelling, usually spindle-shaped, is occasionally most distinct. 
This is commonly observed in leprous neuritis, but Remak saw it also in 
syphilitic neuritis, Meyer and Henschen observed it in facial paralysis, Oppen- 
heim found it in an alcoholic who complained of severe pain in the course 
of the peroneal nerve, and in whom the inner side of the biceps tendon was so 

i Oppenheim, " Zur Differentialdiagnose der Neuritis." Journal filr Psychologic 
und Xeurologie, Bd. I, 1902, p. 129. 

2 Franke, Mittheil. aits dem Grenzgebiete der Medicin und Chirurgie, Bd. V. 1900. 

3 Remak, " Neuritis und Polyneuritis." Wien, 1900, p. 92. 



744 NEURITIS AND POLYNEURITIS 

swollen that its circumference was more than double that of the normal nerve 
of the other side. In another patient he found the median nerve almost 
wholly transformed into a thick hard cord. 

Of subjective symptoms the only important one is paresthesia in the 
area innervated by the diseased nerve; this is usually described as tingling or 
numbness. Such paresthesias are rare under normal conditions, and patients 
describe them variously : for instance, they say the sensation is as if ants were 
crawling over the skin (formication), or they feel as if an electric battery 
were being applied to the skin, etc. These paresthesias are most marked in 
the terminal distribution of a nerve. In comparison with the sensory, the 
motor irritative phenomena are less numerous, but are most common in trau- 
matic neuritis. They consist of slight contractions and spasmodic and fibril- 
lary movements in the paralyzed areas. These phenomena may occur at the 
onset of paralysis as well as during the time of restitution. In cases of severe 
facial paralysis from which there has been only partial recovery, we quite 
often find not only contractures upon the paralyzed side but also conjoined 
movements and clonic contractions. 1 Our patient did not present this 
symptom. 

Among the cardinal symptoms of neuritis is paralysis, which rarely fails 
to appear as soon as the pathologic process attacks a mixed nerve. Its onset 
may be sudden or gradual ; even the entire region of a plexus may be attacked 
by an acute, almost apoplectiform, paralysis, and in localized neuritis pareses 
may implicate one muscle after another. In the majority of cases of localized 
neuritis, paralysis is almost uniform in the entire area of distribution. It 
is frequently noted that the muscles most distally situated are the ones most 
severely damaged. In ulnar neuritis the flexor carpi ulnaris and the ulnar 
portion of the flexor digitorum profundus are usually less markedly implicated. 

For various reasons paralysis affecting the different muscles innervated 
by one nerve may be unequal. The exciting cause may affect a nerve in an 
area where it gives off fibers for certain muscles — a so-called crutch paralysis 
in which the pressure producing neuritis affects the axilla and implicates all 
the muscles supplied by the radial nerve including the triceps. When injuries 
affect the radial nerve in the upper arm, the triceps remains intact, and the 
supinator longus may be exempt even when the lesion is more distally situ- 
ated. Furthermore the pathological factor may have a directly selective action 
upon the muscles supplied by one nerve; a typical example of the elective 
and, we might almost say, of the systematic influence of such pathologic 
causes is radial lead paralysis in which the supinator longus is always exempt, 
and frequently the abductor pollicis. Therefore the diagnosis of neuritis of 
a single nerve cannot be based upon the fact that all of the muscles which it 
innervates are uniformly implicated. 

If the disturbance of conduction is less intense, there is weakness or paresis 
instead of paralysis. In our patient there were distinct atrophic and qualita- 
tive changes in the electric contractility of the paretic muscles, and this find- 
ing is typical in peripheral neuritis. Neuritic paralysis is atrophic and degen- 
erative. In both the acute and subacute forms — i. e., in nearly all cases — 
atrophy soon follows paralysis. In the course of a few weeks it reaches its 

i See Bernhardt's article in this volume, p. 662. 



NEURITIS 745 

acme, and, if marked, produces extremely characteristic deformities which 
aid in the diagnosis. For instance, our patient presented the typical claw- 
hand: hyperextension of the basal phalanges with marked flexion of the mid- 
dle and terminal phalanges, as well as depression of the interosseous spaces 
and flattening of the ball of the little finger. This deformity originates in 
the weakness of the paralyzed muscles and the immoderate tugging of the 
non-paretic ones. We find anomalous and just as characteristic positions and 
deformities in diseases of other peripheral nerves ; thus the ape-hand in median 
neuritis, and the wrist-drop of radial neuritis, etc. We cannot at this point 
discuss these conditions, neither can we now enumerate the special derange- 
ments of innervation which are expressed by paralysis of individual nerves, 
since these are fully considered in another part of this volume. 1 

The paralyzed muscles often show tenderness on pressure, although, cer- 
tainly in localized neuritis, it is not usually prominent. When it appears 
early, even before the paralysis, and is marked, it suggests a coordinate sub- 
stantive disease of the muscles (neuromyositis) . 

The tonus of the paretic muscles is invariably decreased; therefore the 
paralysis is flaccid, and, as is usually the case in other conditions, flaccidity 
of the muscles is combined with an arrest or decided decrease of the tendon 
reflexes. This decrease of the tendon reflexes is, as a rule, an early symptom, 
and is marked even when the paralysis is slight. Occasionally it is the only 
sign of a further extension of the neuritis, as might appear from the paralysis. 
In neuritis of the peroneal nerve the Achilles tendon reflexes alone may be 
absent, although the branches of the tibialis posticus are not wholly intact. 
The decrease or arrest of the knee-jerk may be the only positive sign of impli- 
cation of the crural nerve — provided the power and electric contractility of 
the quadriceps show no conspicuous damage. Absence of the tendon reflexes 
may persist until convalescence and after the disappearance of all other neu- 
ritic symptoms. The reflex may be abolished for years and may never return, 
even though all other functions are restored to the norm. 

But there are exceptional cases. The tendon reflexes may be preserved 
and even increased in neuritis. Moebius and Strumpell first reported this. 
These authors, as well as Sternberg and Dejerine, assume that the cause of 
the phenomenon is neuritic irritation of the sensory fibers of the mixed nerves. 
Such exceptional cases have recently been described also by de Buch, 2 Brissaud 
and Brouardel, and others. Of course an increase of the tendon reflexes may 
be due to paralysis of the antagonistic muscles. At all events a true increase 
of the tendon reflexes in neuritis and polyneuritis is a very unusual phenom- 
enon, which is seen only in mild cases, and always calls for careful diagnostic 
consideration. 

The investigation of the electric contractility is of the greatest diagnostic 
and prognostic importance. Concerning this reaction, I shall mention only a 
few general points, others of special significance being described later. For 
individual conditions I refer the reader to Bernhardt's article in this volume. 

In neuritis we meet with endless variations in the disturbance of the elec- 

i See Bernhardt's article, "Paralysis of Peripheral Nerves," in this volume; also 
Bernhardt, " Die Erkrankung der peripherischen Nerven," 2. Aufl., Wien, 1902. 
2 " Polynevrite et reflexes." Journ, de neurolog., 1901, p. 143. 



746 NEURITIS AND POLYNEURITIS 

trie contractility. In our case there were symptoms of partial DeR ; i. e., a 
decrease of faradic and indirect galvanic excitability, and examination of 
the muscles with the galvanic current showed sluggish contractions ; the quan- 
titative contractility is not decidedly changed in this form of irritation, nor 
is it reduced in a sequence of contractions : cathodal closure contraction — CaCC 
— is greater than anodal closure contraction — AnCC. The earliest disturbances 
of electric contractility, as is well known, appear toward the end of the first 
week. They consist in a decrease of contractility on faradic and indirect 
galvanic irritation; in the course of two or three weeks more, in severe cases 
there is complete arrest of contractility to this form of irritation. In the 
meantime, upon direct galvanic irritation we note a qualitative change in the 
contraction, which becomes sluggish with a slow ascending and slow descend- 
ing motion. The sluggishness of the contraction is the only evidence of the 
DeR inseparably associated with it. Occasionally the contractility in re- 
sponse to the direct galvanic current is increased; often the sequence of con- 
tractions is inverted, and AnCC is more readily evoked than CaCC. When 
this contraction is not sluggish but lightning-like, we cannot refer it to DeR. 
The form here described is absolute DeR (arrest of both faradic and indirect 
galvanic contractility and sluggish contraction with direct galvanic irrita- 
tion). Retained power of contractility to the faradic and indirect galvanic 
currents, although decreased and sluggish with galvanic muscular irritation, 
we call partial DeR. This form was observed in our patient. 

In a diagnostic respect, certainly so far as neuritis is concerned, the dif- 
ferentiation between these two forms (which present many variations) is not 
essential; in a prognostic respect, however, it is of paramount significance. 
The electrodiagnostic finding in this case enables us to assure our patient that 
his paralysis is curable, and that the duration of the disease will probably not 
be more than two or three months. We will subsequently see how the finding 
of complete DeR may modify the prognosis. 

The majority of cases of neuritis are characterized by a qualitative change 
of electric contractility in the form of a partial or total reaction of degenera- 
tion, and, as a rule, the milder forms of paralysis show partial DeR, while the 
most severe forms of complete paralysis often present total DeR. But there 
are cases of neuritic paralysis in which no qualitative changes of electric con- 
tractility can be demonstrated; the derangement (diminution on all forms of 
irritation) is merely quantitative, and, although rarely, there may be no dis- 
turbance of electric contractility. We find this condition in the cases in which 
paralysis is incomplete, these cases having throughout a good prognosis. The 
following fact, the knowledge of which we owe to Erb, is of great diagnostic 
importance : if a point above the presumable interruption of the nerve is stimu- 
lated, no contraction follows, but this appears promptly if the electrode is 
placed over the diseased area. This is illustrated by the following case : 

Case 2. — A young man, after a severe wrenching backward of his right arm, 
noticed weakness in the right hand which in the next few days increased, and was 
accompanied by paresthesia in the area of the ulnar nerve. The paresis in the muscles 
of the hand innervated by the ulnar persisted but was only slight; there was neither 
objective sensory disturbance nor change in the electric contractility. Upon stimu- 
lating the ulnar behind the condyle a distinct effect was produced in the muscles sup- 
plied by this nerve, while no contractions could be evoked by irritation of the fibers 
of the ulnar nerve in the axillary cavity. This proved that an organic change had 



NEURITIS 747 

inhibited conduction in the ulnar nerve, and, from its results and development, this 
change must have been neuritis due to trauma. In traumatic cases in which the opin- 
ion of an expert is desired the importance of such a proof of the interruption of con- 
duction is obvious. 

As in the non-neuritic peripheral paralyses, in neuritis there is frequently 
an incongruence between the functional and the electro-diagnostic findings in 
some stages of the affection. In the stage of regeneration, electric contractility 
may for some time be absent or ma} r be pathologically changed even after the 
function has reappeared — a fact proven by experiment. 

As a final objective symptom in our patient we demonstrated sensory dis- 
turbance, which, as in most cases of neuritis of non-traumatic origin, consisted 
in reduction of sensation for all sensory qualities. This hypesthesia may be 
more or less marked according to the severity of the affection ; it ranges from 
complete anesthesia, which is seldom observed, to slightly decreased sensation. 
Usually all the sensory qualities are simultaneously affected. Partial sensory 
paralysis has been only a few times observed in neuritis. Lahr x compiled a 
number of such cases. The case published by J. B. Charcot proves that dis- 
sociated sensory paralysis may occasionally occur in neuritis, but it is certainly 
exceptional. 

Notwithstanding the appearance of severe, motor, paralytic phenomena, 
sensory symptoms of absence of function are often either slight or wholly 
absent. If these phenomena were observed in true neuritis, we might 
assume that the sensory nerve fibers have a greater power of resistance 
against the causative agent of the disease. Explanations are not wanting, 
and such cases may now and then actually come up for consideration. 
It has been determined experimentally that under gradually increased pres- 
sure the conduction of motor fibers is interrupted sooner than that of the 
sensory (Luderitz) ; an effort was made to account for this by the suppo- 
sition that the sensory nerve fibers are situated in the interior of the 
nerve trunk, and are thus better protected from mechanical disturbance. That 
in many cases a different explanation must be given is taught by the fact 
that, even after the complete severing of mixed nerves, there is either no 
anesthesia or it is restricted to a much smaller area than might be expected 
from the anatomical relations. Amelioration of such a sensory disturbance 
is often very rapid and far exceeds the restoration of the motor function. 
It has been alleged that the sensory cutaneous innervation of such a region 
is performed through manifold anastomoses of the different nerve trunks in 
the periphery. By painstaking investigations Zander proved this for the dor- 
sum of the hand, and he and Frohse working jointly, for the face. 2 For exam- 
ple, Frohse states that in the lateral portions of the face there are many areas 
in which the muscles innervated by the three branches of the trigeminal nerve 
overlap each other, and in other areas there are even branches of the large 
auricular nerve, a branch of the pneumogastric. Anastomoses have also been 
proven between the nerve trunks in the forearm. Other explanations contend 
that fibers of normal cutaneous areas extend into the anesthetic portions; 
some authors (Duvante, for example) have recently assumed a so-called au- 

i Lahr, " Ueber Storungen der Schmerz- und Temperaturempfindung infolge von 
Erkrankungen des Riickenmarks." Arch. f. Psych., Bd. XXVIII, p. 773. 

2 See Fig. 189 (p. 678) in Bernhardt's article, " Paralysis of the Peripheral Nerves.'* 



748 NEURITIS AND POLYNEURITIS 

tochthonous regeneration — i. e., a regeneration of peripheral nerves which 
have no connection with the central organ — to be the cause of this phenomenon. 
No matter what the conditions, there is no doubt of the clinical importance 
of sensory phenomena in the majority of cases of neuritis. 

Although the motor disturbances are as a rule more prominent than the 
sensory, yet there are exceptional cases in which the reverse is true ; therefore 
in which the sensory symptoms prevail. We will later describe such a case. 
There is also a pure sensory neuritis in which only the sensory fibers of mixed 
nerves are affected. Finally we sometimes observe a pure sensory neuritis due 
to the fact that nerves entirely composed of sensory fibers are affected. 

Thus, although rarely, the sensory branch of the trigeminal nerve may be 
the seat of a spontaneous neuritis, as I once had the opportunity of observing. 
Kuner x recently described such a case of neuritis affecting the sensory branch 
of the fifth nerve. The prototype of the second form is meralgia paresthetica, 
described by Bernhardt 2 and Roth, which is a neuritis of the external femoral 
cutaneous nerve. There are but scant reports in literature of other sensory 
forms of mononeuritis. Ballet described a case of neuritis of the greater 
saphenous nerve, Remak one of neuritis of the internal femoral cutaneous. 
Kutner reported two cases, both occurring in alcoholics, in each of whom the 
affection was symmetrical. In one case the superficial peroneal nerve, in the 
other the large saphenous, was affected. In none of these cases was the neuritis 
purely sensory, but it occurred in the larger branches of mixed nerves. 

Compared with the sensory symptoms of absence of function, the peculiari- 
ties of which will be enumerated in other observations, the sensory, objective, 
and irritative phenomena are secondary. Only in traumatic neuritis do we 
often find hyperalgesia of such a nature that irritations not otherwise painful 
will produce pain, and the slightest prick of a pin will cause an indistinctly 
localized, but diffusely distributed and unbearable, burning sensation. This 
hyperalgesia may be located in an area in which sensation is decreased (anes- 
thesia dolorosa). Weir Mitchell, 3 to whom we are indebted for much of our 
knowledge of these neuroses, has pointed out that there is no actual hyperes- 
thesia in the sense of a true increase of cutaneous sensation. 

Etiology. — In our patient the affection can be etiologically traced to influ- 
enza. The case therefore belongs to the great group of infectious and post- 
infectious forms of neuritis, and, since only one nerve was affected, may be 
designated a post-infectious mononeuritis. On attempting to review the sub- 
ject of neuritis and polyneuritis, and to subdivide it, we are confronted with 
difficulties, and in spite of the utmost care it is impossible to make a satisfac- 
tory division which will be uniform from all points of view. We may dis- 
criminate between local and general neuritis (mononeuritis and polyneuritis). 

The majority of cases of mononeuritis are of traumatic origin due to the 
severing, torsion, contusion, or compression of the nerve, and to this form 
belongs the pressure resulting from some occupation (occupation neuritis). 
Among the local forms of neuritis we must also include the local infection of 

i Kuner, " Zur Klinik der sensiblen Mononeuritis." Monatschr. fur Psychiatrie 
und Neurol., 1905, p. 29. 

2 See this volume. 

3 Lectures on " Diseases of the Nervous System, Especially in Women." Second 
Edition, Philadelphia, 1885. 



NEURITIS 749 

a nerve from trauma (wound neuritis), also that in which toxic products have 
effected an entrance and damaged the peripheral nerve (such as injections 
of ether, antipyrin, hyperosmic acid, or corrosive sublimate), also neuritis 
propagated from the immediate surroundings : for instance, a purulent process 
around the nerve may finally generate a local disease of the nerve although 
the nerve for a long time exerts a certain power of resistance. In addition, 
the much discussed ascending neuritis must be included in this group, as 
well as the local neuritis of which our first patient forms an example — local 
infectious or post-infectious neuritis; it must also embrace those cases in 
which, either after refrigeration (rheumatic neuritis) or without a recogniz- 
able cause, a local neuritis develops (idiopathic mononeuritis). The general 
infections and intoxications, whether from exogenous or endogenous poisons, 
occasionally produce a local neuritis. In the alcoholic we find an isolated 
peroneal neuritis, in the diabetic a crural neuritis. Infections as well as in- 
toxications frequently produce a more widely distributed form of the disease 
(polyneuritis), which may also appear as an infectious disease sui generis. 

While the innumerable varieties of neuritis at present prohibit classifica- 
tion, yet the etiology is by no means always uniform. Two or more causes 
may act together to produce the disease. Oppenheim long ago maintained 
this multiplicity of causes. The effects of alcohol may cause an otherwise 
insignificant pressure to interrupt the conducting power of a nerve; tubercu- 
losis, chronic lead poisoning, or other intoxication combined with the dam- 
aging effect of an occupation may cause neuritis, the nature and distribution 
of which depend largely on the latter cause. Thus we may form a conception 
of a toxico-traumatic, toxico-professional, or toxico-traumatico-professional 
neuritis. General disease of the central nervous system from a local cause 
may predispose a person to neuritis, and this is not uncommon in tabes ; here 
no other recognizable cause of neuritis appears. 

Localized neuritis does not implicate merely a single peripheral nerve. If 
the local effect is sufficiently extensive it may attack several nerves in the same 
member, a nerve plexus may be more or less damaged, or infectious and post- 
infectious neuritis may implicate an entire plexus (of which I shall later give 
an example) and thus attack different nerves. Remak * called attention to 
multiple localized neuritis which he designated as mononeuritis multiplex 
or disseminated polyneuritis. It is evident, therefore, that the boundary line 
between local and general neuritis cannot always be sharply defined. 

Localized or general neuritis may occur in some of the acute infectious 
diseases, in influenza, enteric fever, pneumonia, appendicitis, scarlatina, 
measles, whooping-cough, acute articular rheumatism, angina tonsillaris, and 
mumps ; probably no infectious disease can be omitted from this list. In some 
it is common, in others rare; in the majority the nature and distribution 
of the neuritis shows no intimate relation to the preceding infection. An 
exception to this rule is diphtheria, which produces the characteristic patho- 
logic picture of a definite form of polyneuritis. The following illustrations 
of localized neuritis occurring after infectious disease demonstrate the varia- 
tions in the etiology, and furnish other points in the symptomatology of local- 
ized neuritis. 

i L. c, p. 303. 



750 NEURITIS AND POLYNEURITIS 



Case 3. — The case was that of a laborer, aged 40, and was especially interesting 
because of the rare localization of the neuritis. The patient was admitted to the hos- 
pital on the 14th of November, 1901, and remained until the 6th of January, 1902. 
A diagnosis of pneumonia was made. After the severe symptoms of the primary dis- 
ease had abated, the patient complained of pain and weakness in the right arm when 
he attempted to flex it. When he was examined on January 31st, 1902, his condition 
was unchanged, and was as follows : Flexor muscles of the arm flaccid ; on flexing the 
right forearm the supinator longus alone showed powerful contractions, while contrac- 
tion in the biceps and brachialis anticus was very slight. Electric examination revealed 
a typical and conspicuous DeR, which, however, was not quite absolute in the biceps 
and brachialis. The brachial plexus was somewhat sensitive to pressure, and the 
patient complained of numbness and paresthesia on the external side of the forearm 
and external border of the metacarpal bone of the thumb. In this region there was 
also distinct tactile hypesthesia which did not affect the thumb. While he continued 
under observation the symptoms improved, and upon examining him a year later noth- 
ing abnormal was noted. 

Here we find characteristic sensory and motor irritative symptoms and 
those denoting absence of function in a neuritis of the musculo-cutaneous 
nerve, this condition, according to Bernhardt, 1 having so far been observed 
in only 14 cases. The causal affection, pneumonia, has rarely been described 
as an etiologic factor in neuritis. This may be accidental, for I have seen 
several such cases. The favorable prognosis based on the electro-diagnostic 
findings was justified by the subsequent examination. 

Case 4. — A cobbler, aged 35, had suffered six months previously from appendicitis 
in which the temperature rose to 104° F. Even while in bed he had pain in the right 
arm, and he soon noted that he could not elevate it at the shoulder-joint. The greatest 
difficulty was experienced in moving the arm backward. Examination showed marked 
wasting of the right deltoid. The patient could really raise the arm, but the power 
to do so was greatly diminished, and on attempting it there was distinct tension in 
the pectoralis major and the infraspinatus. In the region of the axillary nerve there 
was complete DeR. The patient complained also of numbness in the upper arm on the 
external side of the upper third. Needle pricks were here only slightly painful. No 
objective sensory disturbances appeared. 

Here also the diagnosis was clear. The case was one of neuritis of the 
axillary nerve following a severe febrile attack of perityphlitis. The course 
was unfavorable, inasmuch as the neuritis was followed by atrophy and insuf- 
ficiency of the deltoid, which persisted. I examined the man a few years 
later, and found the same condition of almost complete atrophy and absence 
of function in the deltoid. Nevertheless, even at the first examination, the 
damage in function was not so decided as we might have expected, for the 
patient was able to raise his arm to a horizontal plane. Subsequently the 
power to do this so increased that he was perfectly able to work. In this case, 
as has several times been reported in paralysis of the deltoid, 2 compensatory 
muscles assumed the functions of the deltoid, these being the pectoralis major 
and the infraspinatus. The possibility of this occurrence, which is occasion- 
ally noted in other paralyses, must always be considered if we would avoid 
diagnostic errors. 

Although this patient had suffered but little from the neuritis, yet it is 

i See this volume, p. 707. 

2 Bernhardt, " Erkrankungen der peripheren Nerven." 2. Aufl., p. 404. 






NEURITIS 751 

noteworthy that his condition continued unimproved. The prognosis in neu- 
ritis after acute disease is relatively favorable. In the majority of cases, 
although sometimes only after many months, recovery takes place or there is 
at least marked improvement. Upon what this more or less favorable course 
depends is very difficult to state in the individual case. Of course, the sever- 
ity of the neuritic affection as well as the general constitution and the pre- 
vention of further damage must be considered in prognosis. The results of 
electric examination will chiefly decide the prospects of cure. In those cases 
in which DeR is only partial we may count upon a favorable course. When 
DeR is complete we must expect a long duration of the disease. If complete 
DeR persists for from nine months to a year, if more powerful currents are 
constantly necessary in order to produce contraction, the hope of complete 
recovery or even of great improvement is remote. I previously stated that, 
even when polyneuritis does not develop, post-infectious neuritis may occa- 
sionally implicate several nerves or an entire plexus. Remak x distinguished 
these two groups as amyotrophic plexus neuritis of adults and disseminated 
amyotrophic polyneuritis (mononeuritis multiplex). 

Case 5. — The following case is an example of the first form ; it is that of an 
apprentice, aged 17, who, three months previously, had an attack of acute articular 
rheumatism which lasted a few weeks. According to his report a serum injection was 
given him during this time. Three weeks after defervescence, he felt severe pain in 
the right arm, and a little later weakness. The pain ceased, but the weakness continued 
until he came under observation. The right shoulder was decidedly flattened, and the 
biceps and supinator longus wasted. In the right infraspinatus fossa there was a 
deep groove. Abduction of the arm was impossible, and neither the biceps, supinator 
longus, nor brachialis contracted. Outward rotation of the upper arm was impossible 
(weakness of the infraspinatus). The action of the other muscles was normal. Typical 
DeR was demonstrated in the region of Erb's muscles. 

The plexus in the area of Erb's point was extremely painful. There was 
a hypalgesic zone in the area of distribution of the axillary nerve upon the 
outer side of the upper arm. The supinator phenomenon appeared on the left 
side, but was absent on the right. Therefore the case was one of typical 
Erb's or upper plexus paralysis, in which the sensory as well as motor phe- 
nomena of neuritis, such as painfulness of the nerve on pressure, spontaneous 
pain, and sensory disturbances, are very conspicuous. In addition, motor irri- 
tative phenomena in the form of fibrillary contractions were said to have been 
present at the onset. The post-infectious origin is clear. So far acute articu- 
lar rheumatism has only exceptionally been mentioned as a cause of local 
or general neuritis. 2 Cases of pure mononeuritis have several times been 
attributed to a propagated neuritis. In cases like my own such origin is 
impossible; but, on the other hand, arthritic changes and neuritis may be 
produced by the same cause. 

Such a case was described by Remak, 3 and I saw a similar one in which 
neuritic and arthritic phenomena developed pari passu, I shall later describe 
this case in detail. Whether in this patient the injection of serum had any 
etiologic significance is uncertain, but it is quite possible, for Warrington 4 

i L. c, page 290. 2 Remak, 1. c, p. 533. 3 Remak, 7. c, p. 275. 

* Warrington, The Lancet, 26th September, 1903. 



752 NEURITIS AND POLYNEURITIS 

reported a case in which a prophylactic injection of diphtheria antitoxin was 
followed by urticaria and swelling of the right shoulder with paralysis of the 
deltoid and infraspinatus. We know little of the origin of these post-infec- 
tious paralyses. 

It cannot be assumed that neuritis is caused by specific pathogenic agents. 
On the contrary, all experience leads us to the conclusion that the affection 
is due to abnormal products of metabolism generated by pathogenic agents. 
As is evident from the cases quoted, it is unlikely that the form of the original 
infection has any influence upon the localization or nature of the neuritis, 
although to this there are exceptions; as, for example, in diphtheritic neuritis 
and polyneuritis. Here the poison appears to have a decided affinity for 
special nerves. In mononeuritis and polyneuritis, we can by no means always 
name the infectious agent which produces the neuritis. Occasionally we must 
assume a cause which has fallen into general discredit; that is, refrigeration. 
In one form of localized neuritis— facial paralysis — some authors have re- 
cently again assumed cold to be an important factor. The possibility that 
other forms of neuritis may develop from this cause must always be borne in 
mind, and we must especially take this into account when any portion of the 
affected nerve is superficially situated. Often, however, there is no history 
of chilling although the patients are inclined to attribute the disease to cold. 

Case 6. — I am now treating a woman who at Easter, 1904, was attacked by severe 
pain in the right lower extremity. The pain was chiefly confined to the posterior side 
of the leg, and was frequently combined with numbness and formication. I examined 
her for the first time on August 18th, 1904, and found a typical and severe sciatica, 
but neither sensory nor motor signs of absence of function. The right Achilles tendon 
reflex was normal. Under the ordinary remedies improvement gradually took place, 
and on the 24th of October the patient was discharged as cured. About the middle of 
November a weakness of the right foot appeared, combined with extremely severe pain 
in the sciatic nerve, and some in the crural. Examination revealed paralysis of the 
extensors of the foot and of the toes, the peroneal muscles being apparently normal. 
Tenderness on pressure in all the well-known pressure points of the sciatic nerve and 
its branches, a conspicuous sciatic phenomenon, and distinct hypalgesia in the areas 
of the peroneus profundus, especially at the adjoining borders of the first and second 
toes, were noted. Therefore we were dealing with a partial neuritis which affected 
chiefly the sensory and motor portions of the profundus peroneal. Such affections 
which implicate the peroneal profundus alone have been described as of varying etiology 
(traumatic, Bartel's maternal birth paralysis). While she continued under observa- 
tion atrophy and DeR appeared in the paralyzed muscles. I shall describe the electric 
findings somewhat more minutely than in the previous cases, in order that we may 
correctly appreciate the individual points in the electrical relations. 

Faradic Examination. 
Right. Left. 

Peroneal nerve, 95 mm.D.* to 60 mm., no Peroneal nerve, 105 mm.D. 

contraction in the extensors of the 

feet and toes. 
M. tibial, antic, to 50 mm.D., no con- M. tibial, antic, 95 mm.D. 

traction. 
M. extens. hall., no contraction. M. extens. hall., 95 mm.D. 

M. extens. digit, long., no contraction. M. extens. digit, long., 100 mm.D. 

Extens. digit, brev., no contraction. Extens. digit, brev., 105 mm.D. 

Peroneal muscle, 75 mm.D. Peroneal muscle, 95 mm.D. 

* D. is the distance in millimeters that the secondary coil has been removed from 
the primary. [J. L. S.] 



NEURITIS 753 

Galvanic Examination. 
Right. Left. 

Peroneal nerve with 4 ma.* contracts Peroneal nerve, 2 ma. 

only in the peroneal muscle; with 

15 ma. no contraction in the ex- 
tensors of the feet and toes. 
M. tibial, antic, G ma., CaCC. = 4 ma. M. tibial, antic, 4 ma., CaCC, 8 ma., 

AnCC, contraction sluggish. AnCC, contraction lightning-like. 

Extensor hall, long., 5 ma., CaCC, Extensor hall, long., 4 ma., CaCC. 

AnCC, sluggish contraction. 
M. extens. digit, long., 7 ma., CaCC, M. extens. digit, long., 5 ma., CaCC. 

5 ma., AnCC, contraction sluggish. 
Extens. digit, brev., 4 ma., CaCC. = Extens. digit, brev., 2 ma., CaCC 

AnCC, contraction sluggish. 
Peroneal muscle, 7 CaCC, 5 AnCC, con- Peroneal muscle, 3.5 ma., CaCC 

traction somewhat sluggish. 

* ma. = milliamperes. [J. L. S.] 

Examination demonstrated DeR in the region of the peroneal nerve; this was com- 
plete in the course of the peroneal profundus but only partial in the superficial peroneal. 
Our experience led us to conclude that the partial paresis of the peroneal muscles 
would improve in a few weeks, but that a longer time must elapse before the other 
muscles could completely recover. This prognosis proved to be correct. At the end 
of April, 1905 — therefore after seven months — the power and contractility of the pero- 
neal muscles was normal, but the extensors were still weak and showed partial DeR. 

In point of frequency, among all the causes of mononeuritis trauma occu- 
pies the front rank. If we endeavor to subdivide further the group of diseases 
known as traumatic neuritis, we at once differentiate between neuritis from 
and without external wounds. The first was formerly regarded as very com- 
mon, and was attributed by Weir Mitchell to an injury of the nerve trunks. 
With the introduction of antisepsis and asepsis came a complete change of 
opinion, and we learned that not the traumatic lesion of the nerve in itself, 
but the infection caused by the wound, generated inflammatory disease of the 
nerve. Hence so-called acute neuritis from injury is only very rarely observed. 
The most complete description of the symptoms will be found in Weir 
Mitchell's book, which is chiefly based upon the numerous cases furnished 
by the American Civil War. 1 Besides the local symptoms of pain, paralysis, 
and anesthesia, due to the infectious foundation, fever and mental disturb- 
ances appeared. Motor irritative phenomena, secretory, vasomotor and trophic 
disturbances, which are otherwise rare have often been observed in this variety. 
We designate the neuritis as chronic and subacute if the symptoms at once 
become chronic or gradually assume this form after an acute stage. 

The second variety of traumatic neuritis includes those cases without ex- 
ternal wounds. It is extremely difficult to differentiate these from simple, 
traumatic, non-inflammatory injury to the nerve. It is clear that if a nerve 
— for instance, the radial — is simply severed by a saber cut, the knife of the 
surgeon, or in any other way, and if merely a paralysis of the muscles sup- 
plied by this nerve follows (perhaps hypesthesia in the area of its distribu- 
tion), we may regard it as traumatic nerve paralysis but not as neuritis. Only 

i Weir Mitchell, Morehouse, and Keen : " Gunshot Wounds and Other Injuries of 
Nerves." 

49 



754 NEURITIS AND POLYNEURITIS 

when irritative phenomena are added to the inevitable consequences of nerve 
lesion do we speak of traumatic neuritis (Remak). This standpoint is in 
some respects of little consequence, and, as Remak states, is explained only 
by the historical development of the teaching of neuritis. We do not diag- 
nosticate non-traumatic, post-infectious, or spontaneous neuritis solely from 
the presence of symptoms which indicate more than a mere interruption of 
conduction. In the diagnosis of traumatic neuritis we require more signs 
and symptoms than for the other forms of neuritis, and this demand is unjus- 
tifiable, since no anatomical differentiation between " parenchymatous inflam- 
mation " and " simple degeneration " can be made. In the following descrip- 
tion, in conformity with early usage, the sequel of traumatic destruction in the 
course of the peripheral nerves will be almost excluded. 

Without external wound nerves may be suddenly or gradually damaged 
by various kinds of force : a blow, a contusion, torsion, pressure from dislocated 
fragments of bone, from dislocated bones, and from callus. Maternal neuritis 
ex partu is that paralysis which follows trauma during birth. In traumatic 
neuritis the etiology often varies, for in addition to the exciting trauma there 
may be a predisposing one, such as chronic or acute intoxication, cachexia, etc. 

Case 7. — The patient is a coachman, aged 45, whose appearance shows that he is 
addicted to alcohol, and this he admits. Six weeks ago he fell from a wagon upon 
the left side of his head. Whether or not he made any attempt to protect himself with 
his right arm he does not know ; but there is no external wound, neither a fracture nor 
dislocation. Soon after the accident the patient felt pain in his right arm which 
became numb, and after two weeks gradually increasing weakness developed in the 
right hand, which extended to other parts of the right arm. Severe pain and numb- 
ness persisted, especially in the thumb, and index and middle fingers. On examination 
the movements of the head were free, no change was apparent in the vertebral column, 
not even with the X-rays; the vertebra were insensitive to pressure. The muscles of 
the entire right arm were flaccid, the soft parts of the dorsum of the hand were greatly 
swollen, the volar manus looked as if upholstered, and the right hand felt warmer 
than the left. There was a distinct fibrillary contraction in the right deltoid. Only 
the following movements were possible : pronation and supination as well as adduction 
of the upper arm, and slight flexion of the fingers. There was distinct hypalgesia in 
the region of the right axillary, median, and ulnar nerves. The nerve trunks and 
plexus were sensitive to pressure, the tendon reflexes were abolished, in Erb's muscles 
electricity revealed partial DeR, in the other muscles it was almost complete. Four 
weeks later marked improvement occurred, and Erb's muscles acted as well as some of 
the muscles supplied by the median nerve. 

In this case there can be no question concerning the diagnosis of true 
neuritis; a simple traumatic nerve lesion is excluded by the gradual develop- 
ment and the progress of the affection. We have little information concern- 
ing the accident. It is not likely that the nerves of the right arm were imme- 
diately exposed to injury, but there may have been shock combined with torsion 
of the nerve. Alcoholism must be regarded as a predisposing factor. The 
cases are not rare in which shock, alone or with torsion, is a traumatic factor, 
and in which the symptoms of neuritis gradually develop to their full extent. 
A similar case is described by Redlich. 1 Occasionally a neuritis of the pero- 
neal nerve develops after the so-called " turning of the f oot." In such cases 

i Redlich, " Zur Casuistik der traumatischen Neuritis." Wiener Tclin. Rundschau, 
1902, Nr. 16. 



NEURITIS 755 

we agree with Remak that by a lesion in the surrounding areas and by direct 
injury to the nerve itself the original trauma produces changes which subse- 
quently lead to degenerative paralysis. Such paralysis may also be due to 
trauma which gradually develops and increases, as in so-called callus paral- 
ysis, also in that due to slowly growing tumors. 

Case 8. — This patient met with an accident on the 23rd of May, 1902; in conse- 
quence of the sudden stoppage of a street car in the middle of which he stood and 
supported himself by a strap, he received a heavy blow upon his left hand. The patient 
felt sharp pain in the wrist- joint, which began to swell. It was at first impossible to 
determine whether or not there was a fracture; gradually besides the pain there was 
weakness and numbness in the left hand. I examined the patient a year and a half 
later. He then complained of severe pain in the ulnar side of the hand and a feeling 
of numbness passing upward, also of weakness in the left hand on executing certain 
movements. Examination revealed a typical ulnar paralysis : claw-hand, atrophy of 
the ball of the little finger and of the interossei, with corresponding weakness and 
partial DeR. In the sensory distribution of the ulnar nerves there was slight hypes- 
thesia. Examination with the X-rays disclosed a fracture of the styloid process of 
the ulna which had united and produced an abnormal configuration of the distal end 
of the ulna. As the paralysis came on gradually it must be assumed that it was not 
due to direct injury of the nerve by trauma, but to a dislocation of the styloid process 
■ — that is, to the formation of callus and damage to the nerve by the healing of the 
fracture. 

Here the etiology was purely traumatic — a simple, persistent compression. 
In such a condition it is very difficult to decide whether we are dealing with 
neuritis in a restricted sense or with simple traumatic nerve paralysis. The 
same doubt arises in the relatively common cases in which, after a dislocation, 
especially that of the shoulder-joint, more or less profound plexus paralysis 
at once develops. Here we must assume that the nervous structures are in- 
jured either by direct or indirect force and degeneration follows, although the 
mechanism and the effect of the trauma are by no means clear, even in these 
apparently simple cases. Sometimes the relations between the trauma and 
the damage to the nerve cannot be perceived. The trauma may have occurred 
years or decades previously, and the affection of the nerve may have appeared 
late, following a new trauma in itself insignificant, or in consequence of some 
other deleterious factor, such as an infectious disease or an operation. Cases 
have been described in which during youth there had been a luxation of the 
elbow-joint, and only after decades did ulnar paralysis develop. Weber a re- 
ports a case in which the dislocation dated back twenty-seven years. When 
the patient was compelled for a time to use the left forearm, a gradually 
increasing ulnar paralysis appeared. In a second case reported by the same 
author the patient had in early youth a purulent inflammation of both elbow- 
joints after variola; this healed with deformity of the joints, most marked 
in the right. Thirty years later, and without special cause, formication 
appeared in the right hand with a painful contraction of the fingers — the 
paw-hand; as the irritative symptoms receded ulnar paralysis gradually 
appeared. The patient suffered from migraine. Her occupation was that of 
sewing and washing. Here also other factors aided in the development of the 
neuritic phenomena. 

1 Weber, " Zur Aetiologie peripherer Medianus- and Ulnarislahmungen." Deutsche 
Zettschr. f. Nervenheilk., Bd. XV, p. 181. 



756 NEURITIS AND POLYNEURITIS 

With increasing frequency we have recently noted that cervical ribs must 
be regarded as etiologic factors in neuritic affections of the upper extremities. 1 

Case 9. — A girl, aged 20, reported that for 9 months she had observed weakness 
in the left hand, and had previously had pain in the ulnar side of the arm, radiating 
from the elbow to the hand. She said that the hand always felt cool. There was no 
history of over-use of the hand. The muscles of the ball of the thumb and little finger 
of the left hand, as well as the other small muscles, were emaciated. The function of 
these muscles was almost entirely arrested, even the flexors of the middle and terminal 
phalanges and the pronators failed to act properly; but the muscles supplied by the 
radial nerve were intact. There were no objective sensory disturbances, but the brachial 
plexus was extremely sensitive to pressure. The pain increased when the patient 
raised her arm and stretched it backward. The left hand was usually cooler than the 
right; in the atrophic muscle there was partial DeR. On palpation of the supra- 
clavicular fossa an indistinct abnormal resistance was felt, which was not understood. 
Since all etiologic factors were absent, we were constrained to think of the cervical 
ribs which appeared distinctly in the X-ray picture. 

As this condition is always congenital, another auxiliary factor must be 
held responsible for the later appearance of neuritic phenomena, which, as 
in the above-mentioned cases of late neuritis after luxation, etc., may be due 
to over-exertion, infection, or additional trauma. In this patient we were 
unable to discover any accidental cause; but slight periostitic processes origi- 
nating from these abnormal formations were suggested. We must be cau- 
tious when we refer nervous irritative symptoms and those due to absence 
of function in the upper extremities to congenital cervical ribs; the latter, as 
Oppenheim maintains, are a sign of degeneration, and may appear in many 
general diseases of the nervous system. Oppenheim found them twice in 
gliosis, Levy ? in multiple sclerosis. Therefore we must not regard cervical 
ribs as the cause of nervous phenomena without further investigation. This 
caution is especially important because the treatment of neuritic paralysis due 
to congenital cervical ribs is surgical ; they have often been removed, although 
the operation is technically very difficult ; when the indication is unmistakable, 
it is usually productive of good results. In this patient we advised operation 
for we believed the paralysis to be due to cervical ribs, and no signs of central 
disease could be demonstrated — here syringomyelia, in particular, is suggested. 

We must also consider anesthesia paralyses under traumatic neuritis, and 
it must be emphasized that the trauma itself does not so much come into 
question as the other effects of anesthesia, especially the intoxication. Usu- 
ally one of the upper extremities is paralyzed; at the operation the arm is 
held too tightly or too high, and this produces torsion of the plexus. The 
paralysis may not affect the entire plexus, and it may even be limited to iso- 
lated nerves. The damage may also vary mechanically; there may be pres- 
sure from the head of the humerus on the axillary nerves or pressure from 
the clavicle. Occasionally other factors besides traumatism and intoxication 
must be considered ( Oppenheim 3 does not positively assert the influence 
of the latter, but emphasizes that the complete flaccidity of the muscles favors 
the development of this paralysis). I saw a case in which paresis occurred 

i See Oppenheim, " Lehrbuch," IV. Aufl., p. 448 ; also Seiffer, Berl. Jclin. Wochenschr., 
1904, p. 818; Ascher, ibid., p. 839; Borchardt, Verhandl. d. Berliner medic. Gesellschaft, 
XXXII, p. 432; and others. 

2 Berl. klin. Wochenschr., 1904, p. 840. 3 Oppenheim, " Lehrbuch," p. 443. 



NEURITIS 757 

in the form of Erb's paralysis, and here the anesthesia and abnormal position 
of the hand had lasted a very short time ; operation was performed on account 
of a severe secondary hemorrhage after appendectomy, and it is quite possible 
that the extreme anemia of the nerves thus produced rendered them abnor- 
mally sensitive to slight trauma. At other times chronic cachexia must be 
considered. Anesthesia paralysis of the crural, peroneal, and tibial nerves 
of the lower extremities is more rare than in the upper extremities. Bern- 
hardt x described a case of the latter. An operation was performed under 
anesthesia for prolapse of the uterus; the legs of the patient rested upon 
supports, the pressure falling upon the popliteal space. Paresthesia and paral- 
ysis in the course of the posterior tibial nerve immediately followed. Paralysis 
after the application of the Esmarch bandage is of similar genesis, but in all 
of these cases the prognosis is comparatively favorable. 

The cases of maternal neuritis after parturition 2 represent a special trau- 
matic genesis, and the following case illustrates all of the typical peculiarities. 

Case 10. — A woman, aged 24, the wife of a policeman, came under observation on 
the 6th of December, 1904. This patient, who was otherwise healthy and very strong, 
was attacked two weeks before delivery (which occurred early in November) with 
tearing pain in the right leg and the small of the back. She was a primapara, and 
labor was easy. The puerperal period was normal ; there was no fever, no cachexia. 
Even during the first days of the puerperium and while the pain persisted the patient 
felt a numb sensation, especially in the posterior side of the right leg, and when she 
left her bed nine days later this member was very weak. Examination six weeks after 
delivery revealed the following: continuous pain, numbness, and a cold sensation in 
the right foot. The nerve trunks in the right leg were very sensitive to pressure, and 
there was a well-developed sciatic phenomenon. The right Achilles tendon reflex was 
abolished, the right patella reflex was present, and the tibialis anticus functioned 
freely; but the extensors of the toes were markedly paretic, the peroneal muscles did 
not respond, flexion of the toes was imperfect, but that of the foot was good. In the 
course of the right superficial peroneal nerve there was distinct hypalgesia, while upon 
the external side of the foot in the region of the sural nerve pin pricks were only 
slightly painful. 

ELECTRIC EXAMINATION. 

Fabadic Test. 
Right. Left. 

Peroneal nerve with 100 mm.D., action Peroneal nerve 110. 

upon tibial, antic, with 60, action 

upon extens. digit, comm. and hall., 

but no effect upon peroneal muscle. 
N. tibial, postic. in the popliteal space, N. tibial, postic. 115. 

70 mm.D. 
N. tibial, on the internal malleolus with 

60 mm.D., reaction in the flexor dig. 

comm., not in the flexor hall. 
Muscle tibial, antic. 85 mm.D. Muscle tibial, antic. 100. 

Extens. hall. long. 50 mm.D. Ext. hall. long. 100 mm.D. 

Extens. digit. 50 mm.D. Ext. digit, comm. 100 mm.D. 

Muscle quadric. sur. 50 mm.D. M. quadr. surse 110 mm.D. 

Peroneal muscle, no contraction. Peroneal muscle, 100 mm.D. 

i Bernhardt, " Festschrift fiir v. Leyden," II. Bd. 

2 See Remak, I. c, p. 253 : also Hosslin, " Ueber periphere Schwangerschaftslah- 
mungen." Munchener medic. ^Yochenschr., 1905, p. 636. 



758 NEURITIS AND POLYNEURITIS 



Galvanic Test. 
Bight. Left. 

Peroneal nerve with 6 ma. action upon 3 ma. 

tib. ant.; with 15 ma. no further 

response. 
N. tibial, post, in the popliteal space 3 ma. 

9 ma. 
M. tibial, ant. 10 ma. CaCC, lightning- 5 ma. 

like. 
M. extens. hall. long. 12 ma. CaCC. = 

AnCC, sluggish contraction. 
M. extens. digit, comm. 12 ma. CaCC. = 

AnCC, sluggish contraction. 
M. extens. digit, brev. 6 ma. CaCC, 

AnCC, 8ma., sluggish contraction. 
Peroneal muscle 15 ma. CaCC, sluggish 

contraction. 
M. quadric. surae 15 ma. CaCC, sluggish 

contraction. 
M. flexor, digit, long. 10 ma. CaCC, 

AnCC,. 8 ma., sluggish contraction. 



Everywhere lightning-like contraction. 
CaCC. greater than AnCC. 
" Values between 6 and 10 ma. 



We are therefore dealing with neuritis of the sciatic nerve, which affects 
the branches somewhat irregularly. Muscles innervated by the peroneal nerve 
as well as those supplied by the posterior tibial nerve are affected, and some- 
times the muscles of both areas are exempt. In maternal neuritis it has been 
believed that the distribution of the paralysis follows a certain law, intimately 
dependent upon the etiology of the disease. According to Hiinermann, pres- 
sure paralysis of the nerves of the hip at birth is prone to occur when the 
pelvis is narrow. The majority of cases of obstetric paralysis have followed 
the use of forceps. As Remak * states, in decisive cases pelvic measurements 
have been lacking. Even in the case of one of my own patients I was unable to 
secure the necessary data; but it must be noted that here paralysis appeared 
after a normal delivery, which is somewhat unusual. According to Hosslin 
(I. c.) among 80 deliveries 66 were abnormal, and in 61 of these forceps were 
used. Among the cases in which paralysis occurred during a normal birth 
one is worthy of mention as having occurred in a tabetic. Many of the 
patients were elderly primiparee in whom a severe labor is extremely common. 
In my case the peculiar localization of the paralysis did not conform to our 
original theory; for, according to this, the area of the peroneal nerve is the 
first to be wholly or partially implicated (Htinermann and others). But some 
investigators have described the posterior tibial as being involved. Remak 
believes the statement that the peroneal nerve is exclusively affected in this 
form of paralysis to be an exaggeration not borne out by the actual facts. 
Hosslin declares the implication of the peroneal alone to be extremely rare. 
In another case that I recently saw the type of paralysis differed from that 
described inasmuch as there were severe functional and electrical disturbances 
in the anterior tibial muscle; the other extensors and the peroneal muscles 
were less severely implicated, the muscles of the calves were paretic, their con- 
tractility being only quantitatively diminished, and the Achilles tendon phe- 

i L. c, page 254. 



NEURITIS 759 

nomenon was absent. The predilection for the peroneal area is here distinct. 
An implication of the crural region is usually denoted by neuralgia; paralysis 
of the obturator has also been observed. 

It would lead us too far from our subject to discuss the different hypoth- 
eses, or to explain the predisposition of the peroneal region. In several in- 
stances of non-traumatic sciatic neuritis, degenerative paralysis has specially 
attacked this region, so that these cases seem to follow a general law which 
does not depend wholly upon mechanical conditions. 1 

In my case the first symptoms were observed during pregnancy. These 
consisted of paresthesia and pain in the leg, such as are common in the later 
stages of pregnancy. Marked symptoms often appear during delivery (accord- 
ing to Hosslin 29 times during, and 35 times immediately after the birth). 
Even before the termination of labor profound paralysis may be noted. The 
prognosis is generally good, but is more favorable the more limited the 
paralysis. 

The cases of different genesis developing post partum must be here in- 
cluded; following a phlegmasia alba dolens or an excessive parametritic exu- 
date, paralysis may gradually develop in the course of the sciatic nerve, and 
Hosslin designates this as neuritis puerperalis per contiguitatem; the paral- 
ysis may be readily differentiated by its slow course and incompleteness. The 
chief thing here is to demonstrate the local findings. 

From these forms of neuritis, gravidity or puerperal neuritis must be dif- 
ferentiated; these are usually forms of polyneuritis which will be later de- 
scribed. But their close etiologic relations make it necessary for us briefly 
to consider them. According to Moebius, 2 puerperal neuritis after a simple 
uncomplicated labor is most apt to implicate the terminal branches of the 
median and ulnar nerves. But later observations show that there is frequently 
another localization. Koester 3 described a case in which the pathologic process 
was limited to the axillary and musculo-cutaneous nerves ; Xothnagel reported 
one in which the deltoid, serratus, and pectoral muscles were paretic ; Moebius 
observed one in which there was paralysis of the deltoid, of the supra- and 
infraspinatus, and of the triceps muscles; the facial nerve alone may be 
affected. Those cases in which the puerperal neuritis affects only one nerve 
of the leg are difficult to distinguish from the maternal paralyses which have 
just been described. Since, in the apparently normal labor of our case, a 
traumatic cause could not positively be excluded, the etiology is uncertain. 
The fact that the first signs of neuritis appeared during labor is not proof 
of its nature, for the cause of puerperal neuritis is frequently traced to the 
period of pregnancy. According to Hosslin, of the cases which developed into 
polyneuritis generalisata puerperalis et gravidarum, 36 cases began before, 
and 65 after delivery. 

Puerperal polyneuritis may often be due to pregnancy, especially if this is 
accompanied by excessive vomiting. In its distribution and course it may 
show all the variations which are observed in polyneuritis, and may resemble 

i For many interesting details of the distribution of paralysis in affections of the 
peroneal nerve see the article by Daus, " Zur Pathologie der Peroneuslahmungen." 
Berliner I. D., 1902. 

2 Moebius, " Ueber Neuritis puerperalis." Neurolog. Beitr., IV, 1895, Nr. 25. 

3 Koester, " Ueber puerperale Neuritis." Munchener med. Wochensclir., 1896, Nr. 28. 



760 NEURITIS AND POLYNEURITIS 

Landry's paralysis (as did a case of Eulenburg's) or may simulate post-diph- 
theritic paralysis or alcoholic polyneuritis. 

In severe cases, as in generalized polyneuritis of other etiology, paralytic 
phenomena often develop in the lower extremities, but their localization does 
not conform to the type described by Moebius. When the paralysis ascends, 
the muscles of the trunk and of the cranial nerves may be implicated, as well 
as those of the arms. The facial, the fifth, and the optic nerves, the muscles 
of the larynx, of the pharynx, and of respiration, as well as those of the 
bladder and rectum, are occasionally implicated. Psychical symptoms char- 
acteristic of polyneuritis (Korsakow's syndrome) may be present. Hosslin 
found these symptoms present in 16 among 40 severe cases; of 50, 10 were 
fatal. 

Etiologically an attempt has been made to differentiate two groups, those 
occurring in a febrile puerperal state after abortion, and those following a 
normal pregnanc} r . Since we are ignorant of the underlying cause in either 
form, these distinctions are valueless. But, as in the genesis of polyneuritis, 
the development of a toxic product in the body {autointoxication) appears 
likely. The frequent appearance of polyneuritis in the pregnant who suffer, 
or have suffered, from hyperemesis is remarkable. That in these cases poly- 
neuritis must be attributed to the cachexia caused by hyperemesis is not borne 
out by the facts ; nor is cachexia invariably present in other cases of puerperal 
neuritis. 

Returning to localized neuritis of traumatic genesis I shall describe a few 
forms in which, besides the motor symptoms of absence of function, there are 
others from irritation. The pathologic picture here shows a special implica- 
tion of the sensory fibers, which also occurs in non-traumatic neuritis, as is 
shown by the following brief clinical histories: 

Case 11. — A laborer, aged 57, complained for five weeks of pain and paresthesia 
in the right hand, limited to the ulnar distribution, and increased by rotation of the 
hand. The patient could assign no cause for this condition, although he was a mod- 
erate user of alcohol. The right ulnar nerve was somewhat broader and thicker than 
that of the opposite side. The slightest pressure caused typical paresthesia in the 
area of distribution of the nerve, and its mechanical irritability was also increased. 
There was no claw position of the hand. Separation of the fingers and adduction were 
somewhat impeded, especially in the third and fourth fingers, but there was no decided 
paresis, and only a slight quantitative disturbance of the electric contractility. On 
the contrary, sensory disturbance was very conspicuous. It was wholly confined to the 
ulnar region, and affected all the various sensations, even that of position. In these 
cases, which might well be designated sensory neuritis, I would urge the greatest cau- 
tion in diagnosis. The diagnosis of neuritis cannot be based wholly upon pain on 
pressure; and in differentiating sensory disturbances we must always consider whether 
or not there is hysterical alteration of sensation. If, as in the case just described, this 
alteration is limited to the area of a definite peripheral nerve there can be no doubt 
of the organic and neuritic nature of the affection. 

Case 12. — In this instance sensory irritative phenomena were very prominent. The 
patient was a sailor, aged 30, who, two weeks prior to examination, suffered from an 
accident. He was carrying on his back a heavy sack which began to slide off. In 
order to throw the sack on the floor he had to lift it above his head, and this compelled 
him to bend far forward and to the right. Soon after he noticed a stiffness at the 
nape, and a little later felt pain in the inner side of the left arm, radiating downward. 
Gradually paresthesia appeared in the last three fingers of the left hand. Examina- 
tion revealed the following: the interosseous spaces of the left hand were somewhat 
depressed. There was slight weakness of the interossei and lumbricales which increased 



NEURITIS 761 

from the first; to the fourth finger; the flexor carpi ulnaris showed weakness and 
partial DeR. The pupil and palpebral fissure on the left were somewhat wider than 
on the right. The patient referred his pain to the whole ulnar area of the arm as 
high as the shoulder. Throughout this entire extent he felt a marked sensation of 
cold. Brush contact was only slightly perceptible over the ball and pulp of the little 
finger. There was no hyperalgesia. The paresthesia and pain persisted for about two 
months after the accident. Paresis was slight. Nothing indicated any injury of the 
vertebral column. 

In addition to the prominence of the sensory symptoms this case is inter- 
esting because we are not dealing with disease of a peripheral nerve, but of 
one or two roots: the eighth cervical and first dorsal were implicated. As 
injury of the vertebral column is definitely excluded, we can only assume 
that there was torsion of these roots, the nature of the accident making this 
quite possible. Torsion, in a manner previously described, gradually produced 
neuritis. Such cases are uncommon; often there is profound paralysis, and 
even more frequently we observe the plexus paralysis of which I have already 
given examples. Such a case of root neuritis, although of much greater ex- 
tent, was recently described by Dejerine and Roussy. 1 Bloch 2 mentions a 
case of traumatic torsion neuritis which affected some of the branches of the 
cervical and brachial plexuses, and specially implicated the sensory fibers. But 
this diagnosis of root lesion 3 must be cautiously made, and we must assure 
ourselves that a process starting from the vertebral column or the membranes 
of the cord (which, of course, might readily injure a root) is not masked by 
the apparently substantive disease of the root. 

In descriptions of neuritis little has been said of affections of the skin and 
its adnexa. Traumatic neuritis presents the greatest variety of nutritive 
changes in the skin. There are frequently vasomotor and secretory disturb- 
ances as well as trophic. Investigators have given much attention to a change 
in the composition of the skin called glossy skin, which was thoroughly stud- 
ied by Weir Mitchell. The skin becomes intensely red, then glistening; it is 
louse without creases or hair, and is most sensitive to external irritation. Usu- 
ally the patient complains of an acute sensation of burning and of neuralgic 
pain. Weir Mitchell points out that this cutaneous change is most readily 
produced when there is partial lesion of a nerve which causes continuous irri- 
tation. In this opinion many other investigators as well as I concur. After 
such a partial trauma glossy skin appears with intense sensory irritative 
symptoms ; but, as Remak reports, a total cessation of the function of the nerve 
may follow such changes. In the most severe case of this kind that I ever 
saw traumatic paralysis of the ulnar and median nerves led to the complete 
arrest of nerve conduction. Glossy skin appeared, and, what is rare, the 
growth of the bones was checked; the phalanges were pointed and thin. By 
means of the X-rays it has recently been demonstrated that certain osseous 
changes (osteoporosis) which otherwise elude clinical demonstration are 
common in peripheral neuritis. It had been previously known that after 
severe injuries to the nerves in infancy (birth paralysis) there may be a 
retardation of growth in the affected areas of the member. According to 

1 Dejerine et Roussy, Revue neurologique, 1904, p. 619. 

2 Bloch, Deutsche med. ^Yochenschr., 1902, Nr. 32. 
s See Oppenheim, " Lehrbuch," p. 445. 

50 



762 NEURITIS AND POLYNEURITIS 

Remak 1 glossy skin may possibly be due to direct inflammatory processes 
without neuritic foundation. But if we find such nutritive disturbance lim- 
ited to a definite nerve region, we may feel assured that it is of neuritic nature. 
In addition to glossy skin we sometimes note anomalies in the secretion of 
sweat. That these are directly due to the nerves and not to the distention 
of the blood-vessels is proven by physiology. In neuritis we often find hyper- 
hidrosis, and this is even more common in partial traumatic neuritis than 
in the primary idiopathic form. I shall merely mention the secretion of 
sweat in disease of the peripheral facial nerve (in which a pathologic condition 
is relatively often observed), and diseases of the cervical sympathetic in which 
also there are deviations from the norm. 

Case 13, the history of which I shall now detail, is an illustration of the promi- 
nence of trophic, vasomotor, and secretory disturbances in traumatic neuritis; as a 
rule, sensory disturbances are also markedly prominent, while the motor are less so. 
Four years after the death of her husband the patient, who is of decidedly neurotic 
temperament, attempted suicide by cutting the arteries of her left hand. Since then 
she has had severe but indistinctly distributed pain in the left hand, with signs of 
disturbance in nutrition, of sensation, and of motility which will now be described. 
Immediately above the wrist is a transverse red cicatrix. The third and fourth fingers 
are flexed at the first and second interphalangeal joints, and at the second inter- 
phalangeal joint it is impossible, even passively, to straighten the fingers. There is 
also a fixed contracture in the interphalangeal joint of the thumb. The second, third, 
and fourth fingers are bluish red, the skin over these areas is smooth and glistening 
but quite cool, and the nails of the first, second, and third fingers are said to have 
repeatedly fallen out. They are now abnormally curved, claw-like, and show longi- 
tudinal striae. The ball of the thumb is atrophic. The cicatrix is still painful on 
pressure. The patient complains of numbness in the thumb and first two fingers, and 
on the inner side of the third. Brush contact is not distinctly perceptible on the 
internal surface of the first, second, and third fingers. There are radiating pains in 
these regions, also hypalgesia followed by a subsequent burning sensation. On oppo- 
sition, the power of the thumb is diminished, but that of the small muscles of the 
hand is normal. Extension of the terminal phalanges, in so far as is mechanically 
possible, can be carried out, as well as flexion of the middle and terminal phalanges. 
In the ball of the thumb there is partial DeR. 

The case was diagnosticated as follows : traumatic neuritis of the left 
median nerve with mechanical sequels and essential sensory and trophic dis- 
turbances. A fine discrimination of these cases may be very difficult, since 
in addition to the neuritic phenomena there may be mechanical sequels, as 
in the preceding cases. The latter may be directly due to trauma or to the 
succeeding inactivity which leads to fixation of the injured extremity; this 
fixation is frequently caused by the firmness of the bandage applied {ischemic 
muscular paralysis and muscular contracture). We shall soon revert to this. 
In the case just described the strict limitation of the sensory disturbance, the 
atrophy, and especially the DeR, to the muscles of the hand supplied by the 
median nerve renders the diagnosis of neuritis certain. In all similar cases 
these symptoms form the corner-stone of the diagnosis, and the proof of con- 
spicuous DeR will always stamp the atrophy as neuritic, and distinguish it 
from muscular atrophy due to arthritis. The same diagnostic factor renders 
us valuable service when there are ischemic muscular changes. Among the 
trophic disturbances in this case we note the typical glossy skin, as well as 

i L. c, p. 133. 



NEURITIS 763 

changes in the nails which are commonly observed in this form of the disease. 
The nails are thickened, are bent like a talon, often friable, and sensitive 
to cutting; shedding of the nails, mentioned in our case, has been several 
times reported. The trophic disturbances may reveal other characteristics, 
such as ichthyotic change in the epidermis, which has been repeatedly observed. 
Eemak mentioned it in a case of puerperal perineuritis of the median nerve. 
Lowenfeld described a so-called neuritic flat-hand, the chief feature of which 
was hypertrophy of the subcutaneous cellular tissue. Edematous infiltration 
has also been reported as a symptom of neuritis. True edema of the dorsum 
of the hand may be due to the fact that in consequence of paralysis the hand 
always droops. But occasionally the unusual intensity of this symptom, 
coupled with its rapid appearance, leads us to assume that other causal and 
non-mechanical factors are operative. This symptom is also found in other 
areas which do not become permanently immobile, and are not subject to 
gravity; we find it in some forms of polyneuritis, particularly in beri-beri, 
localized in the areas of the peripheral nerves. Consequently there can be 
no doubt that disturbances in the innervation of the blood and lymph-vessels 
may, at least in part, account for the development of such edema, which must 
be included among the vasomotor trophic disturbances of neuritis. 

Among these symptoms a few must be described and considered in detail. 
First among them is herpes zoster. The combination of herpes zoster with 
neuritis is seldom met with. More frequently we find herpes zoster of the 
neck and head associated with neuritic paralysis of the facial nerve. Eich- 
horst x has compiled statistics which show that zoster occipito-collaris appear- 
ing with pain is the most common form. In this an eruption appears in the 
area of the third and fourth cervical roots (the region of the occiput, ear, 
and anterior throat region). After two to four days, occasionally later, 
even after weeks, peripheral facial paralysis appears on the same side. An 
inverse sequence — first facial paralysis and then herpes — is exceedingly rare, 
and the zoster eruption is then much less extensive than in the former condi- 
tion. Up to this time but few cases have been reported of herpes zoster and 
neuritic paralysis of the extremity; nevertheless they do appear. Remak 
compiled Eulenburg's cases of this kind occurring since the year 1867, as 
well as one of his own, also cases of Hardy's and Joffroy's. In Remak's case, 
simultaneously or a few days after the eruption of zoster femoralis in the 
region of the crural nerve, paralysis of this nerve developed, and lasted about 
five months longer than the accompanying zoster. Considering the relative 
rarity of this form of the disease, I shall report a case of my own which 
belongs in the same category. 

Case 14. — A man, aged 63, was examined upon March 1st, 1904. He reported that 
on the 4th of January of that year he was attacked by an acute illness with feyer and 
seyere pain in the right shoulder. The next morning there was a profuse eruption of 
small vesicles filled with a transparent fluid; these were first noted on the occiput, 
and extended to the ear. Shortly afterward a similar rash appeared oyer the entire 
right shoulder and right arm, especially the external surface. The side of the thorax 
adjacent to the arm was covered with the eruption. The pain was said to have been 
intense. Now there is merely burning and formication. The vesicles, a few of which 
gradually turned black, disappeared in two weeks. While the patient previously had 

i See Remak, 7. c, p. 143. 



764 NEURITIS AND POLYNEURITIS 

perfect use of his right arm, on the appearance of the eruption he noticed that he 
could no longer move it at the shoulder- or elbow- joint. In the next 2 weeks this 
disability had so increased that the patient could no longer make a fist with his hand, 
which was formerly quite possible. There was no difficulty in micturition. Upon exami- 
nation a number of lentil-sized dark brown areas were found in the supraclavicular 
fossa, at the nape, and extending to the third dorsal spinal process, also on the upper 
arm, the entire external and posterior aspect of the lower arm, and in the radial por- 
tion of the hand. Pressure on the plexus caused radiating pain, and the radial nerve 
also was sensitive to pressure. There was neither hypesthesia nor hyperesthesia to 
brush contact, but a conspicuous hyperesthesia to needle pricks, especially on the 
external side of the arm. The right shoulder was flattened; there were no tendon 
phenomena in the right arm. The response in Erb's muscles was imperfect, the deltoid 
being even inactive. The muscles supplied by the radial and ulnar nerves were func- 
tionally intact. On the other hand, the long flexors of the fingers, the radial flexors 
of the hand, and the apposition of the thumb were weak. Faradic contractility was 
diminished in the deltoid, less so in the supinator longus and biceps. Contraction upon 
galvanic stimulation was distinctly slowed in these muscles, as well as in those sup- 
plied by the median nerve. 

The characteristic reports of the patient, as well as the examination, 
made it plain that herpes zoster in the region of the right cervical plexus had 
at once been followed by Erb's paralysis. It is noteworthy that while the 
herpes zoster receded in the usual way, yet even six weeks after its onset 
new eruptions appeared with the same paralytic phenomena; this showed the 
implication of another root in the plexus. I know nothing further of the 
case as the patient did not return for treatment. 

This combination of positive neuritic paralysis with herpes zoster makes a 
neuritic origin seem plausible in the pathogenesis of herpes. We know that 
the discussion of the foundation of herpes has led to many controversies. 
Certain investigations of Barensprung indicated that it might depend upon a 
disease of the spinal ganglia. This view was confirmed by researches which 
Head and Campbell x instituted upon a broad anatomico-physiologic basis. 
But a few other anatomical investigations have shown that, even with an 
intact spinal ganglion, peripheral neuritis may lead to herpes zoster. I cannot 
minutely discuss the development of herpes. Whether in a case like that just 
quoted herpes zoster may be regarded as a purely neurotic symptom is difficult 
to decide. I believe it must be admitted that the same cause may here produce 
on the one hand a disease of the peripheral nerve, especially of its motor por- 
tion , and on the other hand a disease of the sensory conduction tract, without 
our coming to any decision as to whether the latter is seated in the spinal gan- 
glion or in the fibers which pass from it. 

Among trophic disturbances is perforating ulcer of the foot (mal per- 
forant) — an ulcer which forms on the sole of the foot and penetrates deeply 
in funnel shape; it has thickened edges, aften develops painlessly, and is 
exceedingly slow in healing. This ulcer has been repeatedly observed follow- 
ing injuries to nerves, especially to the sciatic, also after compression of this 
nerve by a tumor, while it has never been demonstrated with certainty 2 in 
idiopathic neuritis, whether mononeuritis or polyneuritis. An exception to 

i Head and Campbell, " The Pathology of Herpes Zoster and its Bearing on Sensory 
Localization," Brain, 1900, p. 351. 

2 Adrian, " Das Mal perforant." Jena, 1904, Gustav Fischer. 



NEURITIS 765 

this rule is the formation of a perforating ulcer of the foot in leprous neuritis ; 
the appearance of ulcer in diabetic neuritis can probably be otherwise ex- 
plained. In the development of this trophic disturbance we must consider 
another factor than a change in the nerve influence, and this is the effect 
of pressure, but only upon tissue whose power of resistance is damaged by 
nervous or other influence. As Remak maintains, mal perforant is of decided 
clinical importance in neuritis and polyneuritis, but not as an exclusive symp- 
tom of clinical mononeuritis of the sciatic, and it is still rarer in the case 
of polyneuritis. 

The same considerations apply to bed-sores. Changes in peripheral nerves 
in the area surrounding a bed-sore have often been demonstrated (Dejerine 
and Leloir). 1 Although the causal significance of this change is still doubt- 
ful, certainly bed-sores have not been observed as a clinical symptom of neu- 
ritis, and the same may be said of other trophic disturbances, especially gan- 
grene. Many cases have been reported in literature, especially in connection 
with the investigations of Pitres and Vaillard, but none has proven that gan- 
grene depends upon neuritis; 2 there is still less justification for regarding 
certain peculiar forms of gangrene, particularly Raynaud's gangrene, as symp- 
tomatic of neuritis. How far other symptom complexes which belong to vaso- 
motor trophic neuroses, particularly erythro melalgia, asphyxia, and local 
syncope, depend upon neuritic changes cannot here be stated. It is certain- 
that these disturbances play no role in ordinary neuritis ; but it is also prob- 
able that some cases which may be grouped with ervthromelalgia, and in 
which the symptoms are confined to certain peripheral nerve regions, are due 
to disease of portions of special peripheral nerve fibers. 3 

There are cases which are most obscure because, in addition to the symp- 
toms of traumatic neuritis, there are other pathologic phenomena which can 
be referred neither to trauma nor to the treatment which the injured extrem- 
ity has received. If the trauma was fracture of a bone, the treatment of which 
is usually absolute rest of the member and a firm bandage, there may have 
been simultaneously torsion of a nerve; if a growing callus subsequently com- 
presses the nerve, on removing the bandage we often find not merely the signs 
of a neuritic affection, but also those of ischemic muscular paralysis and mus- 
cular contracture. At first there is usually severe pain, the hand which re- 
mains free becomes swollen, and the fingers are implicated ; then the muscles 
rapidly atrophy, become hard as wood and rigid, and the affected parts assume 
the fixed position of contracture. Electric examination reveals only quanti- 
tative changes in the contractility. There are no sensory disturbances. These 
symptoms enable us to distinguish ischemic muscular paralysis from the neu- 
ritic form. When these forms are combined, in which case ankylosis accom- 
panied by muscular atrophy and cicatricial adhesions of muscles and tendons 
may appear, the pathologic picture may be disguised. I have most frequently 
seen this combination in fractures of the elbow-joint in young persons. 

Case 15. — A child, 11 years old, suffered a year previously from fracture of the 
right arm involving the elbow-joint. After removal of the splint, severe disturbances 
in the motility of the right arm were noted. Only the movements of the shoulder were 

i Cassirer, " Die vasomotorisch-trophischen Neurosen," p. 89. 
2 Cassirer, I. c, p. 312. 3 Cassirer, I. c, p. 186. 



766 NEURITIS AND POLYNEURITIS 

both actively and passively free. Pronation and supination were mechanically limited 
in the elbow- joint, and the wrist- joint was almost absolutely rigid in a flexure posi- 
tion. The right hand and forearm were cooler than the left and cyanotic ; the muscles 
were firm and hard. The finger-joints showed many ankyloses. That the changes were 
not wholly arthritic and ischemic was proven by the fact that the muscles of the ball 
of the thumb were free, while the joints acted imperfectly, and there was partial DeR; 
a disturbance of sensation limited to the region of the median nerve was also demon- 
strated. The circumstance that, in addition to all other lesions, some area of the 
nerve itself may be damaged is naturally of great significance in the treatment, but 
I shall discuss the therapy of neuritis later. 

Another form of neuritis has often been described ; but, while its occur- 
rence is not actually doubted, it must be regarded as extremely rare; I refer 
to ascending neuritis. Ever since the time of R. Remak, we have understood 
by the designation " neuritis ascendens " those neuritic symptoms which pro- 
ceed from a primary and distally situated disease of a nerve and which ex- 
tend upward, and may be referred to perineuritis or neuritis interstitialis. 
This was said to be distributed, not in the path of the nerve, but irregularly, 
even to other nerves and to the spinal cord. Our experience with traumatic 
neuritis showed this to be based chiefly upon the palpable swelling of the 
nerve found in such cases. The term ascending neuritis was by no means 
limited to traumatic cases, but was given a much wider application. Later 
researches have thrown discredit upon this teaching, and in 1895 Moebius 
pronounced it traditional. But in this he seems to have gone too far. True, 
many of the assumptions of our earlier conception were untenable, but it has 
been experimentally demonstrated that in purulent neuritis it is possible 
for the process to ascend from the periphery to the center. Homen and 
Laitinen have proven that streptococci and their toxins introduced into the 
nerve sheaths reach the spinal cord in the tract of the nerve and spinal cord 
roots, and this coincides with the results of the early researches of Rosenbach, 
Treub, and Kast. Marinesco 1 reported a notable case which led him to simi- 
lar conclusions. Here there was gangrene of the right leg, and just where 
the internal popliteal nerve was embedded in these gangrenous foci numerous 
streptococci were found. A marked infiltration of leukocytes was demon- 
strated in the central portion of the sciatic nerve, and was most distinct in 
the right side of the spinal cord in the posterior external vessels of the ante- 
rior gray substance. In his opinion the microbes generated in the nerve a 
substance which was disseminated along the nerves as far as the spinal cord, 
where it produced anatomical changes in the vessels and nerve cells. There- 
fore, this was an ascending neuritis in the true sense of the word. Recently 
other authors have reported cases in which a neuritis proceeding centrally 
was assumed to be present. Remak 2 published such a case, and this was also 
seen by Oppenheim. The patient had cut his index finger, and urine had 
been applied to the wound. Pain soon appeared, and extended to the shoul- 
der; two weeks later Oppenheim found a paralysis of the radial nerve in the 
corresponding arm, and three months later when examined by Remak the 
biceps also was paralyzed. Kausch, Gerhardt, and Krehl have described 

i Marinesco, " Contributions a. l'etude de la nevrite ascendante." La Presse medi- 
cate, 23. November, 1898. 
2 Remak, I. c, p. 265. 



NEURITIS 767 

similar cases. Redlich 1 also demonstrated ascending neuritis in one of his 
cases. Here, after a fall upon the right elbow, there was paralysis in the 
region of the median and ulnar nerves, while all of the muscles of the arm 
with the exception of Erb's muscles were subsequently attacked. In this 
instance one factor was absent which was present in all of the other cases — 
i. e., there was no open wound — while in the others the process originated in 
an open and, moreover, infected wound. It is certain that the diagnosis of 
ascending neuritis can be made only after most careful investigation and con- 
sideration. At most it is an extremely rare affection. Special diagnostic care 
is necessary in the cases in which sensory symptoms alone follow the trauma ; 
as when there is merely sensitiveness of the nerve to pressure and hypesthesia. 
Here we must always consider whether or not the symptoms are due to trau- 
matic hysteria, and it is astonishing how often this diagnosis will be found 
correct. 

Another uncommon form of traumatic neuritis is inflammation of the 
nerve from the subcutaneous injection of ether. This is usually due to a 
careless or two deep injection through failing to pinch up a fold of the skin, 
hence the ether penetrates the fascia. In the arm it always produces paral- 
ysis of the radial nerve, while, as a rule, the supinator longus and the exten- 
sor carpi radialis longus are unimpaired. The other muscles supplied by the 
radial nerve are affected differently. Pain and objective sensory disturb- 
ances rarely appear. The paralysis is only moderate, there is partial DeR, 
and in the overwhelming majority of cases recovery takes place in one or 
two months. Neuritis has occasionally been observed after the injection of 
other substances, such as chloroform, liyperosmic acid, corrosive sublimate, 
calomel, biniodid of mercury, etc. The cases due to the last drug are caused 
by injections in the gluteal regions, and the paralyses affect the sciatic nerve 
or its branches ; therefore we should be most careful when giving these injec- 
tions. The pathogenesis is here readily understood ; the nerve is directly altered 
by the injected chemical agent. In a pathogenetic sense these cases must be 
distinguished from the previous forms of traumatic neuritis. 

Occupation Neuritis. — The cases which we designate as occupation neu- 
ritis (professional paresis) occupy a special position. We apply this term to 
all those injuries of the peripheral nervous system which are caused by a 
certain occupation, and in which we can demonstrate other typical symptoms 
of neuritis. Among these we first note degenerative atrophic paralysis to 
which are often added sensory, and occasionally also vasomotor, secretory, and 
trophic irritative symptoms, and those due to absence of function. Of course 
we cannot deny that there is a purely sensory occupation neuritis, since this 
finds its analogy in a sensory form of other localized forms of neuritis. But 
we must approach this subject with great circumspection, for its differentia- 
tion from neuralgic and occupation neurosis is most perplexing. We know 
that exhaustive labor leads to derangement of those parts which are exces- 
sively used. But the affections designated as occupation neurosis I shall con- 
sider in a special chapter. 

In the development of occupation paresis, various and by no means simple 

i Redlich, " Zur Kasuistik der traumatischen Neuritis." Wiener Jclin. Rundschau, 
1902, Nr. 16, 



768 NEURITIS AND POLYNEURITIS 

factors are operative. I consider these in connection with traumatic neuritis 
because the pressure exerted upon a nerve trunk or its branches is frequently 
of pathogenetic importance. We have learned to recognize the damage wrought 
by continuous pressure. To these pressure paralyses or forms of neuritis due 
to pressure, to compression by a fragment of bone, by a callus, or by an Es- 
march bandage too tightly applied, we may add others which, in their mode 
of development, form a transitional stage between the first mentioned forms 
and occupation paresis. Here I must mention the so-called crutch paralysis 
— paresis due to the pressure of a crutch in the axilla — which implicates the 
triceps and causes a decided radial paralysis. 

Pressure upon the terminal twigs of the median and ulnar nerves suffi- 
cient to produce paralysis may be caused by an improper cane carried in the 
hand and used as a support. Paralysis of the serratus, due to the bearing of 
loads upon the shoulders, as in carpenters who carry beams, in butchers who 
lift heavy meat, and in other workers who move sacks, is more closely related 
to occupation pareses. A number of similar localized paralyses particularly 
involve the brachial plexus. Rieder (after Remak) 1 described as hod-carrier's 
paralysis a plexus paralysis particularly involving the axillary and radial 
nerves ; this is due to the pressure of the hod which rests upon the clavicle, and 
is most common on the left side ; in young laborers the clavicle is still yielding, 
and compresses the brachial plexus. Coal-heaver's paralysis, described by 
Osann, is of similar origin, as well as the paralysis which Curschmann 2 re- 
ported in a man who carried trees upon his shoulders. On the left side it 
affected Erb's muscle; on the right side only the deltoid. I recently saw in a 
wood-carrier paralysis which affected chiefly the left radial region. The acute, 
almost apoplectiform, appearance of the paralysis and its course tending 
toward improvement, which was very slow, were noteworthy. But in these 
cases, besides the mechanical factor we must often include a toxic one in 
the form of chronic alcoholism. In a somewhat restricted sense, over-exertion 
plays the chief role in professional pareses. How this is brought about, 
Edinger attempted to explain by his substitution theory. There can be no 
doubt that in the activity of the nervous system processes of metabolism occur 
in which definite substances are utilized, and these accordingly require renewal. 
In the form of activity designated as exercise, this renewal is normally suffi- 
cient, and the affected parts are strengthened or undergo higher development. 
In over-exertion, this substitution is insufficient, and decomposition takes place 
in the peripheral nerves, and affects the medullary sheaths and the axis cylin- 
ders. As confirmation of his views, Edinger mentions the fact that those 
persons who suffer from occupation paresis are from one cause or another 
under-nourished. Undoubtedly we must agree with him that, besides over- 
exertion, other factors are frequently operative in the development of this 
paralysis, some of which we have already mentioned, and which will be more 
clearly understood from a few illustrations. This explains why comparatively 
so few persons engaged in the kinds of work spoken of are attacked by occu- 
pation paresis. These examples also show us in how far other factors, besides 

i L. c, p. 282. 

2 Curschmann, " Beitrage zur Lehre von der Beschaftigungsneuritis.'* Deutsche 
med. Wochenschr., 1905, pp. 570 und 634. 



NEURITIS 769 

injurious pressure, are responsible for the development of the pareses. In 
regard to the localization of these affections, of course the muscles of the 
hand supplied by the median and ulnar nerves are most frequently affected, 
but the location varies, and only the cerebral nerves have so far been exempt. 

Case 16. — The following case is calculated to reveal the most important etiologic 
and symptomatologic phenomena of professional pareses. It is the case of a woman, 
aged 25 and very pale., who was employed in the manufacture of dress trimmings. 
She was obliged to hold a round wooden block in the hollow of the left hand, grasping 
it tightly with her fingers. At the same time the hand had to be flexed at the wrist- 
joint — that is, she had to bend the wrist frequently. She worked in this way 12 hours 
daily for 8 years. For 4 years she suffered from tearing pain in the left arm, and in 
the last 6 months there was weakness and increasing emaciation in the left hand. 
Five weeks ago she stopped work, but there has been no improvement. On examination 
degenerative paralysis was found in all of the small muscles of the hand, which were 
decidedly emaciated, and showed partial DeR. The plexus was not painful on pres- 
sure, but the ulnar and median nerves were sensitive to pressure at the elbow and the 
wrist-joint. Pressure upon the muscles of the vola manus was particularly painful, 
and upon passive extension this pain radiated to the hand and fingers. In the ulnar 
region of the hand, particularly in the little finger and its ball, there was hypesthesia 
for tact and pin pricks. Notwithstanding the fact that the patient had long since 
discontinued her work, there was no improvement in the paresis, but the pain had 
lessened. After about 6 months, the paralysis began to yield. The patient did not 
continue under observation. 



We note here that pressure and excessive exertion on the part of a poorly 
nourished and anemic person combined to produce occupation paralysis. Paral- 
ysis is now the most prominent symptom, but pain had for years preceded, as 
has often been observed by other authors, so that it may be regarded as char- 
acteristic of these cases. In addition to the pain there were objective sensory 
disturbances and tenderness of the nerve trunks on pressure. The fact is also 
remarkable that pressure upon the vola manus was painful, and although this 
does not actually prove the existence of a more or less substantive myositis 
(neuromyositis) in addition to the neuritis, yet many experiences seem to indi- 
cate that myositic as well as neuritic symptoms appear in this disease; and 
when we consider the nature of the damage sustained, it appears quite plausible. 
There is a form of occupation paralysis, recently reported, in which the proc- 
esses seem to run their course in the muscles or tendon sheaths; this is the 
drummer's paralysis described by Bruns and Zander, and usually affecting the 
extensor pollicis longus, more rarely the flexor pollicis longus. 

A pathogenesis similar to that in the history just detailed is found in the 
common paralysis of laundresses, which manifests itself by atrophy and weak- 
ness of the muscles in the ball of the thumb, of the adductor pollicis, and of 
the interosseous primus. In addition to excessive use, these are attributed to 
pressure in grasping the laundry iron. Similar neuroses are found in many 
other occupations — for example, in gold burnishers, in carpenters, in smiths, 
etc. I saw a similar picture in a man, aged 38, who for 24 years had worked 
8 or 9 hours a day at stamping letters, while for 6 years previously he had 
sorted letters; a somewhat similar form of occupation paralysis has been de- 
scribed in letter sorters. This patient had suffered for years from pain which 
radiated extensively, also paresthesia and slight object sensory disturb- 
ances. 



770 NEURITIS AND POLYNEURITIS 

Case 17. — I shall here describe a form of paralysis limited to the distribution of 
the median nerve, and it is in many respects interesting. It is the case of a tailor, 
aged 37, who, upon the 26th of August, 1902, sustained a knife cut in the left fore- 
arm. The blood loss was insignificant. On the morning of the 17th of September, he 
was unable to flex the first three fingers of the right hand, whereas during the night, 
while asleep, as was his habit, he had placed the arm under his head. As a cutter 
he was obliged to use heavy scissors with his right hand. There was slight median 
paresis, with distinct objective sensory disturbances and slight electrical changes. The 
paralysis disappeared 3 months after stopping his work. Nine months later he was 
again suffering from radial paralysis which appeared suddenly, apparently from torsion. 
From this also he recovered. 

We notice how many etiologic factors must be considered in this case, for, 
besides loss of blood and consequent anemia, pressure on his arm during sleep, 
over-exertion, and pressure from the heavy scissors were also operative. It is 
remarkable, too, that after a brief time another nerve became paralyzed after 
a slight injury; this indicates a lessened power of resistance in his peripheral 
nervous system. 

Remak saw a case in some respects analogous. A lathe- worker, who worked 
at a plane, and used alcohol in moderation, had a bilateral ulnar paralysis 
which disappeared 4 months after he stopped his work. After a year and a 
half he returned with a right-sided median neuritis due to the continuous use 
of a brush, and from this also he recovered. Special etiologic factors were 
noted in the next case. 

Case 18 is that of a laundress, aged 22. In her youth, this patient had suffered 
from a traumatic peripheral paralysis of the left ulnar nerve, and 3 weeks after 
beginning her present work a median neuritis appeared. The sudden onset of this 
hyperexertion neuritis is explained by the fact that in consequence of the preceding 
paresis the patient was compelled to over-exert the small muscles of the hand. 

Case 19. — A somewhat unusual cause was noted in the case of a woman, aged 49, 
who presented bilateral atrophy in the distribution of the median nerve. She had 
been obliged frequently to lift heavy pots. She admitted having consumed a great 
deal of coffee, but denied the immoderate use of alcohol. Here the excessive use of 
coffee may have caused the paralysis. 

It has been reiterated that chronic intoxication is a common cause of this 
affection, and it is proven by numerous cases. Besides chronic alcoholism and 
nicotinism, chronic occupation intoxication — for example, with lead in paper 
glazers — has been mentioned. Other predisposing factors are the following: 
Chronic diseases which lower the general nutrition; at other times a lessened 
power of resistance of the nervous system, especially the peripheral portions. 
Tabes has been repeatedly noted as a cause. I once treated a tabetic tailor for 
occupation paralysis. Remak mentions tabetic cigar-makers; Oppenheim re- 
ports the case of a patient in whom, during convalescence from influenza and 
when she undertook to carve wood, atrophy appeared in the small muscles 
of the hand. Sano 1 reported the case of a painter, who held his palette in 
his left hand, and who, during the course of scarlet fever, became paralyzed. 
The muscles most used in his work — on the right side the muscles of the 
shoulder-blade, particularly the serratus anticus and deltoid, and on the left 
the biceps — were affected. Perhaps lead poisoning, to which his work exposed 

i Journ. d. Neurol., 5. Mars, 1898. 



NEURITIS 771 

him, was also operative. Oppenheim believes that patients who have had 
acute anterior poliomyelitis in childhood are specially predisposed to occupa- 
tion paralysis. He treated a man suffering from poliomyelitic paralysis of the 
i-ight leg who supported himself with a cane carried in the right hand, and 
who presented an atrophic paralysis of the muscles of this hand; under rest 
of the muscles and electricity it improved. 

In such patients symptoms of general nervousness are often present, and 
occasionally even the marked stigmata of a hereditary neurotic diathesis. To 
this I attribute a marked kyphoscoliosis which I observed in one of my 
patients, a seamstress, who suffered from occupation paresis, the same deform- 
ity being presented by several other members of her family. Remak states 
that he has found paralysis to be very common in persons with this deformity. 
Possibly improper technic may be responsible for these disturbances ; in young 
girls chlorosis and anemia are frequent accompaniments. Hence it is evi- 
dent that the etiologic secondary factors in the development of occupation 
pareses are manifold. 

Symptoms. — Following this discussion of the etiology, I return to the 
symptomatology of occupation pareses. Of course I cannot enumerate all 
those callings in which pareses follow the excessive use of the small muscles 
of the hand. In those mentioned, pressure as well as over-exertion led to the 
paralysis. In a case of Curschmann's, the patient worked on metal clocks, and 
combined with pressure and over-use of the muscles shock from continuous 
light blows on the surface of the hand produced paresis of the left ulnar 
nerve. In other cases no cause was apparent save excessive use; for instance, 
the pareses of seamstresses, embroiderers, cigar-makers, and diamond-cutters. 
In cigar-makers, as Coester first pointed out, paresis of the median nerve 
may also implicate the ulnar nerve. Pains often long precede, as well as 
vasomotor symptoms, especially numbness of the finger. In cigar-makers I 
have frequently observed this local syncope combined with acroparesthesia and 
pain, but without paralysis. The chief cause of the vasomotor symptoms 
seems to be the fact that these patients are compelled to work with wet, 
cold fingers. When paralytic symptoms and objective sensory disturbances 
peripherally distributed are lacking, it is doubtful whether we are justified 
in assuming a neuritic basis for the affection. But the discussion of this 
question would include acroparesthesia in general, upon which I cannot here 
touch. 1 

It is well to bear in mind that acroparesthesia is occasionally merely a 
preliminary stage of subsequent and true degenerative paralysis and neuritis. 
This has been described by Raymond and Courtellemont in a coachman, 2 and 
by Levy and Wormser in a bicyclist. 3 

A localized degenerative atrophy of the interosseous primus and secundus 
combined with sensory disturbances, particularly in the little finger, was ob- 
served by Wertheim-Salomonson in diamond-cutters, these workmen being 
compelled to hold the diamonds with both hands. To milker's spasm, an occu- 
pation neurosis, neuritic paralysis and sensory disturbances may be added. 
Mild motor irritative symptoms are by no means rare in occupation pareses. 

1 See Cassirer, " Vasomotor Trophic Neuroses," in this volume. 

2 Rev. neurol., 1904, p. 500. * Rev. neurol., 1904, p. 1219. 



772 NEURITIS AND POLYNEURITIS 

A degenerative paralysis localized in the small muscles of the hand supplied 
by the ulnar nerve is observed in glass-cutters, engravers, lathe-workers, and 
xylo graph ers. Although the point of lesion in these cases is to be sought at 
the elbow- joint, the nerve being here pressed against a hard, underlying sur- 
face and thus irritated, nevertheless, as Bruns asserts, only the terminal 
branches of the nerves in the hand are as a rule implicated. If continuous 
pressure causes inflammatory processes to develop in the tissues covering the 
nerve, the damage to the nerve is even more intense. For instance, Cursch- 
mann reported the case of a gold polisher, in whom a bursitis with induration 
had developed over the ulnar nerve; of course such occurrences greatly retard 
the disappearance of the paralysis. 

Occupation pareses more rarely implicate the nerves of the upper arm 
than the small muscles of the hand. Bernhardt 1 reported left-sided radial 
paresis in a waiter. This patient, for an entire winter, had carried plates in 
the left hand, which was strongly flexed dorsally while his head was turned 
toward the right. A weaver, treated by Oppenheim, had paralysis of the right 
triceps due to the fact that he was compelled to extend the lower arm about 
20,000 times daily. The same author saw paralysis of the deltoid and the 
supra- and infraspinatus muscles in a railroad guard whose business it was 
to depress the lever of a signal. Paralysis of the serratus from over-use in 
various occupations has been described by Bernhardt and Remak. Hoefl- 
mayr 2 described cases of occupation neuritis which implicated the brachial 
plexus (the axillary nerve) in carpenters, tanners, and watchmakers. 

Paralyses of the lower extremity from over-exertion are much more rare. 
I recently saw a case implicating the crural nerve which I must regard as 
occupation neuritis. 

Case -20. — A smith, aged 42, and healthy, was compelled while at work to fix his 
left leg firmly at the knee-joint and to extend it; at the same time with considerable 
force and various forward and backward movements of the trunk he swung a heavy 
hammer high above his head. For a year he had complained of weakness in the left 
thigh. Walking was difficult, especially the ascent of stairs. He also felt dull, un- 
pleasant sensations on the anterior surface of the thigh. I found the typical signs of 
crural neuritis — sensitiveness of the nerve to pressure, atrophy of the quadriceps, mark- 
edly decreased knee-jerk, weakness of the ilio-psoas and quadriceps, partial and mild 
DeR in these muscles, and a decided disturbance of sensation in the thigh along the 
course of the crural nerve, and over the saphenous major on the inner side of the leg 
and foot. Here the affection was attributed to over-exertion combined with turning 
and torsion of the nerve, especially since all other etiologic factors were absent. 

Except under such circumstances, professional paresis in the muscles of 
the thigh is extremely rare. I must call attention to a case reported by 
Curschmann of professional paresis in a stone breaker and farmer; this ex- 
tended along the course of the peroneal and tibial nerves, and implicated the 
crural nerve. He localized the injury as occurring in the flexor of the groin, 
and attributed the compression of the nerve to constant kneeling. Occupation 
pareses in the nerves of the leg are somewhat more common. I saw a tailor 
who, two weeks before he came under treatment, was suddenly attacked by 
paresis of the right thigh followed by weakness and degenerative paralysis of 

i Bernhardt, " Festschrift fur v. Leyden," II. 

2 Hoeflmayr, Miinchener med. Wochenschr., 1901, 45. 



NEURITIS 773 

the muscles of the right leg; the latter affected chiefly the posterior tibial, 
but there was no sensory disturbance. The patient reported that his work 
compelled him to flex the right leg against the thigh as much as possible, and 
at the same time to cross the left leg over the right. The paralysis improved 
after the patient suspended his work; here excessive use of the muscle com- 
bined with pressure was the cause. A similar origin for the paresis was re- 
ported by Bernhardt * and Jones in saddlers. Muthmann quotes a case de- 
scribed by Bernhardt of professional and bilateral neuritis of the peroneal 
nerves in a patient, aged 28, who had for years used his leg to turn a lathe; 
he usually stood on his left leg while he turned the machine with the right. 
The neuritis was most marked on the left side. I saw a woman, aged 35, 
the wife of a painter, and poorly nourished, who 3 months previously was 
suddenly attacked by weakness in the left ankle-joint and a numbness in the 
dorsum of the foot. I found a decided peroneal paralysis, all the muscles 
supplied by the peroneal nerve being uniformly implicated. The nerve was 
sensitive to pressure; on the dorsum of the foot there was distinct hypalgesia. 
There was partial DeE. The patient had previously sewed on a machine for 
about 15 hours daily. A similar case was described by Charcot and Meige. 
In some cases the genesis of this form of neuritis is not always clear. In 
addition to over-exertion, pressure and torsion have a deleterious effect. An 
origin similar to that of the peroneal paralysis from the use of the sewing- 
machine was reported by Raymond and Courtellemont 2 as following excessive 
bicycling. The most common form of occupation paresis in the lower extrem- 
ity is probably observed in potato diggers, farmers, brick-layers, and the like 
— paralyses due to the kneeling posture necessary for the work of these men. 
This was first described by Zenker, then by Kron, Hoffmann, Remak, and 
Bernhardt, finally by Schultz 3 and Schleutzka and Curschmann (/. c). 

Case 21. — The following case is an example of this type of professional paralysis. 
The patient, a girl aged 20, was engaged in planting beets when in May, 1902, with 
symptoms which came on gradually she was taken ill. She noticed that her right foot 
clung to the ground, and that the dorsum of the foot was numb. On continuing her 
work she felt severe pain in the foot. The affected right leg was the one she was 
compelled to use most. I found paralysis, chiefly in the peroneal region, but also 
implicating the muscles of the calf. The Achilles tendon reflex was abolished; there 
were electric disturbances which were most marked in the extensors of the foot and 
toes, but also distinct in the muscles of the calf. 

In these cases the peroneal nerve is the one chiefly implicated, but other 
investigators as well as I have known the posterior tibial nerve to be affected. 
The reason why the peroneal nerve is usually attacked is that in kneeling the 
tendon of the permanently contracted biceps presses the nerve against the head 
of the fibula. The posterior tibial nerve is less severely compressed by the per- 
sistent contraction of the gastrocnemius. Schultz asserts that, besides mechan- 
ical factors, such as a kneeling posture, other causes are operative ; for instance, 
when planting beets the person usually kneels upon the cold, moist earth while 
he is frequently over-heated and perspiring. 

i " Die Erkrankungen der peripheren Xerven," 2. Aufl., I, p. 514. 
2L. c. 

3 Schultz, " Ueber Fusslahmung, speciell Peroneuslahmung bei Riibenarbeitern." 
Deutsch. Arch. f. klin. Med., 1904, Bd. 80. 



774 NEURITIS AND POLYNEURITIS 

Diagnosis. — It is unnecessary again to discuss occupation pareses in gen- 
eral, and only a few differentio-diagnostic considerations are called for. 
Oppenheim x states that the condition is not infrequently confounded with 
spinal progressive muscular atrophy. The points of differentiation are the 
following: occupation pareses are usually, although not invariably, unilateral. 
Paresthesia, pain, and objective sensory disturbances in the affected nerve 
regions are common, and are often permanent. If, as sometimes happens, a 
differentiation is not at once possible, the course of the disease must be con- 
sidered. In the great majority of occupation pareses the prognosis is favor- 
able if the patient can abandon his work; if the atrophy has not existed too 
long, it then usually disappears. As a rule the cases chiefly due to pressure 
run an especially favorable course; and, in consonance with this, the changes 
in the electric contractility are usually slow — as is also the case in non-profes- 
sional paralyses from pressure. When the pareses are due in the main to 
over-exertion, the course is often very prolonged, and does not become aggra- 
vated. In a case that I observed the diagnosis of occupation paresis was indi- 
cated only by the course. Here, combined with indefinite sensory irritative 
disturbances and degenerative atrophy, there was a weakness of the right tri- 
ceps pectoralis and teres major. No objective sensory disturbances were pres- 
ent, nor was either muscle or nerve painful on pressure; certainly not when 
I first saw the patient. He had strained these muscles bilaterally by athletic 
exercises. It was impossible at first to decide whether the case was chronic 
anterior poliomyelitis at the height of the seventh cervical segment, whether 
spinal progressive muscular atrophy had gradually taken place, or whether 
it was neuritis from excessive use of the muscles. After being under observa- 
tion for several months, the course indicated that the last view was correct. 
The differentiation from syringomyelia may be even more difficult since in 
this disease there are motor as well as sensory disturbances. The marked limi- 
tation of the motor as well as sensory disturbances to peripheral regions of 
innervation, the exemption of proximal parts of the upper extremity, the uni- 
lateral implication, the conspicuous sensitiveness of the nerve to pressure, all 
favor occupation paresis; oculo-pupillary symptoms, severe trophic disturb- 
ances in the bones and skin, thermo-anesthesia, and spastic symptoms in the 
legs favor the diagnosis of syringomyelia. In doubtful cases prolonged obser- 
vation is most necessary. 

Treatment. — The therapy primarily demands that the work which pro- 
duced the disease must be abandoned. This should be done as soon as possi- 
ble, best during the stage showing sensory irritative phenomena, which, how- 
ever, is not always practicable. The treatment is the same as for other forms 
of neuritis. Rest is the first and chief remedy, whether we are dealing with 
spontaneous, infectious, or traumatic neuritis. It is obvious that all motion, 
every muscular contraction, will intensify the inflammatory process. We must 
give the diseased member rest, and, on the other hand, we must see that 
there is no pressure upon the diseased nerve and muscles. Therefore a firm 
bandage cannot be used, but, as Edinger proposes 2 a relative position of rest 

i " Lehrbuch," p. 247. 

2 Edinger, " Behandlung der Krankheiten in Bereiche der peripheren Nerven." 
Penzoldt-Stintzing's Handb., 1. Aufl., Bd. V, p. 568. 



NEURITIS 775 

— as in bed — for the legs, and a sling for the arm, is much better. In trau- 
matic neuritis, in which the nerve lesion necessitates that the bone and joint 
be firmly bandaged, direct pressure or torsion of the nerve must be carefully 
avoided, and we must guard against even the slightest compression of the 
paralyzed muscles. 

When there is a rheumatic or infectious etiology, general diaphoresis ac- 
cording to the ordinary methods is highly recommended by Oppenheim, who 
believes in removing the deleterious substance from the organism as soon as 
possible. For local relief, derivatives to the skin and occasional blood-letting 
with wet cups or, better, with leeches may be tried. We must be very cautious 
in the use of vesicants, particularly over anesthetic areas of the skin. Local 
inflammation may also be relieved by various hydriatic procedures. As a 
rule, cold in some form should first be employed, preferably ice-water com- 
presses, while the application of chemical agents to produce a sensation of 
cold (as ethyl chlorid spray, etc.) should be reserved for the later treatment 
of pain. When cold increases the pain — there is no rule for this — moist heat 
in the form of packs or hot compresses, bags of sand, local hot baths, and hot 
air (with Tallermann's apparatus) should be resorted to. That these have 
any direct effect to influence regeneration favorably is doubtful. Certainly 
with one or the other method we may frequently lessen the severe inflamma- 
tion and nerve pain. Some drugs, particularly arsenic and strychnin, have 
had ascribed to them a paralyzing action upon toxins. Remak quite properly 
doubts this, but, nevertheless, advises their occasional use in small doses. To 
relieve the pain, we require other remedies, among which the newer anti-neural- 
gics are especially active. Sodium salicylate has occasionally been regarded 
as an absolute cure for rheumatic and infectious neuritis ; as belonging to this 
category I must also mention antipyrin in doses of 7J to 15 grains, salipyrin, 
lactophenin, trigemin, and especially aspirin in the same dose; in small doses 
phenacetin (7^ grains), antifebrin (3 grains), and pyramidon (3 to 5 grains). 
The last remedy frequently has an excellent effect, but, as with all of the 
others, the patient must be carefully watched. Only on the rare occasions 
when there is severe pain in the lumbar or sacral nerves should we resort to 
epidural injections of cocain, as Chatelineau advised. In some cases we find it 
impossible to get along without an occasional injection of morphin. 

Electricity is generally regarded as the agent which, above all others, is cal- 
culated to stimulate the regeneration of a diseased nerve. This is not the 
place at which to discuss electrotherapy theoretically. Practically, except in 
the mildest forms of neuritis, there is scarcely a case in which electro-thera- 
peutic treatment is unnecessary. In the earliest stages when pain is severe, 
experienced authors advise the use of only the constant current. For the 
amelioration of the pain the stabile employment of the anode has in many 
cases proven effective. An anode having a diameter of 10 to 20 cubic centi- 
meters should be applied to the most painful area, while the broader cathode 
is placed at an indifferent point. When a nerve of great extent is susceptible 
to the electric current — for instance, the sciatic — the cathode may be applied 
at some other point of the nerve itself, so that the nerve will be, as it were, 
between two electrodes. After the current has been allowed to pass through 
the entire extent of the nerve, by slowly moving the electrodes we may occasion- 
ally increase the anodyne effect. The current is to be gradually increased to 



776 NEURITIS AND POLYNEURITIS 

the necessary strength of 4 to 6 milliamperes, and all marked fluctuations 
therein are to be avoided. In the later stages we may apply stronger currents. 
The duration of the sitting should at first be for about 5 minutes ; this may be 
increased to 20 minutes or longer, and even alternating currents may be em- 
ployed. During this time unpleasant sensations of numbness can be relieved 
by the occasional use of the f aradic brush ; even permanent results are attained. 
Electrical treatment of neuritic paralysis should begin after the severe irrita- 
tive symptoms subside. Whether it facilitates the histologic regenerative 
process is uncertain, but experience has shown that, at the time when ana- 
tomical restitution is to be expected, suitable electrotherapy may decidedly 
benefit the motor function. Remak x maintains that a diseased nerve which 
has been subjected to the influence of the constant current is much more read- 
ily innervated voluntarily than prior to this treatment (investigations in 
so-called pressure paralysis of the radial nerve). In such cases it is best to 
institute labile cathodal treatment, perhaps combined with stabile anodal treat- 
ment. Where faradic irritability is not too markedly lessened, the faradic 
current also is indicated. In the treatment of motor paralysis, particularly 
its later stages, massage is often used; this is said to produce a mechanical 
irritation of the paretic muscles and an alteration in the circulation, also to 
prevent deformities from contracture of the antagonists. From the onset the 
development of these deformities should be counteracted by a suitable posi- 
tion and the bracing of the paralyzed parts; later, in addition to massage, 
gymnastic exercises (active and passive movements with and without appa- 
ratus) should be advised. 2 In the chronic stage thermal baths occasionally 
give remarkable results. At first the indifferent baths may be used, such as 
those of Wildbad, Gastein, Badenweiler, Teplitz, Warmbrunn, Schlangenbad, 
Johannisbad, Ragatz, etc.; subsequently the thermal salt springs (Baden- 
Baden, Wiesbaden) and the carbonate springs (Nauheim, Oeynhausen, 
Soden). Simple salt baths as well as peat baths may be recommended, and 
the local application of peat compresses (fango). 

If neuritic paralysis is incurable, improvement in function may be brought 
about by operative treatment; especially by the transplantation of tendons 
which was first advised by Nicoladoni, Drobnik, Franke, and others for the 
deformities of infantile spinal paralysis. This has been ingeniously adapted 
to cases of neuritis, and in incurable neuritic radial paralysis good results have 
been attained. In the last few years another form of substitution has been 
attempted, and the peripheral end of a diseased or injured nerve has been 
inserted within the fresh edges of a healthy nerve. This " graffe nerveuse " 
has several times been performed between the facial and spinal accessory nerves, 
also between the facial and hypoglossal nerves. In several cases the operation 
has undoubtedly promoted regeneration, but the functional result is question- 
able on account of the constrained movements produced; further experience 
is awaited. Lethievant prefers to unite the nerve loops which are formed by 
longitudinal splitting of the end of separated nerves. 

A few words may be added in regard to special procedures occasionally 

i Remak, " Ueber die antiparalytische Wirkung der Elektratherapie bei Drucklahm- 
ungen des N. radialis." Deutsche Zeitschr. f. Nervenheilkunde, Bd. IV, p. 377. 

2 Further consideration of these therapeutic questions will be found in Edinger's 
article. 



NEURITIS 777 

necessary in traumatic neuritis. Here I follow Oppenheim. 1 If there is an 
open incised wound of the nerve, it should at once be sutured, and this, as a 
rule, is successful if carefully performed. If we see the patient only in the 
later stages, the question whether there is a complete interruption of conduc- 
tion (which excludes the possibility of a spontaneous reunion) is difficult to 
answer. If there is complete paralysis and total DeR, we should not at once 
operate. It is better to wait and see whether there is any chance of sponta- 
neous recovery, which cannot take place earlier than from 9 to 12 months after 
the appearance of paralysis. Then we are justified in attempting a secondary 
nerve union, which has been successfully done even after the lapse of years. 
Improvement after such an operation may be very slow, and hope should not 
be relinquished too soon. When the nerve is compressed by foreign bodies, 
these should be removed. Fragments of bone must be extirpated, or removed 
from a cicatrix (neurolysis). 

Pathology. — I have not as yet touched upon the anatomical foundation 
of localized neuritis. It was long ago shown by Sigmund Mayer that cer- 
tain degenerative and regenerative processes may take place in normal periph- 
eral nerves, and that here and there under normal circumstances local changes 
of structure may appear in the constituents of peripheral nerves. These 
" degenerations " affect only individual fibers or segments of fibers. The axis 
cylinders and medullary sheaths may be disturbed, the nuclei of Schwann's 
sheaths may be increased and enlarged, and apparently play the role of phago- 
cytes. We must bear this in mind when considering the anatomical relations 
of peripheral nerves. Regenerative processes may also appear. We find thin, 
delicate fibers with a narrow medullary sheath, also very fine axis cylinders 
which must be regarded as newly formed nerve fibers. 

Regarding the changes which take place in traumatic neuritis, we find it 
difficult to differentiate nerve inflammation due to traumatism from traumatic 
nerve degeneration. This point we previously discussed, and were forced to 
conclude that for clinical purposes a clear formulation and sharp differentia- 
tion of their modes of development was impossible. This is also true of the 
anatomical condition. After the aseptic section of a nerve a consequent degen- 
eration takes place in the peripheral (centrifugal) portion of the nerve 
(Waller's degeneration). Recent experience shows that the first histologic 
sign of this degeneration appears in the neurofibrillae, the conducting elements 
of the axis cylinders (Homen, Monckeberg and Bethe) 2 The fibrillae are no 
longer so smooth as they formerly were, they are less tense, and here and 
there they show granular thickening. The granulation gradually increases 
and contraction and granular destruction of the fibrillae take place. The 
perifibrillar substance is also slowly destroyed by swelling from the taking 
up of water. The changes in the axis cylinder are soon followed by those 
of the medullary sheath. The first signs appear from 2 to 5 days after 
separation. This structure is probably converted by a chemical change into 
a substance analogous to fat, small or large globules partially surround the 

i Oppenheim, " Lehrbueh," p. 428. 

2 Monckeberg and Bethe. " Die Degeneration der markhaltigen Nervenfasern," etc. 
Archiv f. mikrosk. Anatomie, 1899, p. 135; also the latest investigations of Schultz, 
including references. Centralbl. f. pathol. Anatomie, 1904; of Kattwinkel and Kerschen- 
steiner in Lubarsch-Ostertag's Ergebnissen, 1904, IX, p. 8. 



778 NEURITIS AND POLYNEURITIS 

axis cylinders (medullary ellipsoids). Figure 1 in Plate I distinctly shows 
these changes in a Marchi preparation. The medullary sheath undergoing 
destruction is indicated by black globules, clumps, and rings, while the intact 
medullary sheath is yellowish. The changes appear to begin at the point of 
trauma, and progress rapidly toward the periphery. The removal of decayed 
material is partly effected by their passage into the lymph tracts, partly by 
phagocytosis. The role of the phagocytes is assumed by various cells, some 
lymphocytes and leukocytes, some proliferated cells of Schwann's sheath, some 
from the endoneurium, perineurium, and the surrounding connective tissue. 

The changes demonstrated in Schwann's sheaths are important for another 
reason. They are closely related to reparative processes in the peripheral 
nerves. These begin long before degeneration has run its course. But before 
discussing this feature, we must consider the processes in the central stump. 

Great changes also take place in the first of the interannular segments of 
Ranvier : the medullary sheaths and axis cylinders are rapidly destroyed ; iso- 
lated fibers degenerate far upward centrally ; as a rule, the process is confined 
to the first six segments. In the nerve cells and in its fibers other changes 
appear, a knowledge of which is necessary for the full understanding of many 
pathologico-anatomical details. In the main these invade less deeply; the 
processes are less distributed, they appear later, and progress more slowly 
than those which cause Waller's degeneration. In the cells, particularly with 
Nissl's staining, we find transitory but obvious phenomena: dissolution of 
Nissl's bodies (chromatolysis) with a swelling of the entire cell and with 
nuclear deposit. It is certain that at least many of these cells are not de- 
stroyed and that they recuperate. Subsequently the cells themselves, as well 
as the fibers and all of their constituents, become thinner, smaller, and more 
delicate. Whether this retrogressive degeneration is histologically any differ- 
ent from Waller's degeneration is not yet known. 

Eeparative changes are early noticeable. Only a portion of the proliferated 
cells of Schwann's sheath assume the role of phagocytes. The others form 
spindle-celled bands, some of which are arranged within, some without, the 
old sheaths. As to the further process, there are two opinions which are 
diametrically opposed to each other, and a decision is at this time impossible. 
One view is that these bands are composed of newly formed nerve fibers which 
grow out of the central stump; the other (this is based upon Bethe's recent 
experiments) assumes that new nerve fibers are formed from the protoplasm 
of these spindle-celled bands (autogenous regeneration). Investigators admit 
the energetic growth of fibers from the central stump, and assert, what is 
clinically of great significance, that the reunion of the stump renders complete 
regeneration far more certain. According to Bethe, we can determine by ex- 
periments in young animals that autogenous regeneration* is perfect, while in 
full grown animals, although always quite apparent, it is never complete. The 
great recuperative power of the peripheral nervous system in contrast with that 
of the central, is of the utmost importance in pathology, no matter how it 
may be explained. 

If union of the severed ends of a nerve does not take place, a nerve cicatrix 
develops at the point where the trauma occurred. The connective tissue of 
the peripheral nerve, the endoneural, the perineural, and the epineural, prolif- 
erates further, becomes fibrous and poor in nuclei, and in dense, irregular 



NEURITIS 779 

bands encircles the nerve fibers which force themselves between the individual 
nerve bundles. In the peripheral stump these gradually atrophy, but prob- 
ably not until processes of repair begin. 1 At all events conduction from the 
center to the periphery is permanently and completely abolished. 

Paralysis of such a nerve cicatrix is illustrated by Figure 2, Plate I. 
This is from a preparation of a section of the brachial plexus in a young man, 
who was cut by a knife in the supraclavicular fossa. To promote secondary 
union of the nerve this piece completely embedded in connective tissue was 
extirpated. We see that the entire, external, and otherwise very regular forma- 
tion of the nerve is destroyed. In many areas the separate layers of connective 
tissue covering cannot be distinguished. The connective tissue is poor in 
nuclei, close, and fibrous. In many areas it presses upon the glistening, red- 
stained portions (/) in the van Gieson preparation, and also between the finer 
nerve bundles. Most of the retained axis cylinders are extraordinarily thin 
and fine. The changes are partly atrophic, partly the formation of new 
fibers. The medullary sheaths are very narrow, in stained preparations they 
are not sufficiently blackened, they do not form complete rings. This incom- 
pleteness and irregularity of the medullary sheaths is most distinctly evident 
in longitudinal sections. In many areas the nuclei in Schwann's sheaths 
appear to be increased. This increase is perhaps only apparent, and is ex- 
plained by the compact arrangement of the individual constituents. The other 
connective tissue nuclei are diminished. 

These changes may all be regarded as simple degenerative processes and 
their immediate consequences; there is no inflammation. Yet the alterations 
which we recognize as the invariable sequela? of nerve injury, as Waller's degen- 
eration, are considered to form the foundation of parenchymatous neuritis; in 
fact, whenever the diagnosis of neuritis intra vitam has been based on the 
previously described landmarks, these very lesions were demonstrated in the 
peripheral nerves. 

The number of necropsies performed in cases of simple, localized mono- 
neuritis is naturally scant; and such cases will only accidentally be found 
on the post mortem table. In a case of " rheumatic " facial paralysis, Minkow- 
ski demonstrated a purely degenerative process in the peripheral nerve 
branches, while the nerve trunk from the geniculate ganglion to the center 
was normal; the neurilemma was not implicated. In another case Dejerine 
and Theohari found degeneration in all of the branches, while the root was 
intact. After traumatic lesions of a peripheral motor nerve (see above) the 
cells of the nuclei show with Nissl's staining the same characteristic changes 
as in the experiment. 

In a case recently reported by A. Westphal - of plexus neuritis in an 
apoplectic form without actual mononeuritis, there were severe, parenchy- 
matous, neuritic changes which increased from the center to the periphery; 
slight lesions were also demonstrated in the entrance zone of the posterior 
roots. The broad pale fibers, marked by divisions which Gudden has de- 
scribed, were here noteworthy. They resembled spindle-celled bands, and like 
these are probably to be attributed to regenerative processes. 

i See the article by Egger and Armand Delille {Arch, de ~N enrol., 190,3, p. 474). 
2 A. Westphal, " Ueber apopleetiforme Neuritis." Arch. f. Psychiatrie, Bd. 
XL, p. 64. 



780 NEURITIS AND POLYNEURITIS 

The accompanying figures 3 and 4, Plate II, show, in an osmic acid prepa- 
ration after van Gieson, the prominent changes on a weak and powerful 
enlargement of a longitudinal section made in a case of " parenchymatous 
neuritis " ; the medullary sheath is stained black, and its irregular destruction 
is especially distinct; in some areas it is still intact, in others, partly belong- 
ing to the same fiber, there is complete destruction. This reminds us of speci- 
mens furnished by segmentary neuritis, which was first described by Gom- 
bault, and designated nevrite segmentaire periaxille; he found it in guinea 
pigs experimentally poisoned with lead salts. Stransky 1 has recently reviewed 
these investigations, which had elicited but little appreciation. The most char- 
acteristic feature of the process is its lack of continuity, its limitation to more 
or less lengthy areas within the confines of an otherwise normal nerve fiber. 
The origin of the process is a fine, granular, drop-like decay of the myelin, 
while in Waller's degeneration the medullary sheath decomposes into much 
less massive clumps; from the onset there is said to be a conspicuous prolif- 
eration of the cells of Schwann's sheath (nuclei as well as protoplasm), so that, 
unlike Waller's degeneration, the entire process reveals a conspicuous paren- 
chymatous inflammation. Some of the proliferated cells temporarily change 
into phagocytes. Gradually the axis cylinder is implicated, it becomes broader 
and paler, until finally it can no longer be demonstrated with the ordinary 
staining methods; Stransky doubts whether it is actually destroyed, because 
the peripheral portions of the nerve fibers do not show Waller's degeneration 
which might otherwise be expected. The process admits of perfect restitu- 
tion, the histologic signs of which are easily recognized. Stransky believes this 
form of disease to be much more common than would appear from literature. 
The chief value of these investigations lies in the fact that, if Stransky's view 
is correct, it enables us to differentiate inflammatory parenchymatous disease 
of the peripheral nerve from secondary Wallerian degeneration, and the whole 
subject gains in clearness. But whether such a sharp distinction as Stransky 
proposes is practicable can be learned only by further investigations. It 
appears plausible in the mild forms of certain paralyses — for example, pres- 
sure paralysis during sleep — to assume that a periaxial neuritis in its initial 
stage is the anatomical foundation, since from the rapid restoration of the 
faculty of conduction in these cases we must conclude that the nerve element 
of conduction has not been greatly damaged. 

Dejerine-Klumpke and Falkenheim reported a few instances of changes 
produced by subcutaneous injections of ether after the question had been ex- 
perimentally investigated by Arnozan and Pitres and Vaillard. If a nerve 
is exposed to the action of sulphuric ether for 24 hours, changes may be 
demonstrated in its area, these corresponding to necrosis of a portion of the 
nerve; this is followed after an interval by parenchymatous degeneration of 
the peripheral portion. In pathologic cases nerve destruction to a varying 
extent has been demonstrated in the radial nerve. As to the propagation of 
the inflammation from the diseased vicinity of the nerve in the different forms 
of neuritis, we naturally often find true inflammatory changes. This is the 
case when suppuration from surrounding areas is conveyed to the nerve — for 

i Stransky, Ueber discontinuirliche Zerfallsprocesse an der peripheren Nerven- 
faser." Journ. f. Psychologie u. Iseurologie, 1903, H. 5 u. 6. 



NEURITIS 781 

example, caries of the petrous portion of the temporal bone to the facial 
nerve (E. Flatau), suppuration of the cervical glands to the pneumogastric 
nerve (Lugaro). In these cases the inflammatory process in the interstitial 
tissue of the nerve is to some extent propagated, dilatation is produced with 
immoderate fulness of the vessels, hemorrhages, small-celled infiltration, and 
a serous exudate ; besides these signs of true inflammatory interstitial changes, 
there are lesions of the parenchyma, swelling of the medullary sheaths and 
axis cylinders, and degeneration and destruction in the peripheral portion. 
Changes of this kind are macroscopically recognizable as swelling of the periph- 
eral nerves; subsequently, in place of inflammatory infiltration we find a 
round-celled exudate and hemorrhage from the connective tissue, or new for- 
mations may produce the picture of neuritis and perineuritis nodosa, the most 
characteristic type of which is the neuritis or polyneuritis of leprosy, which 
we shall later describe. As an invariable and secondary result of neuritis 
which has interrupted conduction, we learn to recognize degeneration in the 
most peripheral portion of the peripheral nerve. With the same regularity 
as in animal experiment, we find changes in the muscles supplied by the 
diseased nerve. The first sign is a fatty metamorphosis in the muscular 
fibrillae which Obersteiner and I demonstrated with the Marchi method in a 
case of hemiatrophy of the tongue after neuritis of the hypoglossal nerve. 
In a further progression of the process and its prolongation, this change in 
the muscle advances and leads to severe atroplw, a decrease in the size of the 
fibrillae, the disappearance of the transverse stria?, and such a massive deposit 
of fat in the muscles that the change is recognizable even macroscopically. 
Westphal found the alterations less marked in his case. The caliber varied, 
and large voluminous fibers were found alongside small atrophic ones and 
some of medium size. Some of the fibers were rounded off; there were cen- 
tral nuclei and rows of nuclei, as well as spaces which had formed between 
them. Transverse striation was still evident. There was no fatty degenera- 
tion. Little is known of the changes in the motor end-plates in neuritis. The 
changes which take place in the central portion of the diseased sensory or 
motor nerve, and in the skin and its dependent structures from alteration of 
its sensory innervation, are more variable and more difficult to explain than 
the changes in the muscles. 

The former are found as so-called retrogressive degeneration, such as 
appears in the central stump of the severed nerve as above described; in the 
nerve fibers a slow and tardy atrophy in all of its constituents, in the cells 
there is at first an alteration in the stainable substance — XissPs bodies — 
which, after producing slight changes, passes away. In the subsequent course, 
atrophy of the cellular elements causes a reduction in the size of the individual 
elements or even their complete wasting, most of the finer structures remain- 
ing unchanged. These experiences (which in man are based particularly upon 
the findings in the spinal cord of persons who had lost a member by amputa- 
tion some time before death) teach us that when in a case of peripheral neuritis 
severe lesions, like acute degeneration of the fiber or the cell, are found in the 
central portion of the affected nerve or its spinal origin, we are not dealing 
with phenomena immediately due to peripheral disease, but substantive ones; 
this is true both of localized and multiple neuritis, as well as of the spinal 
changes which often accompany the latter. In some cases for which another 



782 NEURITIS AND POLYNEURITIS 

explanation seemed obvious, as in a case of carcinomatosis of the left brachial 
plexus and degeneration in the left posterior column, published by Jacobsohn, 
this cannot be considered to apply. 

A word must still be said of the dependence of the changes in the skin 
and its structures on disease of the peripheral nerves. Under the symptomatol- 
ogy I called attention to the most important of these trophic, vasomotor, and 
secretory disturbances. They are gangrene, bed-sore, perforating ulcer, some 
eruptions of vesicles, and glossy skin which represent the combination of many 
different symptoms, etc. The comparative rarity of these symptoms precludes 
the theory that they directly depend upon a peripheral nervous process — as, 
for instance, muscular atrophy. To some extent they are no doubt due to a 
coordinative and substantive disease of the skin, others are the consequences 
of analgesia of the skin from neuritis, which makes it more susceptible to 
injury; but all clinical experience seems to point to the fact — in spite of 
the great labor expended upon experiments, proof by this means is much less 
positive — that there is a direct trophic dependence of the skin and its struc- 
tures upon the nervous system, although the nature of this dependence is by no 
means always clearly appreciated. 1 

POLYNEURITIS 

The method of division which I have so far followed is, as stated, not a 
strictly systematic one. I have considered only the inflammation of individual 
nerves, mononeuritis, and I shall now describe polyneuritis. In tabulating the 
different forms of neuritis with which we have become acquainted, we find 
the following: 

(1) Traumatic mononeuritis of any origin; (2) Occupation neuritis-, (3) 
Wound neuritis; (4) Injection neuritis; (5) Propagated neuritis ; (6) Ascend- 
ing neuritis; (7) Post-infectious mononeuritis; (8) Rheumatic and idiopathic 
mononeuritis. 

Groups 1 to 6 include all cases of neuritis from local cause, and groups 7 
and 8 those due to internal cause. It would be perfectly justifiable to con- 
sider the two last mentioned groups with polyneuritis, since these are invari- 
ably produced by internal conditions. But if we adopt this method of group- 
ing we shall meet with difficulties; for, as has been stated, in isolated cases 
local and internal causes may act jointly. On the other hand, the line of 
demarcation between polyneuritis and mononeuritis is not sharply defined; 
between these two groups are affections of several isolated nerves by a local 
cause, and plexus neuritis and mononeuritis multiplex must be included. 
Polyneuritis, in a restricted sense, occupies so large a field that some subdivi- 
sion appears necessary. I shall follow Remak and classify it as follows : 

(1) Polyneuritis from exogenous intoxication; primarily alcoholic neu- 
ritis. This is the most common form of polyneuritis, and serves as the type 
of the affection; other poisons to be considered are lead, arsenic, carbon bisul- 
phid, carbonic oxid, and mercury. While it is unlikely that a single acute 
intoxication with alcohol ever produces polyneuritis, this is quite possible in 
the case of arsenic, hence we must differentiate between polyneuritis due to 
acute and to chronic intoxication. 

1 See also Cassirer, " Vasomotor Trophic Neuroses," p. 88, et seq. 



POLYNEURITIS 783 

(2) The second group consists of polyneuritis due to chronic, and, more 
rarely, to acute autointoxication, such as occurs in diabetes, gout, tuberculosis, 
syphilis, pregnancy and the puerperium, senility, general cachexia, poison- 
ing by meat and sausage, and in other forms of intestinal autointoxication. 

(3) Polyneuritis during and after acute infectious diseases. If we class 
polyneuritis following diphtheria with that occurring in other infectious dis- 
eases, it is not strictly correct; for diphtheritic polyneuritis differs in type 
from the other infectious forms of polyneuritis. 

(4) Idiopathic polyneuritis. This peculiar form of the affection occurs 
as an infectious disease, and includes certain forms of Landry's paralysis. 

(5) Beri-beri, which, as a rule, must also be considered as a disease sui 
generis. 

(6) The polyneuritis of leprosy, which, because of its typical clinical and 
anatomical features, cannot be included in the second group. 

Polyneuritis is frequently produced by the combination of several deleteri- 
ous factors; for example, the combination of alcohol and lead, or alcohol and 
tuberculosis. Oppenheim calls attention to the fact that those suffering from 
alcoholic polyneuritis are much more susceptible than others to infectious 
diseases. 

Symptoms. — The symptomatology of polyneuritis does not correspond to 
that of mononeuritis; in many respects it is peculiar, and demands a special 
description. The individual forms show many variations, but, on account of 
its preponderating frequency, alcoholic polyneuritis is the most important. 

This occurs chiefly in spirit drinkers, but occasionally in those who drink 
beer and wine ; for example, it is said to be exceedingly rare in Munich, where 
enormous quantities of beer are consumed. I will quote a case which will 
best illustrate the essential features of the disease, and also describe the 
symptoms. 

Case 22. — The patient is a beer brewer, aged 39, who formerly drank upward of 
4 liters of beer a day, and now consumes a large quantity of spirits. The patient 
presents a number of the signs of chronic alcoholism. Early in the morning there is 
retching and vomiting of mucus. It is difficult for him to fall asleep, and before doing 
so he is subject to the familiar occupation hallucinations, believing that he is still at 
work ; he has frightful visions, sees black men, and the like. For a year past he has 
felt some exhaustion on walking, formication and numbness in the legs being added, 
and in the last few weeks all of these symptoms have become aggravated; periodically 
there are severe drawing pains. There is no difficulty in urination, no double vision, 
no girdle sensation. On examining the patient, his gait is striking. He lifts his legs 
immoderately high from the floor, and the feet wabble to and fro in a flaccid way. 
When he stands with his eyes closed and his feet close together, there is marked sway- 
ing (Romberg's symptom). Inspection of the lower extremities reveals marked atrophy 
of all the muscles of the leg, especially the extensors ; to palpation they feel coarse, 
like fibrous tissue. These muscles, especially of the calf, are very sensitive to pres- 
sure, as are also the nerve trunks — particularly the peroneal and tibial nerves in the 
popliteal space and behind the internal malleolus. There is also moderate hypotonia. 
Upon tapping the patella tendon there is a feeble contraction of the quadriceps, fol- 
lowed by a tremor which simulates an increase of the patella reflex. The Achilles 
tendon phenomenon is abolished bilaterally, movability and power are good in the 
hip- and knee-joints, and the leg can be extended bilaterally with almost normal force. 
The power of extension is absent in the right foot ( tibialis anticus ) , in the toes it is 
minimal ; the power of abduction is diminished in the foot ; the flexors of the foot and 
toes show loss of function. On the left side the dorsal flexors and the peroneal muscles 
are decidedly affected, while the flexors of the foot and toes function normally. On 



784 NEURITIS AND POLYNEURITIS 

the plantar surface of the toes and the anterior portion of the foot, brush contact is 
not felt bilaterally; in the same areas there is hypalgesia, while the sensation of posi- 
tion is not markedly disturbed. On the other hand, the " sensation of vibration " in 
the tibiae is lost. On electric examination the right peroneal nerve and the muscles 
supplied by it fail to react to the faradic current, the posterior tibial and the muscles 
it innervates respond feebly to powerful currents; in the right peroneus longus very 
strong galvanic currents are necessary to produce even a minimal contraction. Stimu- 
lation of the posterior tibial nerve causes a feeble contraction in the triceps suras. 
On direct irritation of the muscles of the lower leg (extensors as well as flexors) we 
obtain with strong currents only a sluggish AnCC. Consequently partial DeR with a 
marked decrease of contractility has here been reported. 

In the extended hands there is an active vibrating tremor. The grasp of the hand 
and all other movements of the upper extremities are normal ; the median and ulnar 
nerves are bilaterally painful to pressure, which produces radiating pains. The tendon 
reflexes are preserved and the tongue is tremulous, but the cranial nerves, the eye- 
grounds, and pupils show no pathologic change. 

The patient presents the familiar general symptoms of chronic alcoholism, which 
are of diagnostic value, although the alcoholic often denies the excessive use of spirits; 
among these symptoms are the well-known vomitus matutinus, the retching and vomit- 
ing of mucus, insomnia or restless sleep, disturbed by frightful hallucinations, dreams, 
and a tendency to spasmodic contraction of the muscles (particularly of the calf which 
is perhaps the first indication of a neuritic or myositic change) and numbness of the 
legs, especially when these members rest upon a hard surface. Furthermore we note 
the alcoholic tremor, usually most marked in the tongue and hands, the general signs 
of nervous irritability which is often of characteristic type, uncontrollable anger which 
may culminate in a tendency to destruction, as well as fear, the maudlin state which so 
readily leads to tears, and jealousy. The toper may often be recognized at a glance 
from his external appearance. 

The early symptoms of neuritis are not often so sharply denned as those 
of chronic alcoholism. Paresthesia in the leg gradually becomes more marked ; 
more severe pain develops as well as weakness in the legs which becomes notice- 
able upon long standing, and the fundamental symptoms of neuritis then 
appear. In contrast to the cases with more or less chronic development, the 
initial stage of which may extend through several months, are those with 
acute onset. The disease may develop immediately after an attack of delirium 
tremens. By no means rarely it is due to a predisposing cause; therefore 
anything which lowers the alcoholic's power of resistance, such as an infec- 
tion, influenza, pneumonia, trauma, etc., may be sufficient. 

As to the frequency with which alcoholics are affected by neuritis, the sta- 
tistics of the Hamburg hospitals show that it occurs in about 3 per cent, of 
all cases of alcoholism. Apparently women are relatively more frequently 
attacked than men (Gowers states three times as often) which is attributable 
to the more delicate constitution of women. According to Ross, the majority 
of cases occur between 30 and 50 years of age (64 out of 88) ; 13 of his 
patients were between 20 and 30, 10 between 50 and 60. Before and after 
these limits polyneuritis is very rare, but it has been reported in children aged 
7 and 5 years. I saw a case of alcoholic polyneuritis in a child, aged 3, whose 
father drove a beer wagon. Every evening the child was given a glass of beer, 
and sometimes spirits, to drink. Three months previously, it was attacked 
by severe pain in the feet accompanied by fever. Gradually weakness of the 
legs appeared, and, seemingly, also of the hands, and sometimes it was diffi- 
cult for the child to sit up. The pains and weakness in the arms and trunk 
disappeared, and upon examination sensation so far as we could judge was 



POLYNEURITIS 785 

normal, but the muscles of the calves still appeared to be painful on pressure. 
The knee- and heel-jerks had disappeared. There was decided paresis of the 
extensors of the foot and toes, as well as the abductors of the foot. The paresis 
was more marked on the right than on the left side, and the power of flexion 
in the toes was defective. Electrically the paretic muscles showed partial 
DeE. From the development and nature of the affection, there could be no 
doubt of the correctness of the diagnosis, polyneuritis; that alcohol is espe- 
cially injurious to the nervous system of a child is proven by many experiences. 
It appears that even the inhalation of alcohol may produce polyneuritis. 
Eemak quotes from Lancereaux the case of a woman, aged 35, who continually 
inhaled the vapor of alcohol which she had in her bed-room, and this caused 
polyneuritis. Several of my patients have attributed their neuritis to the 
fact that they were compelled to»work in rooms filled with the vapor of alcohol. 
Under such circumstances it is extremely difficult to exclude the immoderate 
consumption of alcohol, yet there is no reason why the inhalation of alcohol 
should not likewise be injurious. 

Even when there are no complications alcoholic neuritis may set in with 
-fever, but fever may also be absent — especially in those cases which develop 
more slowly. It is not necessarily present in idiopathic polyneuritis, not even 
in severe cases. Among the general symptoms those referable to the gastro- 
intestinal tract must be especially mentioned, and it will often be doubtful in 
how far we may ascribe these to neuritis in a restricted sense and in how far 
to chronic alcoholism. Enlargement of the spleen is probably only observed 
in idiopathic polyneuritis, and the same may be said of the pathologic urinary 
findings (urobilinuria and albuminuria) as of the gastrointestinal disturb- 
ances. 

As in our case the pains preceded the paralysis, and as is usually the case 
with polyneuritic pains they are often most intense, drawing and tearing in 
character and are situated deeply; they may readily be differentiated from 
the lancinating pains of tabetics by the description. As a rule they do not 
occur in typical attacks as do the lightning pains of the tabetic. Frequently 
they are combined with cutaneous hyperesthesia, and hyperesthesia plantaris 
is often an exceedingly annoying symptom which may persist for a long time, 
and markedly affect the gait of the patient. Although the pains are often the 
earliest symptom of alcoholic neuritis, they frequently disappear or decidedly 
lessen in intensity after the appearance of the paralysis, certainly in those cases 
in which the paralysis is amyotrophic. 

Besides the pain there are usually various paresthesias as well as thermo- 
paresthesia, and pain upon pressure of the nerve. This may be present when 
there are no other symptoms of neuritis. It is most prone to appear in the 
peroneal and posterior tibial nerves, also in the crural and other nerves of the 
lower extremities; but, as in one of my cases, it may likewise be observed in 
the upper extremities in which as a rule there are no pathologic phenomena. 
All manipulations which cause torsion of the diseased nerves evoke sponta- 
neous pain or pain which radiates in these nerves (sciatic phenomenon and 
similar symptoms). The muscles are often sensitive to pressure, sometimes 
even more so than the nerves, yet we are unable to diagnosticate neuromyositis 
from this early sensitiveness of the muscles to pressure (Eemak). Distinctly 
palpable swelling of the nerve is very rarely observed in alcoholic neuritis or 
51 



786 NEURITIS AND POLYNEURITIS 

in most other forms of polyneuritis ; leprous polyneuritis only being an excep- 
tion. The paralysis gives to the pathologic picture its peculiar stamp, and its 
essential features are as follows: The paralysis is almost symmetric, it usu- 
ally implicates the distal ends of the extremities, and, in alcoholic neuritis as 
well as most other forms of polyneuritis, chiefly the lower extremities. Here 
the peroneal muscles are preferably attacked, being more markedly implicated 
than those supplied by the tibial nerve. When the upper extremity is attacked 
— usually the radial region — this generally occurs later, and the paralysis is 
of ascending type, also degenerative and atrophic, and in its course shows 
the various stages of the reaction of degeneration. 

Corresponding to the chief localization, the first symptoms appear as weak- 
ness in walking. The patient soon tires. The symptoms as described resemble 
those of so-called intermittent claudication. For example, a patient, aged 23, 
who a few weeks ago suffered from difficulty in walking (he could walk only 
ten minutes at a time), stated that he soon felt intolerable formication, a feel- 
ing of tension and sensation of cold in the skin, and weakness in the legs, like 
paralysis, which compelled him to sit down. After ten minutes he was able 
to resume walking, but the same symptoms constantly recurred. Examination 
revealed the early signs of an alcoholic polyneuritis affecting the muscles of 
the leg, especially the peroneal, but no alteration in the vascular apparatus. 
In these cases walking gradually becomes more difficult. If the patient is 
examined in a somewhat advanced stage, which is apt to be the case, the gait 
usually resembles the steppage gait. The legs are abnormally lifted at the 
hip- and knee-joints because, otherwise, the tip of the patient's flaccid foot 
would not leave the floor. Close examination generally reveals paresis, most 
marked in the extensors of the foot and toes and in the abductors, while the 
flexors, although paretic, usually show less disturbance of function. The 
extensors of the leg and the muscles which raise the thigh toward the*pelvis 
are usually less implicated. The muscles supplied by the peroneal nerve are 
not affected to the same extent ; in fact, in all forms of polyneuritis paralysis 
throughout the extent of a nerve is remarkably frequent, but only in excep- 
tional cases are all the muscles of an extremity completely paralyzed. The 
paralysis is usually symmetrical, but to this there are numerous exceptions. 
One leg in toto or individual muscle groups may be much more markedly 
affected than others, and there are cases in which the symptoms of paralysis 
are almost wholly confined to one side. 

In the further progress of the paresis walking becomes impossible; the 
patient is confined to bed. The muscles of the upper extremity and of the 
trunk are also weak. There are but few exceptions to the rule that the paral- 
ysis ascends, yet cases have been reported in which the affection appeared in 
the arms. The paralysis of the arms is usually less profound and less extensive 
than that of the legs. As a rule, the radial region is more markedly impli- 
cated, and examination shows that the muscles supplied by the nerve are not 
uniformly attacked. The supinator longus and the abductor pollicis longus 
may be exempt from the paralysis, so that a type resembling lead paralysis is 
produced (Oppenheim). The small muscles of the hand and the long flexors 
of the hands and fingers are occasionally implicated, while the muscles of the 
arm and shoulder are usually exempt or only temporarily affected. Finally, 
there are cases with absolute paraplegia cervicalis. I once saw the rare picture 



POLYNEURITIS 787 

of polyneuritis due to alcohol affecting the arms to a much greater extent 
than the legs. Here the special localization was apparently due to the occupa- 
tion of the man — that of coachman. 

In severe cases the muscles of the trunk may be implicated, so that rising 
from a recumbent posture, turning, and sitting become difficult or impossible. 
Oppenheim 1 has made explicit reports concerning the implication of the 
abdominal muscles in neuritis and polyneuritis, of which little had previously 
been known. This condition may be a partial phenomenon of general poly- 
neuritis, and was so regarded in a case of probable post-typhoid polyneuritis 
in a young man, aged 18. This was a patient from the private practice of 
Professor Oppenheim, and he was under my observation. Paralysis of the 
abdominal muscles was bilateral, it especially distressed the patient on cough- 
ing, sneezing, and sitting up, and persisted longer than the other symptoms 
of polyneuritis. Eoss also reports having seen loss of power in the abdominal 
muscles in advanced cases of polyneuritis. In other cases reported by Oppen- 
heim paresis of the abdominal muscles was due to herpes zoster, malaria, and 
enteric fever ; once, apparently after the chronic abuse of alcohol, he observed 
isolated neuritis of some of the intercostal nerves. 

The occasional implication of the phrenic nerve in the neuritic process is 
of special prognostic importance. Strumpell first noted this in alcoholic poly- 
neuritis, in which it was revealed by the cessation of diaphragmatic respiration; 
with every inspiration the epigastrium was deeply retracted. Paralysis of the 
diaphragm is usually an ominous symptom, but Oppenheim once saw the bilat- 
eral phrenic paralysis of alcoholic polyneuritis disappear in a month and com- 
plete recovery take place. 

Among the cerebral motor nerves, the vagi are most often attacked; occa- 
sionally the laryngeal branches are the seat of such changes (implication of 
the recurrent laryngeal in cases reported by Minkowski, Kast, Roth, Rennert, 
and Sorgo). On the other hand an acceleration of the pulse to 120-150 
beats per minute is relatively common in all forms of polyneuritis; and the 
relation of this disease to an affection of the pneumogastric nerve is con- 
firmed by necropsy. I shall later describe the symptoms of diphtheritic poly- 
neuritis which are referable to the vagus, but naturally other causes of tachy- 
cardia must also be considered. Serious as the symptom is, it has been known 
to disappear. Much more rare is a decided bradycardia. 

Paralysis of the ocular muscles of neuritic genesis is much more common 
in alcoholic polyneuritis than in other forms. Among the latter we frequently 
note unilateral paralysis of the abducens, more rarely unilateral paralysis of 
the oculomotor nerve. That even complete paralysis of all the external ocular 
muscles, combined with disturbances on the part of other bulbar nerves 
(difficulty in deglutition), may be due to polyneuritis is taught by a case of 
Damron-Meyer, in which anatomical examination revealed complete degenera- 
tion of all the nerves of the ocular muscles, also of the hypoglossal, ' glosso- 
pharyngeal, facial, and some of the spinal nerves, while the brain trunk was 
in normal condition. 

In alcoholic polyneuritis, paralyses of the ocular muscles are not uncom- 

i Oppenheim, " Abdominaler Syrnptomencomplex bei Erkrankungen des unteren Dor- 
salmarks." Deutsche Zeitschr. f. Nervenheilkunde, XXIV, p. 330. 



788 NEURITIS AND POLYNEURITIS 

mon. Here we may see unilateral, complete, or partial oculomotor and abdu- 
cens paralyses, but their neuritic character is uncertain. Some of them are 
due to inflammatory changes in the region of the nuclei of the ocular mus- 
cles; in alcoholics we find an inflammation which was first described by Wer- 
nicke as polioencephalitis superior, the chief local symptoms of which are 
paralysis of the ocular muscles; combined with polyneuritis this has been 
demonstrated anatomically by Jacobaeus and others. We will be particularly 
suspicious of a polioencephalitic basis when instead of complete or partial 
paralysis of one nerve, we find the so-called glance (Blicklahmung) paralysis, 
a paralysis of the muscles which draws the bulbus to one side (for instance, 
it may affect the right external and the left internal muscles which are inner- 
vated by different nerves, but which have a common center of innervation in 
the trunk of the brain). We have no positive guide for the differentiation of 
peripheral neuritic and central polioencephalitic paralysis of the ocular mus- 
cles. Nystagmus has several times been observed in polyneuritis, usually of 
alcoholic origin. The condition of the internal ocular muscles is most impor- 
tant in the diagnosis of pohmeuritis, especially alcoholic neuritis. In poly- 
neuritis we must first determine whether there is true reflex pupillary rigidity 
— i. e., arrested reaction to light with normal reaction to convergence — and 
whether it is permanent. 

Among 1,000 cases of chronic alcoholism Uhthoff found reflex pupillary 
rigidity with retained reaction on convergence in only 10, and in these it was 
doubtful whether the pupillary rigidity was persistent and unchanged; there- 
fore it must be regarded as rare. Pupillary rigidity of this form has also been 
observed in alcoholic polyneuritis (cases being reported by Gudden, Dresch- 
feld, Rennert, Xonne, Thomsen, Oppenheim, and others). But in two of 
Kramer's x cases of polyneuritis in which this symptom appeared, the reac- 
tion was normal after recovery took place. Reflex pupillary rigidity is evi- 
dently an unusual symptom in alcoholic polyneuritis; moreover, it disappears 
much more often than in tabes dorsalis which is the affection with which it 
must be contrasted in the differential diagnosis. Differences in the size of 
the pupil, as well as ophthalmoplegia interna which appear to occur in chronic 
alcoholism as well as in alcoholic polyneuritis, are much more common. In 
other forms of polyneuritis changes in the size of the pupils are seldom 
observed. 

The other motor cranial nerves are rarely implicated in polyneuritis. Only 
facial paralysis is common, and this may be either unilateral or bilateral. 
Facial paralysis may even be the first symptom of the affection, but, as a 
rule, it appears at the acme of the disease, and in cases which, from their 
development, must be considered severe. According to statistics at hand, the 
percentage of cases of facial paralysis occurring in alcoholic polyneuritis is 
comparatively small. Quite exceptionally we note difficulty in mastication and 
deglutition ; a paralysis of the soft palate is most apt to occur in diphtheritic 
polyneuritis. Bilateral paresis most severely affecting all of the muscles of 
mastication was noted in a singular case reported by Gaspero (Monatsschrift 
fur Psychiatrie und Neurologie, XIV, p. 161). In some cases an impaired 
movement of the tongue has been observed (with corresponding neuritic 

i Kramer, " Riickenmarksveranderungen bei Polyneuritis," Breslau, Juli, 1902. 



POLYNEURITIS 789 

changes in the hypoglossal nerve). Pastrovich saw isolated neuritic hypo- 
glossal paresis in an alcoholic. 

The paralysis of polyneuritis is of degenerative nature. It runs its course 
with atrophy and changes in electric contractility which are manifest as DeR; 
the tendon reflexes are abolished in the paretic muscles. All of these symp- 
toms correspond to the conditions described in mononeuritis. Atrophy in- 
variably follows paralysis, and depends upon its degree. Edema, which is not 
rare, occasionally masks the atrophy. The muscles are flaccid and soft, and 
extremely painful to pressure. In the later stages a distinct hardness, a kind 
of induration, is occasionally noted as an expression of connective tissue pro- 
liferation in the atrophic muscles. In this stage appear the contractures to 
which I shall later refer. In the earlier stages passive movements are free 
in so far as that they are not prevented by pain. 

The electric contractility is here of the greatest diagnostic importance. In 
daily practice, especially in the acute period of the disease, we should be con- 
tent with ascertaining the most important facts, as the suffering patient is 
often unable to endure the painful and fatiguing processes of a minute elec- 
trical examination, especially children in whom even a superficial test can 
be only made with the utmost difficulty. As a rule we find DeR, and, in 
accordance with the fact that paralysis in polyneuritis is usually incomplete, 
partial DeR is more common than the complete. The most marked changes 
are found in the distal regions, in the small muscles of the foot, the exten- 
sors of the foot and toes, the small muscles of the hand, and those supplied 
by the radial nerve. Besides the characteristic sluggishness of contraction in 
the reaction of degeneration upon direct galvanic irritation, occasionally we 
note mere absence of electric contractility, which, however, may be due to 
the accompanying cutaneous edema. Rare reactions of degeneration (indirect 
sluggishness of contraction, faradic DeR) are also observed, but they are of 
little diagnostic interest; also in regions which are functionally less severely 
implicated, there is only a simple quantitative diminution of contractility, but 
we must be cautious in utilizing this finding in cases of bilateral paralysis. 
The demonstration of electric changes in contractility is often easier than 
that of atrophy, although the former symptom invariably follows the paralysis 
by one and a half or two weeks. 

The fact is of great interest that changes of electric contractility may 
occasionally be demonstrated in muscular regions in which functional damage 
has never been apparent. Remak l reported a case of general neuritis in a 
servant girl, in whom by electricity severe changes were demonstrated in the 
facial nerves which had never been paralyzed. The author assumed the ana- 
tomical foundation of this to be the periaxial neuritis of Gombault. Similar 
cases were previously and subsequently reported by Kahler and Pick, Hosslin, 
and Bernhardt. In alcoholic polyneuritis I have occasionally demonstrated 
changes in the electric contractility of the non-paralyzed muscles supplied 
by the radial nerve, but this finding is probably most common in saturnine 
polyneuritis (see below). As in mononeuritis electric contractility returns 
long before the restoration of function. 

The tendon reflexes are usually abolished. Sometimes we find them ar- 

i Remak, I. c, p. 355. 



790 NEURITIS AND POLYNEURITIS 

rested or pathologically diminished in areas in which apparently neither the 
power furnished by the muscles nor sensation are pathologically affected. 
Under such circumstances, particularly in diphtheritic polyneuritis or diabetic 
polyneuritis, the knee-jerk may be absent ; in alcoholic polyneuritis, Westphal's 
symptoms may be present without paralysis, DeR, disturbance of sensation, 
or ataxia in the course of the crural nerve. This symptom, therefore, may be 
the only one which reveals the further distribution and generalization of the 
neuritic process, and for this reason such cases must be considered as on the 
border-line between neuritis and po^meuritis. I saw a man, aged 40, an alco- 
holic and presenting the ordinary signs of chronic abuse of alcohol, who showed 
degenerative right-sided paralysis of the peroneal nerve with corresponding 
disturbances of sensation, DeR, and atrophy of the extensors, yet in whom 
the right patella tendon reflex was abolished while both Achilles reflexes were 
present. The power of the quadriceps was quite normal. When the upper 
extremities are implicated in the paralysis, the tendon reflexes are absent. 
That the tendon reflexes form a fine test for very slight neuritic changes and 
those in the reflex arc is shown by the foregoing reports and by the fact that 
after complete restoration of function they may long be absent, even for years, 
yet finally reappear. 

The Madness of the tendon reflexes in areas affected by polyneuritis, as 
well as in mononeuritis, is extremely rare, but exceptionally it has certainly 
been observed. I have seen such a case. Occasionally these reflexes show a 
pathologic change. For instance, Westphal noted a crossed abductor reflex 
while the patella reflex was absent, and I saw a similar coincidence in a typical 
case of arsenical polyneuritis. 

Objective sensory disturbances are usually less marked than paralytic phe- 
nomena, both in intensity and extent. There is a slight diminution of the 
faculty of perception, and it can be determined only by very close examina- 
tion. It is usually combined with hypesthesia and hypalgesia, which con- 
spicuously involve the distal portions of the extremities, being most severe in 
the toes, and gradually disappearing as it passes upward through the foot and 
leg; in the upper extremities the fingers, especially their tips, are most mark- 
edly implicated while the hypesthesia gradually disappears centrally. Such 
slight disturbances of sensation in the fingers are not uncommon. They 
may be the only symptom of the disease in the upper extremity. Occasion- 
ally we find merely paresthesias, without objective disturbances to prove the 
extension of the polyneuritic process. More rare than a disturbance in motion 
is a disturbance of sensation which appears to affect only one nerve, but affects 
this in toto. Such cases are certainly exceptional. 

As to the nature of the disturbance, all forms of sensation may be uniformly 
decreased. Characteristic combinations are noted, especially that of esthesia 
on contact with hyperalgesia. Sometimes it is impossible for the patients to 
find words with which to express their peculiar sensations, which are outside 
the realm of physiology. They speak of a " dull pain," but this is much 
more unpleasant than that produced by the priek of a pin (anesthesia dolo- 
rosa) ; hypalgesia combined with hypersensitiveness to contact is also noted, 
and hyperesthesia plays an important role among the sensory disturbances of 
the polyneuritic. In the various nerve disturbances the changes in sensation 
may also vary greatly so that examination reveals a manifold and ever-chang- 



POLYNEURITIS 791 

ing picture. The sensation of position as well as that of deep sensation may 
be markedly disturbed, and this usually corresponds to the change in the sen- 
sation of vibration. Synchronous with these is a derangement of the power 
of motion to which I shall refer later — ataxia. 

It must still be stated that a retardation of the conduction of sensation is 
found among polyneuritic sensory disturbances. This was formerly thought 
to belong only to spinal processes, particularly tabes, but my experience leads 
me to believe — what is anatomically confirmed — that in polyneuritis there is 
a slowing of the conduction of sensation and also, in the absence of irrita- 
tion, of contact and pain sensation {double sensation). I have several times 
observed this among my patients. 

In a small number of cases we find no trace of objective disturbance of 
sensation, even on careful examination. On the contrary, there are some in 
which sensory disturbance and pain are so marked that these have been con- 
sidered a purely sensory form of polyneuritis. I shall presently describe these 
cases, in which ataxia is usually prominent (acute polyneuritic ataxia). The 
cutaneous reflexes, as a rule, show a decrease of sensation, being either weak 
or abolished ; in hyperesthetic regions they may be increased. When deep sen- 
sation is severely affected, ataxia necessarily follows, and shows all the char- 
acteristics of sensory ataxia, particularly a distinct increase on closing the eyes. 
Romberg's symptom, which is one of the typical features of well-marked 
polyneuritis, is the first expression of this disturbance. When ataxia is promi- 
nent, the gait also is of characteristic type; it becomes stamping, uncertain, 
the legs diverge widely. In milder grades of ataxia the movements of the 
hands and fingers are similarly impeded; the patients are clumsy in the exe- 
cution of all fine movements, as in writing, picking up small objects, in dress- 
ing, in buttoning their clothes. 

Neurotabes Peripherica. — Cases in which ataxia dominates the clin- 
ical picture, and in which the symptoms of paralysis are secondary deserve 
special mention. Such cases were first described by Dejerine in 1884 as a 
special form of polyneuritis which he designated neurotabes peripherica. 
Remak quite properly declared this designation to be improper and proposed 
for this form the more suitable term of ataxic polyneuritis; a strict differen- 
tiation is impossible, for there are many transitional stages between this and 
" am} T otrophic " polyneuritis. There appear to be no special etiologic factors 
to account for the ataxic elements in the symptom picture. The affection 
is most common in alcoholics, but it is also noted in arsenical, diabetic, rheu- 
matic, and post-infectious polyneuritis. It is generally admitted that in post- 
diphtheritic polyneuritis ataxia is the most marked symptom. 

In this condition there is occasionally no evidence of weakness, which rarely 
happens in cases of other etiology. No matter how prominent the ataxia may 
be, careful investigation will usually disclose some defect in motion, and we 
should first direct our attention to the usual points of predilection of poly- 
neuritic paralysis — the muscles supplied by the peroneal nerve. Minute elec- 
trical examination often aids us in the diagnosis, which is obscure in those cases 
in which paralysis and atrophy are uncertain, and in which positive changes 
of electric contractility denoting partial DeR are not recognizable. 

When ataxia is more prominent we occasionally find spontaneous move- 
ments, which, in the toes and fingers, usually appear as slight flexion, exten- 



792 NEURITIS AND POLYNEURITIS 

sion, or abduction, and are sometimes jerking, sometimes slow. They may- 
be painful, but are usually imperceptible to the patient. I shall not here dis- 
cuss the pathogenic foundation of ataxia. The question whether or not the 
ataxic movements now so frequently observed in polyneuritis are of spinal 
origin calls for special consideration, and I shall subsequently refer to this 
point. 

Among the nerves of special sense, the optic nerve not infrequently shows 
changes, especially in alcoholic neuritis, the primary ones being pallor of the 
temporal half of the papilla and optic atrophy or optic neuritis. These changes 
are the sign of a retrobulbar optic neuritis, and usually accompany the dis- 
turbances in function so characteristic of this affection; we find a marked 
diminution of the central acuity of vision, and relative or central scotomata 
for white and colors. What relation these changes bear to the polyneuritic 
process is doubtful, because they are found also in chronic alcoholism without 
other conspicuous neuritic change. Uhthoff found among 1,000 alcoholics no 
less than 139 with pallor of the temporal half of the papilla. Optic neuritis 
has also been observed in spontaneous, puerperal, and carcinomatous polyneu- 
ritis (Remak, Schanz, and Miura). Gaspero (I. c.) found choked disc in his 
case (previously mentioned); the head of the optic nerve showed a dark, 
grayish-red discoloration, it was edematous and prominent, the arteries indis- 
tinct, and the veins congested. There were no proliferative processes nor did 
hemorrhages ever appear. All of the phenomena ameliorated. This case must 
be considered an example of intense inflammatory change at the entrance of 
the optic nerve (papillitis), since choked disc is occasionally (although ex- 
tremely rarely) found also in multiple sclerosis, while in this instance, as in 
polyneuritis, both optic atrophy and optic neuritis were present. 

Strumpell reported a case, long considered unique, of a nervous defect in 
hearing which occurred in the course of polyneuritis, and which he attributed 
to neuritis of the auditory nerve. Alt 1 recently published a case of alcoholic 
polyneuritis combined with nervous disturbances of hearing which disappeared 
with the polyneuritic symptoms. At the same time he reported three other 
cases in which there were symptoms of intoxication of the auditory nerve due 
to immoderate smoking. In Meyer's case 2 deafness appeared on the third day 
with general symptoms of fever and, at the same time, disturbances in equilib- 
rium. Neuritic symptoms (paresis and loss of the power of coordination) 
developed in the course of the fifth nerve and in the extremities. All of the 
symptoms disappeared except the deafness. Findings on examining with the 
ear speculum were negative. There was a diminution in the perception of 
high tones and the perception of bone conduction; subjective noises in the 
ear, as elsewhere, must in the main be attributed to the nervous origin of the 
defective hearing. 

Bladder disturbances are not a feature of the ordinary picture of poly- 
neuritis. On the contrary we hold it true that when severe bladder disturb- 
ances are persistent multiple neuritis is unlikely. On the other hand, it must 
be admitted that mild and transitory bladder disturbances do now and then 

1 Alt, " Ueber Erkrankungen der Hornerven nach iibermassigem Genuss von Alkohol 
und Nikotin." Wiener med. Wochenschr., 1903, p. 210. 

2 Meyer, " Ein Fall von multipler Neuritis mit besonderer Betheiligung des Acus- 
ticus und Trigeminus." Monatsschr. f. Ohrenheilk., 1903, Nr. 2. 



POLYNEURITIS 793 

appear. We must, however, disregard those cases of delirious, unconscious, 
insane, or very marantic patients in whom these bladder symptoms occur 
immediately before death. Oppenheim saw a case of severe polyneuritis in 
which the catheter had to be used for the first five or six days, after which 
the bladder disturbance disappeared while polyneuritis developed still further, 
but finally terminated in recovery. In the severe cases of polyneuritis reported 
by Kramer there was temporary incontinence of urine and difficult micturi- 
tion, v. Leyden long ago pointed out the possibility of such an occurrence 
because of two cases under his observation. Therefore this symptom and a 
certain weakness of the rectum must be regarded as rare in the clinical pic- 
ture of polyneuritis. It is at present uncertain whether we can assume neu- 
ritic lesions to be the anatomical foundation of these disturbances or such 
changes in the posterior column of the spinal cord as have been observed in 
polyneuritis with increasing frequency in the last few years. 

Among vasomotor, secretory, and trophic disturbances edema is most com- 
mon. I refer to true edematous swelling in the distal portions of the extrem- 
ities (back of the foot, the ankle, the back of the hand), pitting persisting 
for a long time after the pressure of the finger, but decreasing while at rest 
in bed. This can be regarded as a symptom of neuritis only after all other 
factors (a complicating cardiac affection, nephritis, or cachexia) have been 
excluded. If there are no such complications, the edema can be referred to 
implication of vasomotor nerves, and to this we also attribute the other vaso- 
motor symptoms which occasionally appear, such as cyanosis, erythema, attacks 
of syncope, and local asphyxia. Trophic disturbances are sometimes combined 
with vasomotor symptoms, such as glossy shin, which is much rarer than in 
traumatic neuritis. In another article I have expressed myself concerning the 
doubtful relations of Raynaud's disease and erytJiromelalgia and need not here 
repeat what was stated. I shall merely emphasize that perforating ulcer of 
the foot (mal perforant) is not a symptom of polyneuritis which is of any 
importance in the differential diagnosis from tabes; on the contrary, it is a 
condition occurring in chronic alcoholic intoxication. Adrian 1 refers the 
changed composition of the blood in such cases to a neuritic genesis. In two 
cases of Bonhoffer's 2 , extensive pemphigus-like vesicles appeared in different 
areas and became gangrenous, even those not subjected to pressure, and they 
were regarded as the direct cause of death. In a case of Oppenheim's, lupus 
developed after the onset of polyneuritis and persisted after the latter affec- 
tion had disappeared. In a case which we shall later study as an example 
of incomplete recovery, Frankel saw multiple granulation tumors the nature 
of which he could not understand, but which he did not attribute to neuritic 
changes. There are cases in which both vasomotor and trophic disturbances 
are very prominent ; chief among these is a form of beri-beri regarded as poly- 
neuritis. Similar and isolated cases have been observed when there was no 
endemic polyneuritis, and these were described as dropsical polyneuritis. 

Secretory disturbances, particularly hy panhidrosis, are not rare in any 
form of polyneuritis, but are especially marked in arsenical polyneuritis. It 
is doubtful what position we can assign to the arthritic swellings which appear 

i Adrian, " Das Mal perforant," Jena, 1904. 

2 Bonhoffer, " Die aeuten Geisteskrankheiten der Gewohnheitstrinker," Jena, 1901. 
52 



794 NEURITIS AND POLYNEURITIS 

in the various stages of polyneuritic. When they develop as multiple, painful, 
arthritic effusions, accompanied with fever and preceding the neuritic symp- 
toms, they are generally regarded as belonging to acute articular rheumatism 
and as etiologically important in the development of the neuritic affection. 
The solitary or multiple arthritic swellings occurring at the height of the 
affection must be regarded as symptoms of polyneuritis, but their relations 
are by no means clear, and their strict division into the two groups mentioned 
can by no means always be made. I recently saw a case of amyotrophic plexus 
neuritis in an elderly lady, in whom a painful effusion into the shoulder- joint 
had occurred and closely simulated a neuritic affection. 

Mental disturbances appear in all forms of polyneuritis, but are most com- 
mon in alcoholic polyneuritis. This, as I have stated, may develop from a 
preceding and ordinary but acute delirium tremens, which runs a typical 
course in a few days. But the critical sleep which usually terminates an 
attack of delirium may not supervene. The delirium persists while the hallu- 
cinations become less real, and with steady progress new psychopathological 
phenomena appear, a pathologic condition which was first described by Korsa- 
kow under the name of neuritic psychosis or cerebropathia psycliica toxcemia. 
As Jolty suggested, we now usually designate this as Korsakow's syndrome. 
Bonhoffer * gives us a clear description of this psychosis. After the introduc- 
tory stage of delirium, stupor follows, or a slow but progressive impairment 
of memory. Defective memory is characteristic of the psychosis, inability to 
associate places and scenes, and " confabulations." The impairment of mem- 
ory may be extreme. The time of day, the date, and all that concerns the 
patient himself, are forgotten in a few moments. Syllables and numerals 
are also forgotten in the shortest time. Not only is memory defective for 
occurrences during the time of the disease, which is readily explained on other 
grounds than by a loss of memory, but it almost invariably applies also to 
circumstances of previous months or years {retroactive amnesia). Patients 
may forget that they are married, or that they have children. They believe 
themselves to be again living in times long past, and are lost to their present 
surroundings. Finally loss of memory is closely related to the symptom of 
confabulation. The patients attempt to cover the gaps in their memory by 
all sorts of excuses, at the same time spontaneously relating adventurous tales 
which bear a close resemblance to their distorted dreams. In spite of their 
fantastic and extravagant descriptions the absence of emotion is remarkable. 
In contrast to these serious disturbances, the power of reflection is often well 
retained in these patients, and their deportment is excellent. These symptoms 
may remain at their acme for weeks or months. As a rule, they disappear more 
slowly than the neuritic symptoms. According to Bonhoffer, whose experiences 
relate only to the alcoholic form of this psychosis, recovery is never complete; 
the patients are no longer able to follow their occupations, memory is de- 
fective, and it seems impossible for them to take the initiative in anything. 
Other authors have reported a more favorable outcome. 

This psychosis is noted also in other forms of polyneuritis which are not 
of alcoholic nature. It has been described after septic, post-typhoid, tuber- 
culous, gastrointestinal and post-innuenzal polyneuritis. Oppenheim saw a 

i Bonhoffer, I. c. 



POLYNEURITIS 795 

case in which the continued use of pyramidon was the only apparent cause. 
The relation between the neuritic and mental symptoms is not always marked ; 
the former may be less prominent, or even absent. 

The symptoms which have been described may be combined in a single 
case in manifold and varying ways. Their sequence and development may 
be more or less rapid, and may show various limitations, yet these may be 
neither so positive nor conspicuous that a classification based upon them would 
be of value. 

Leyden divided the long course of the affection into four stages: the 
initial stage, the progressive stage when the paralysis is distributed, the stage 
of acme, the process being arrested at its height, and the stage of regeneration. 

The initial stage, especially in the not too severe cases of alcoholic neuritis, 
may be prolonged through weeks or months. As, in the case I reported, it is 
often difficult to differentiate the sensory irritative phenomena and the gen- 
eral weakness, especially of the legs, from the more common symptoms of 
chronic alcoholism, and to ascribe them to a beginning polyneuritic process. 
In marked contrast to this, the mental phenomena of delirium tremens, or 
Korsakow's psychosis, may appear acutely and clearly mark the onset of the 
affection. Severe constitutional and gastrointestinal symptoms, fever, ano- 
rexia, vomiting, diarrhea and lassitude may mark the onset of the affection 
in the alcoholic as well as in other forms of the disease. A fulminant onset 
usually prefigures a similar course. The paralysis attacks the distal portions, 
usually of the lower extremity, in rapid succession, one member after another 
being attacked, both arms and legs soon becoming incapable of function. Both 
the recumbent posture and the power to turn around become impossible, and 
respiration is impeded. Symptoms also appear in the cerebral innervation. 
There is paralysis of the muscles, of the face, of deglutition, of speech, and 
of the eyes, and, even at the beginning of the second week, more often after 
a longer interval, respiratory and cardiac disturbances are the prelude to 
death. Some of these cases have been considered to be Landry's paralysis, and 
Eichhorst described such a case as acute progressive neuritis. Xotwithstand- 
ing these alarming symptoms, in spite of the implication of the pneumogas- 
tric, the phrenic and the facial nerves, etc., I maintain that at any moment 
the affection may be arrested and recovery ensue. 

The more protracted cases in which the paralysis is of slighter extent are 
more common. Here, at least in the early stages, pain and hyperesthesia are 
often prominent in the clinical picture. The paralysis is but slowly progressive, 
and halts before it decidedly involves the muscles of the trunk and the bulbar 
portions. Katurally its progress is not always continuous ; the neuritic process 
may attack isolated proximal areas, both spinal and cerebral, may cause paral- 
ysis of the phrenic, pneumogastric, or facial nerves, without implicating the 
entire musculature of the extremities and trunk. According to Eemak, its 
duration varies from two weeks to about two months, and during this time the 
prognosis is always grave. Aside from the localization of the paralysis, which 
in itself may be a menace to life, the prognosis depends also upon the general 
condition of the patient. If he is weak from a preceding infectious disease 
(enteric fever, sepsis, influenza, etc.), or if chronic intoxication is the founda- 
tion of the process (alcoholism, diabetes, etc.), or if, as so often happens, a 
complication such as tuberculosis is present, the prognosis is more serious, 



796 NEURITIS AND POLYNEURITIS 

and the patient perishes in this or the next stage. Bladder disturbances are 
of unfavorable prognostic import inasmuch as they show the extension of the 
process or the implication of the spinal cord. From its transitory nature, the 
cystitis is not very serious, nor is there much danger of the bed-sore which is 
so common in complete paraplegia, although to this rule there are exceptions 
(the eases reported by Bonhoffer). 

A polyneuritis which affects the distal extremities in general, as well as 
other forms with less widely distributed and distinct phenomena (abortive 
forms, rudimentary cases with varying localization) offer a favorable progno- 
sis. But in the majority of cases the course is very protracted. After the 
paralytic phenomena become well marked, they usually persist for a long 
time and until the pain disappears — often from two to three months or longer ; 
very favorable cases terminate in three months. In the more severe cases the 
deformities and contractures of which I have spoken develop subsequently. 
Pes equinus, the inevitable result of the paralysis, which more frequently 
affects the extensors of the foot and toes than the flexors, may become chronic, 
for the muscles of the calf are forced to sustain permanently an excessive 
weight. Moreover, the fibrous degeneration of muscles may cause retraction. 
As in the cases above reported, we were several times enabled by palpation to 
demonstrate such a fibrous hardening of the muscles. Oppenheim reported 
an ossification of the joints and tendons. Contractures may also develop in 
the knee-joints (flexure contracture), in the joints of the upper extremities 
(drop-wrist), and an attempt must be made from the onset to prevent these 
by a proper position and braces, and the early use of passive movements. 

The great majority of cases end in recovery, but in severe cases this may 
require years. Not only do the paralysis and atrophy slowly yield, but also 
the pain on pressure as well as on active and passive motion. Sometimes 
hyperesthesia disappears but tardily. It has been previously stated that the 
reappearance of the tendon reflexes and of normal electric contractility is 
frequently long delayed. The paralyses usually disappear in an inverse se- 
quence from that in which they originated. When recovery is incomplete, 
those distal regions which were first and most severely attacked, are the ones 
which are the seat of residual paralyses and anesthesia; although this rule is 
by no means without exception. On the whole incomplete recoveries are 
rare, and they naturally are most common in the toxic, especially the alco- 
holic, forms of neuritis in which the effect of the intoxication continues. I 
will now quote from Frankel {Deutsche med. Wochenschr., 1896, Nr. 45) 
the history of a patient whose recovery was incomplete, this case being also 
mentioned by Oppenheim in his text-book (page 529). 

Case 23. — The patient states that, in the year 1894, he suffered from a severe poly- 
neuritis of the arras and legs which implicated also the left half of the face. Now, 
after the lapse of ten years, he applies for treatment on account of weakness, con- 
tinuous pain, and paresthesia in the legs. Both feet show pes equinus, the toes in 
the position of marked plantar flexion. The leg is emaciated, the right knee-jerk is 
distinct, the left feeble, and the Achilles tendon reflexes are absent. The muscles 
of the leg and their nerve trunks are sensitive to pressure, particularly the left peroneal 
nerve, which appears to be thickened. On the left side the sciatic phenomenon is 
present. The dorsal flexors of both feet act imperfectly, but retain some power. The 
region of the peroneal nerve shows a marked decrease in contractility; there is no 
DeR and no hypesthesia, but merely hyperesthesia plantaris. The grasp of the hand 



POLYNEURITIS 797 

is somewhat weak. In the left facial nerve we note a conspicuous paresis and con- 
tracture, also constrained movements and a diminution of the electric contractility. 

Another case, which I saw in the private practice of Professor Oppenheim, 
recovered, but there was paresis of the muscles of the abdomen (post- typhoid 
polyneuritis) ; paralysis of the ocular muscles may persist as well as other 
conditions. 

The prognosis is rendered less favorable by the somewhat common re- 
lapses, and we must omit from consideration those cases in which the contin- 
ued action of a deleterious factor may cause another attack (alcoholic and 
lead neuritis). Such relapses may occur after several months or after years; 
a case was reported in which this happened after 11 years. Cases are also 
reported in which the affection appeared annually at about the same time 
(Oppenheim). 

The preceding description of the symptomatology applies mainly to that 
of alcoholic polyneuritis; I have not, however, wholly confined myself to this 
form, but have included the spontaneous, rheumatic, and infectious forms 
and have now and then described others. Concerning some of these I must 
be somewhat more explicit, partly because they are of practical importance, 
and partly — with the same fundamental symptoms — they vary so much as to 
merit a special description. 

Arsenical Polyneuritis. — This is symptomatologically analogous to the 
form described. It may appear after the administration of a single large dose 
with suicidal or murderous intent, or by accident, but it may also develop after 
the prolonged effect of the poison taken medicinally or from chronic intoxica- 
tion caused by living in rooms covered with arsenical wall-paper, handling 
artificial flowers, etc. Midler has lately asserted that this affection is rela- 
tively common among so-called arsenic eaters who were formerly supposed 
to be immune to arsenical paralysis. A few years ago arsenical neuritis was 
widely prevalent in England. It was traced to a combination of glucose with 
sulphuric acid containing arsenic, which was so largely used in the manu- 
facture of beer that even moderate beer-drinkers were poisoned. Many Eng- 
lish investigators (Reynolds, who discovered the admixture of arsenic in the 
beer, Ross, Buzzard, and others) reported these cases, and enriched our knowl- 
edge of this affection. Bordas reported that no less than 4,181 persons, prin- 
cipally in Manchester and its vicinity, were poisoned, and that at least 300 
died. Some authors ascribed the intoxicating effect to the selenium present 
in the sulphuric acid or the beer. The quantity necessary to produce intoxica- 
tion varied decidedly. Some were attacked after the consumption of only 
small quantities, and some infections — influenza, for instance — were markedly 
predisposing. It is significant that arsenical neuritis has often been observed 
after treatment by arsenic (usually the administration of Fowler's solution in 
chorea). Karplus and Kinsman saw symptoms after the administration of 
about 50 grams of Fowler's solution. 

Case 24. — A woman, aged 40, on the 21st of November, 1903, took arsenic in 
unknown quantity with suicidal intent. Soon afterward severe gastrointestinal symp- 
toms appeared, such as vomiting, diarrhea, and cardiac palpitation. After this acute 
gastroenteritis had run its course — i. e., after 8 days — the patient complained of sensa- 
tions of cold and formication in the calves, followed by a dull sensation in the hands 
and feet, severe tearing pain and increasing weakness in the legs and arms. The 



798 NEURITIS AND POLYNEURITIS 

hands could not be used for the finer manipulations. Walking was irksome, and only- 
possible with support on both sides. The affection reached its acme in January, and 
then gradually receded. There were no bladder symptoms. On the 4th of June, 1904, 
the following conditions were noted: there was no disturbance of the cranial nerves, 
and no muscular atrophy in the upper extremities; the nerves and muscles were 
insensitive to pressure, the tendon reflexes were preserved, the motor power was good, 
the sensation on the volar surface of the finger to brush contact was diminished, the 
left knee-jerk markedly decreased, the right abolished, as well as the Achilles tendon 
reflex. There was swaying on closing the eyes, and pressure upon the muscles of the 
calf and the peroneal and posterior tibial nerves was very painful. In both legs the 
muscles supplied by the peroneal nerves were weak, especially in the left, and the flex- 
ors of the feet were by no means normal. There was bilateral hypesthesia and hyper- 
esthesia on the dorsum of the foot ; no ataxia. Electric examination revealed partial 
DeR in the peroneal region and in the flexors of the toes. Hands and feet showed a 
persistent and abnormal secretion of sweat; there were no conspicuous deformities. 

The patient presented the typical symptoms of arsenical polyneuritis in the 
stage of improvement. When the poisoning is acute a period of 8 to 10 days 
or even of a few weeks elapses before the appearance of the neuritic symptoms. 
Exceptionally, as in a case of Kron's, 1 after minor intestinal symptoms, pain 
and paralysis appear within 24 hours after the poisoning. In a case of Kor- 
zou's, severe pain set in after 3 days. The sequence of the symptoms is invari- 
ably the same: paresthesia, pain, paralysis, atrophy. The paralyses show a 
predilection for the distal portions of the extremities — the peripheral portions. 
The legs are most seriously affected, but the arms are more frequently impli- 
cated than is the case in alcoholic neuritis. In our patient the paralysis had 
disappeared from the arms when we saw her the first time. The small mus- 
cles of the hand are first attacked, then the extensors, perhaps also the long 
flexors of the fingers. In a case of arsenical polyneuritis which I recently 
saw, the small muscles of the hand as well as those innervated by the radial 
nerve were severely implicated. As in the case just reported, the extensors of 
the legs were chiefly affected, especially the extensors of the toes. Electric 
examination showed that, as in the case reported, the flexors were not exempt. 
The absence of the knee-jerk almost invariably points to the extension of the 
neuritic process. An increase of the knee-jerks at the onset has also been 
observed. In severe cases paralysis, atrophy, and DeR — all three of which are 
combined — are more extensive. Kron's patient could neither cough nor exert 
abdominal pressure. In this patient incontinence and weakness of the blad- 
der appeared and were so persistent that for three months she had to be 
catheterized ; there was also incontinentia alvi. The cerebral nerves are usu- 
ally not implicated, even in severe cases, and this fact may aid us in the diag- 
nosis of Landry's paralysis when the course appears to be a rapidly ascending 
one. Cardiac palpitation here, as in a few other cases, was attributed to an 
implication of the pneumogastric nerve. Recovery occurs according to the 
rule above mentioned, that the muscles last paralyzed first regain their motil- 
ity. When the paralysis is persistent and recovery is complete, contractures 
may appear in the hands and feet. In the majority of cases there is retraction 
of the flexors of the feet, especially the toes, and in the hands and fingers 
(Erlicki and Rybalkin). Exceptionally a flexor contraction has been observed 
in the knee-joint. 

iKron, "Ein Fall von Arseniklahmung." Neurol. Centralbl, 1902, p. 930. 



POLYNEURITIS 799 

Sensory irritative symptoms and those due to absence of function are promi- 
nent in the clinical picture. The pains are often of extreme intensity, the 
sensitiveness of the nerves and muscles to pressure is very conspicuous and 
long-continued. In addition to the hyperalgesia, and even without this, 
objective sensory disturbances are reported in the majority of cases, the distal 
portions being most severely implicated, and the proximal less so. 

In the case of a woman with arsenical poisoning a year after the appear- 
ance of the neuritis, the involvement of the hands was shown only by hypes- 
thesia and hyperhidrosis. Ataxia could not be demonstrated in our patient, 
at least while under observation, but it appears to have been an earlier symp- 
tom. In my recent case it was conspicuous, affecting both arms and legs, a 
so-called static ataxia. On the whole it is very common, but is usually not 
so marked as the paralytic phenomena. Yet the inverse may be true. Ataxia 
may precede the paralysis, which for a long time may be but slight. The 
clinical condition then resembles tabes {pseudo-tabes arsenicosa), but there 
is no danger of confounding these diseases if sufficient attention be given to 
the development, the sensitiveness to pressure, and the absence of bladder 
disturbances. 

The frequency of vasomotor, secretory, and trophic disturbances is re- 
markable. Hyperhidrosis is reported as extreme in many cases. In a case 
of Jolly's there was conspicuous glossy skin. Herpes zoster from the medi- 
cinal employment of the drug (arsenic) has been observed as an isolated 
symptom after neuritis. Erythromelalgia has been frequently reported, par- 
ticularly by English authors. Alopecia, abnormal pigmentation, changes in 
the nails, erythema, and edema have all been observed. Eeynolds attaches 
great significance to keratoses of the skin and to pigmentation which are 
often observed in arsenical paralysis. 

Psychical disturbances are more rare than in alcoholic neuritis, but Korsa- 
kow's symptom-complex is occasionally observed, as well as impairment of 
memory and similar conditions. In some respects the prognosis of arsenical 
polyneuritis is favorable. Among 130 cases Alexander saw only 2 who had 
permanently lost the power of walking. A few cases were fatal (pneumonia, 
paralysis of the heart), 1 but the course may be greatly protracted. In Kron's 
severe case, walking and even standing were impossible after the lapse of two 
years, but electric treatment instituted at this time brought about marked 
improvement; a permanent cure and incomplete restoration to health with 
defects varying in degree have repeatedly been observed. The diagnosis is con- 
firmed by the demonstration of arsenic in the urine, the hair, and the nails. 

Lead Paealysis. — Lead paralysis deviates in many ways from the picture 
of ordinary polyneuritis. It is a common affection, probably always the result 
of chronic intoxication, which in the majority of cases is acquired from the 
occupation. It is most frequently seen in painters, typesetters, lacquerers, 
plumbers, workers in accumulator (storage batteries) factories, etc. In con- 
sequence of the great amount of lead used in the last mentioned occupation 
the danger of intoxication is said to be particularly great at first, and the 
disease appears with extraordinary rapidity (Guillain et Lhermite). There 
are many other occupations which occasionally expose people to lead poison- 

i The cases in the English epidemic were much more unfavorable. 



800 NEURITIS AND POLYNEURITIS 

ing, for instance, those of workers in artificial flowers, paper glazers, and 
weavers among whom poisoning by lead was formerly common, but now 
under more favorable hygienic conditions is much less so. Intoxication has 
also occurred from the ingestion of water which had been conducted through 
lead pipes, from an admixture of lead in flour (Weber), in children, from 
playing with toys containing lead (Variot), from cosmetics and from the 
use of snuff containing lead, etc. 

Lead paralysis is scarcely ever the first sign of poisoning. Among the 
symptoms which, in the individual case, are often valuable in diagnosis I 
must mention the lead line on the gums, pallor of the face, tremor, muscular 
and arthritic pain; also objective findings such as reports of a single attack 
of lead colic or repetitions of it; finally symptoms of general functional 
nervous weakness and irritability, and severe cerebral symptoms (lead encepha- 
lopathy). It must be borne in mind that many patients are attacked by lead 
paralysis without ever experiencing an attack of lead colic. In comparison 
with other symptoms of lead poisoning, such as colic, myopathy, and neuras- 
thenia, lead paralysis is much less common. Oppenheim reports a case of 
" hereditary " lead paralysis in a child. 

Lead paralysis owes its special prominence to two circumstances : First, 
the absolute insignificance of sensory irritative symptoms and those due to 
loss of function; second, to the conspicuously elective character of the 
paralysis. The predilection of some muscles to, and the exemption of others 
from, the disease is more striking than in the preceding cases of poly- 
neuritis. Remak therefore designates it as elective systematic neuritis or poly- 
neuritis. 

In typical cases we find the following picture: A patient whose occupa- 
tion has for a long time exposed him to the effects of lead, and who shows 
signs of chronic lead intoxication, reports to the physician that he has noticed 
a weakness in the hands, especially of the right, which has slowly increased. 
He has no pain or numbness. Examination shows degenerative paralysis in 
the extensors of the hand and fingers. The extensor digitorum communis 
appears to be the earliest and most severely implicated, especially the parts 
extending to the second and third fingers. The remainder of the extensor 
digitorum communis, the indicator (index finger), and the extensores pollicis 
are next affected. As the process advances the extensors of the hand are 
involved, the extensores carpi radiales usually prior to the extensor ulnaris. 
The characteristic " drop-wrist " appears. In grasping there is marked volar 
flexion of the hand, and its power is more or less decreased. Last among the 
muscles of this group the abductor pollicis longus is implicated. On the other 
hand — and this is of the greatest diagnostic importance — the supinators, espe- 
cially the supinator longus, as well as the triceps, are exempt in typical cases. 
Thus the paralysis reveals a distinctly selective action among the muscles sup- 
plied by the radial nerve. In the severe cases, which are not rare, more mus- 
cles are involved in the paralysis, and the small muscles of the hand sup- 
plied by the median and ulnar nerves are implicated. First the interossei, 
particularly the first interosseous muscle, and the muscles of the ball of 
the thumb are affected; then, as a rule, prior to the implication of the ab- 
ductor pollicis longus, another muscle, the deltoid, is paralyzed, although only 
partially. 



POLYNEURITIS 801 

Case 25. — The patient is a painter, aged 35 ; he has a lead line upon the gums, 
has suffered from repeated attacks of lead colic, and for three months paralysis has 
gradually been involving both hands and arms. The extensors of the finger and thumb 
are completely paralyzed. The extensors of the hand show but little power, the abductor 
pollicis longus is somewhat stronger, but there is a distinct wrist-drop. The extensor 
muscles of the forearm are atrophic, only the supinator longus becoming prominent 
on flexion, and showing normal power. The interosseous spaces are depressed, particu- 
larly the first, and the ball of the thumb shows atrophy. The power of the correspond- 
ing muscles as well as that of the abductor pollicis longus is decreased. It is evident 
that he cannot fully raise his arm, and partial DeR is especially distinct in the deltoid 
as well as in all of the paretic muscles. The disturbance is more marked in the right 
arm, but shows no sensory changes. 

As in our patient, lead paralysis invariably appears as a degenerative form 
of atrophy. DeR is occasionally noted in muscular regions in which neither 
paralysis nor atrophy can be demonstrated. From this type of paralysis there 
are, upon the whole, very few deviations. It may be that paralysis and atrophy 
are more prominent in the small muscles of the hand, developing simulta- 
neously with the paresis of the extensors and to a similar degree. Usually a 
special cause is evident, primarily the over-use of the muscles in the occupa- 
tion (the toxico-professional paralysis of Oppenheim). An affection of the 
muscles of the ball of the thumb has been reported by Gowers, Bernhardt, and 
Remak. Another uncommon type is that involving the upper arm, first re- 
ported by Remak. Then come the rare cases in which the deltoid is impli- 
cated, the other Erb's muscles, the biceps, the brachialis internus, and even 
the supinator longus and brevis. But these forms of paralysis are added to 
an already existing extensor paralysis. A saturnine, total paralysis of the 
plexus has been described by Ladd. 

In adults the lower extremities are usually exempt. In 98 cases Remak 
saw these affected only thrice ; the muscles supplied by the peroneal nerve are 
frequently, although not always, attacked while the tibialis anticus is unaf- 
fected, Koester saw a very unusual localization of the paralysis and atrophy 
in the interossei and the abductores hallucis so that a daw-foot appeared. It is 
remarkable that in all the lead paralyses of youth the lower extremities are 
implicated, and show the peroneal type just described. 

The bulbar nerves are usually exempt. But cases have been described of 
paralysis of the vocal cords in which there was either unilateral or bilateral 
posticus paralysis, or a total paralysis of the recurrent laryngeal. Persistent 
acceleration of the pulse has been mentioned as a symptom referable to the 
vagus; paralysis of the spinal accessory as an isolated saturnine neuritis. 
Retrobulbar neuritis of the optic nerves with the same symptoms which appear 
in alcoholic neuritis has frequently been reported, as well as paralysis of the 
ocular muscles from lead. Some of the symptoms attributable to the cerebral 
nerves are difficult to differentiate because they occur in a so-called enceph- 
alopathia saturninu, a full description of which is impracticable in this arti- 
cle. Therefore the neuritic nature of these paralyses should always be demon- 
strated — a proof several times lacking in some facial paralyses which have 
been reported (Bewy, Debove). Finally, there is a general saturnine polyneu- 
ritis, of which but few cases are known. Some of these were marked by a 
febrile onset and a most acute course. Even the muscles of the trunk were 
implicated, and there was paralysis of the diaphragm with fatal asphyxia. 



802 NEURITIS AND POLYNEURITIS 

From the onset there was tearing pain, which is usually absent, but there 
were no objective sensory disturbances. 

Typical lead paralysis yields, certainly ,the first attack, in the course of 
several months, or it improves decidedly. The extensors of the fingers, par- 
ticularly in the second and third fingers of the right hand are the last to 
regain their power, and even in the first attack these may remain paretic. 
If the patient, as often happens, again exposes himself to lead intoxication 
there is a relapse of the paralysis, and in these relapses the prognosis is always 
unfavorable. Even without renewed exposure to the poison, relapses may 
occur from the effect of other deleterious agents (alcoholism, over-exertion). 
The therapeutic indications are obvious. Sulphur baths and potassium iodid 
have long been employed to promote the rapid elimination of the poison. 
Otherwise the treatment, especially the electric, is the same as for other forms 
of polyneuritis which I shall describe in detail. This is also true of the diag- 
nostic considerations provided there is no special etiology. Here we find 
in the characteristic localization an extremely valuable proof of the saturnine 
etiology. The absence of sensory disturbances is also of great significance. 
It becomes necessary to distinguish this from certain forms of chronic, sub- 
acute, cervical poliomyelitis which, at least occasionally, produce a picture 
resembling lead paralysis, but which subsequently runs an entirely different 
course. Careful search must be made for the occasionally concealed source 
of the intoxication, and the signs of chronic lead poisoning must be consid- 
ered. Of course, atypical cases can be explained only when the etiology is 
known. 

There are other forms of toxic polyneuritis which, in comparison with 
that described, are of secondary importance. Gowers reported after the use 
of silver pills a paralysis resembling the lead form. A few cases of copper 
neuritis in copper workers have been observed, but Lewin doubts that copper 
exerts a toxic action. Phosphorus, whose toxic action is so evident in other 
regions, is rarely dangerous to the peripheral nervous system; but Leyden 
and Henschen have reported such cases. In Henschen's 1 last case there were 
merely symptoms of sensory irritation and slight impairment of function. 
Lowenfeld describes neuritic phenomena after taking creosote containing 
phosphorus. Erbsloh automatically proved a polyneuritis after the admin- 
istration of sulphonal, but carcinomatous cachexia and hemorrhage were also 
mentioned in the history. 

Mercurial polyneuritis, which we shall discuss with syphilitic polyneuritis, 
is of somewhat greater interest. It is certain that working with mercury 
does not generally cause neuritic phenomena. Whether polyneuritis can be 
produced by the use of mercury administered as a drug is difficult to decide 
because this treatment is usually instituted in syphilitics, so that at least 
two etiologic factors for neuritis are present. There are, however, a few cases 
in which the etiologic influence of mercury cannot be doubted (cases of 
Ketly, Spillmann, Etienne, Spitzer, 2 and others). In these instances either 
there was no syphilis (corrosive sublimate was taken with suicidal intent in 

i " Ueber Phosphorneuritis." Neurol. Centralbl., 1900, p. 555. 

2 Spitzer, " Em Beitrag zur Kenntniss der mercuriellen Polyneuritis acuta," 
Deutsche Zeitschr. f. Nervenheilk., Bd. XIX, p. 215. 



POLYNEURITIS 803 

Ivetly's case, and by accident in Faworski's), or the discontinuance of the 
mercurial treatment, as in Spitzer's patient relieved the polyneuritis while 
the luetic manifestations persisted throughout the entire body and even fresh 
symptoms developed. Brauer considers the experimental foundation upon 
which Letulle and Heller attempted to base mercurial polyneuritis unreliable. 
Syphilitic polyneuritis, the differentiation of which from the mercurial form 
is, for reasons, above mentioned, most perplexing, is proven in a few cases 
(Schultze, Oppenheim, Cestan). 1 When its syphilitic nature is recognized, we 
can exclude all other etiology (alcohol, mercurial poisoning, autointoxication 
from a syphilitic, hepatic, or renal affection). In one of Cestan's cases poly- 
neuritis appeared -i weeks after a chancre, in the other 6 weeks afterward; 
mercury was beneficial. In a few other instances, mercury aggravated the 
patient's condition, therefore this is not an absolutely certain indication of 
the etiology. Polyneuritis cannot be regarded as an actually specific disease, 
but as due to the toxins generated by syphilis. Neither the symptoms of 
mercurial nor of syphilitic polyneuritis absolutely prove its etiology. In the 
secondary stage of syphilis we sometimes observe a facial mononeuritis which 
we attribute to a similar syphilo-toxic origin. 

Carbon bisulpliid poisoning, which has been much studied in the last few 
years, may in conjunction with other nervous disturbances, partly functional 
and partly of organic, cerebral origin, produce neuritic and polyneuritic 
symptoms; but the number of these cases is small and in comparison with 
the other phenomena of poisoning the svmptoms of neuritis are secondary. 
Experiments (Koester) 2 have shown that the poison has little affinity for the 
peripheral nervous system. No neuritic phenomena can be demonstrated 
in the rabbit after the inhalation of carbon bisulphid ; on dipping the paw 
of the animal into a solution made from this agent, changes are apparent in 
the affected part, but we are not certain of their neuritic nature. 

Neuritic and polyneuritic phenomena are more common after carbonic 
oxid intoxication. This poison produces various localized pathologic phe- 
nomena in the nervous system as well as in the vessels and the skin (edema, 
bed-sores, abscesses, and gangrene). Remak assumes that the symptoms on 
the part of the peripheral nervous system are circumscribed perineuritic proc- 
esses originating from the vessels, to which those of neuritis are subsequently 
added. The paralysis of carbonic oxid poisoning is peculiar in that.it shows 
a predilection for the members which are exposed to pressure during the 
asphyxia, hence traumatic factors appear to be also operative. That there are 
exceptions to this rule is evident from the fact that paralyses appear in the 
course of nerves in which there is no possibility of pressure (for instance, 
in paralysis of the ocular muscles which, as in a case of Mezkowski's, may be 
combined with polyneuritic symptoms). It is noteworthy that this paralysis 
occurs immediately after the poisoning, and is apparent as soon as the person 
comes out of coma. It is usually confined to a nerve or plexus region. When 
nerve paralysis is multiple, the affected nerves of the side subjected to pressure 
present the signs of hemiplegia as well as of neuritis. Isolated forms of 

i Cestan, "La polynevrite syphilitique." Xour. Ieonogr. de la Salpctriere, 1900, 
p. 153. 

2 Koester, " Zur Lehre von der Schwefelkohlenstoffneuritis." Arch. f. Psych., 
Bd. XXXIII. 



804 NEURITIS AND POLYNEURITIS 

toxic neuritis have been noted after poisoning by toluol and nitrobenzol. I 
have now enumerated the most important of the toxic forms of neuritis, and 
must reiterate that the etiology is frequently a mixed one. Various external 
poisons, particularly lead and alcohol, may be combined, and, what is even 
more common, the intoxication may affect a portion of the body already dis- 
eased. Here tuberculosis has a paramount influence. Tubercular alcoholics 
are especially predisposed to neuritis. Inversely, as I have several times stated, 
alcoholic neuritis predisposes to other infections. 

Diphtheritic Polyneuritis. — Of the many forms of infectious and 
post-infectious polyneuritis that have been considered, there are a few which, 
on account of the substantive position they occupy, I must describe somewhat 
more in detail: these are diphtheritic polyneuritis, leprous neuritis, and beri- 
beri. While the other post-infectious forms of polyneuritis differ but little 
from the original infection, the conditions are different in these three forms, 
the most important and most common of which is diphtheritic polyneu- 
ritis. 

Case 26. — A school-teacher, aged 25, had in November, 1904, an attack of diph- 
theria which was treated with diphtheria antitoxin. After the wound had healed the 
patient noticed that his sight was affected, his vision being dimmed; he had previously 
observed a change in his speech, which had become nasal. Deglutition was difficult, 
cough appeared, and fluids were regurgitated through the nose. These disturbances 
improved, but to some extent persisted. Early in January, 1905, he noticed formication 
and numbness in his legs and arms. The legs became weak, particularly the right, 
and he subsequently had pains in the legs; there were no bladder disturbances. The 
gait was uncertain. The patient was easily fatigued, and felt himself in danger of 
falling. He was unable to recognize small objects distinctly by the sense of touch; 
as, for instance, on putting his hand in his pocket to take out money. It was impos- 
sible for him to put on his stockings or to know whether the stockings were on his 
feet without looking. On washing his face he could not distinctly feel his hands. 
Examination of the strong, robust-looking young man revealed the following conditions : 
Speech is distinctly nasal; deglutition of fluids is very difficult, causing cough, and 
the regurgitation of some of the fluid through the nose. The soft palate rises imper- 
fectly, and the palate reflex is weak but not absolutely abolished. Mastication, the 
movements of the lips, and of the tongue are undisturbed. Sensation is intact in 
the face. The pupils react promptly, the ocular movements are free, but there is a 
moderate paresis of accommodation. The tendon reflexes of the arms are arrested, 
but there is no atrophy of the muscles. The grasp of the hand is quite weak, and the 
apposition of the thumb is decidedly paretic on both sides. The nerve trunks are 
insensitive to pressure; besides the marked paresthesia of which the patient complains, 
there are secondary objective sensory disturbances. Pain, temperature, and tactile 
senses are unimpaired. The sensation of position is disturbed. The so-called vibration 
sensation is most distinctly affected, as well as the power of recognizing objects by 
touch, especially with the right hand. There is also a mild degree of ataxia. The gait 
is waddling and uncertain ; Romberg's symptom is noted. The knee-jerk is markedly 
diminished, and can be produced only with the aid of Jendrassik's grasp. The Achilles 
tendon phenomena are absent. The nerve trunks and the muscles of the leg are tender 
on pressure. The motor power of the leg is moderately decreased, but this is not strictly 
localized. The movements of the leg are slightly ataxic, even when the patient is in 
the recumbent posture. Superficially, sensation is not impaired; but deep sensation, 
especially that of vibration, is wholly lost. The pulse is slow and weak. The cutaneous 
reflexes are preserved. Electric contractility reveals nothing. 

If we had no knowledge of the etiology of this case we would at once 
make a diagnosis of polyneuritis, which differs somewhat though from the 
typical form of the disease. Instead of paralysis, ataxia is most prominent 



POLYNEURITIS 805 

(ataxic polyneuritis) , but the paralysis implicates definite structures, which 
are usually uninvolved, and we find paralysis of the palate, of the muscles of 
deglutition and of the pharynx, as well as paralysis of accommodation. These 
symptoms give to diphtheritic polyneuritis its characteristic stamp. They are 
so typical that this form of polyneuritis can usually be attributed to diph- 
theria. 

Post-diphtheritic paralysis attacks adults just as frequently as children. 
In its various forms it is by far the most common of the nervous sequels of 
diphtheria. Cerebral hemiplegia rarely follows diphtheria. Polyneuritis ap- 
pears to occur just as often in cases treated with as without injections of serum ; 
Slawyk noted it respectively in 5.5 and 5.3 per cent, of his cases. It must be 
borne in mind that, in the opinion of the great majority of physicians, many 
more severe cases of diphtheria are now cured than was formerly the case. 
AVoollacott regards the greater frequency of paralysis after serum treatment 
as the direct consequence of the decreased mortality. 

The first and most common symptom of paralysis following diphtheria 
is paralysis of the palate, which usually accompanies hypesthesia of the pharyn- 
geal mucous membrane and the loss of the soft palate reflex. It may appear 
at the end of the first week of diphtheria, but usually not until the beginning 
of the second week, or even after six or eight weeks. Very exceptionally the 
condition is unilateral. The soft palate becomes perfectly flaccid and droops; 
even reflexly there is no contraction, and this accounts for the nasal tones 
as well as the regurgitation of fluid through the nose. If the electric con- 
tractility of the soft palate is tested, we not rarely find partial DeR. Our 
case proves that such paresis of the soft palate may long persist, for even 
after three months it was quite distinct, although improvement had begun. 
In rare instances a longer time elapses before the paralysis yields. Remak 
saw a case of paresis which had existed for 2^ years recover under galvanism. 
In a case which Oppenheim saw in an idiotic child, the paralysis was per- 
manent. Paralysis of the soft palate may be the only paresis. This is prob- 
ably the most common condition; but in many cases other pareses follow, 
primarily of the muscles of the pharynx, the esophagus, and of accommoda- 
tion. 

The first is shown by impairment of the power of deglutition. The con- 
tents of the mouth find their way into the larynx ; this is partly due to 
hypesthesia of the entrance to the larynx. The disturbance in deglutition 
may even lead to deglutition pneumonia. Hypesthesia is caused by alterations 
in the superior laryngeal nerve, but the inferior laryngeal nerve also may be 
attacked, as is proven by the fact that paralysis of the vocal cords has been 
observed a few times. If the heart is affected, it is usually due to implication 
of the pneumogastric nerve, which is not rare in diphtheria. We may assume 
this if the damage to the heart does not occur during the first days of the 
infectious disease, but appears subsequently with the other symptoms of 
paralysis. Usually there is bradycardia and irregularity of the pulse; later 
we frequently note tachycardia. This implication of the heart is of the great- 
est importance in the prognosis, as will be shown later. Paralysis of accom- 
modation is as certain an indication of diphtheria as is paralysis of the soft 
palate ; as a rule, it occurs after the latter, and disappears sooner. It is revealed 
by a sudden disturbance of sight, a characteristic of which is that the pupillary 



806 NEURITIS AND POLYNEURITIS 

reaction is always retained, and nothing anomalous is noted in the pupils. 
On the other hand, although much more rarely, there may be paralysis of 
the external muscles of the eye, usually of the abducens, either unilateral or 
bilateral. Oculomotor paralyses are more seldom seen. Optic neuritis also 
has been observed in diphtheria (Botton). 

The cases in which we observe the foregoing signs of paralysis of the 
soft palate and of the muscles of deglutition and accommodation, perhaps 
also the signs of implication of the heart, have been grouped as localized 
forms of diphtheritic paralysis, and they have been differentiated from those 
in which the paralysis affects other nerve regions, these having been regarded 
as generalized or polyneuritic paralyses. But there is no sharp line of demar- 
cation between these two groups. In the cases in which the paralysis is ap- 
parently localized, the extension of the pathologic nervous phenomena is 
occasionally indicated by symptoms which are not subjectively apparent but 
can be determined only objectively. Foremost among these is the absence of 
the tendon reflexes in the lower extremities, which may appear with the paral- 
ysis, and without any other disturbance of motion or of sensation. Of course, 
this is not an invariable rule; in many cases disturbances appear in the legs 
after paralysis has fully developed, or even when the patients have v begun to 
improve. Their gait becomes uncertain, they are soon fatigued, and this 
cannot be reconciled with their improved general condition. Then pares- 
thesias appear, usually first in the legs, then in the arms, as well as the other 
sensory disturbances which were so characteristic in our patient. 

Statistics show that the arrest of the knee-jerk is a common sign. Among 
51 cases of diphtheritic paralysis, Eothe found this condition in 15 — while 
ataxia of the legs were noted in only 9, and paralysis much less frequently. 
Therefore the absence of the knee-jerk may be the only sign of a general 
condition. Remak found Westphal's sign to be much more common, for 
among 78 cases of diphtheritic paralysis of the soft palate, the knee-jerk was 
abolished in 40. As in my patient, we often note that the knee-jerk is not 
absolutely lost but only greatly decreased, and that it may be evoked by Jen- 
drassik's grasp. In the overwhelming majority of cases the disappearance of 
the knee-jerk follows paresis of the soft palate; but, according to Baginsky, 
the disappearance of the patella tendon reflex on one or both sides is the 
first symptom of diphtheritic paralysis. Since the Achilles tendon reflexes 
have been more accurately studied, we can demonstrate their absence in this 
stage of diphtheritic polyneuritis. The tendon reflexes may be abolished for 
a long time, and this symptom may persist months after all other signs of post- 
diphtheritic paralysis have yielded. 

In addition to the disappearance of the tendon reflexes, in some cases other 
symptoms are noted. The most common is ataxia, which is of the usual type : 
the gait is uncertain and stamping, the movements lack coordination, and this 
is especially marked on closure of the eyes, but may even be demonstrated 
when in the recumbent posture. Romberg's sign becomes distinct. Uncer- 
tainty of motion may also be apparent in the upper extremities, especially 
in the hands ; the ordinary finer movements of writing, buttoning the clothes, 
counting money, etc., become impossible. Sensory disturbances, either sub- 
jective or objective, may be combined with the ataxia. The first to appear 
are numbness, tingling, and formication, especially in the distal parts of the 



POLYNEURITIS 807 

legs and arms; these were described by our patient. Objective disturbance 
is shown less by the temperature and pain sense, than by that of contact, 
particularly the sense of position, and that of deep sensation. As an expres- 
sion of this bathyanesthesia, there is a marked decrease or absence of vibra- 
tory sensation; this was noted in my case as well as in many similar ones. 
This sensory disturbance renders the patient incapable of recognizing objects 
by the touch (astereognosis) which was also true of my patient. Although 
in these cases the ataxia is usually regarded as sensory, yet, as Eemak main- 
tains, there is often a disproportion between the sensory disturbance and the 
ataxia, the former by no means always being demonstrable. A delay of pain 
conduction has rarely been observed. There may be no pain or it may be 
present, and pain has been repeatedly observed on pressure over the nerve 
trunk and muscles, although it is not an invariable symptom. The finer 
details of all of these sensory disturbances can be more clearly demonstrated 
in adults than in children. 

The implication of the muscular apparatus becomes more and more ap- 
parent as general weakness, most marked in the distal portions of the extremi- 
ties, and by a circumscribed paralysis which bears the characteristic stamp 
of degenerative paralysis — atrophy, flaccidity of the muscles, qualitative 
changes in electric contractility. Among 32 cases Eemak found only 9 such 
pareses, and these in a decreasing ratio were noted in the peroneal muscles, 
the ilio-psoas, the ulnar and median region, in the deltoid and the muscles 
of the nape, usually without decided electrical changes. In severe cases the 
paralysis may reach an extreme degree, and make the patient absolutely un- 
able to move. Then severe disturbance in the muscles of respiration impli- 
cates the thoracic muscles; paralysis of the diaphragm may be caused by the 
involvement of the phrenic nerve, after which the respiration becomes 
dyspneic. In a case of Epstein's 1 there was apnea necessitating artificial 
respiration for 11 hours; but in spite of repeated attacks of dyspnea the child 
finally recovered. An extremely severe case of post-diphtheritic paralysis is 
recorded in Hansemann's report of his own case. After passing through a 
series of infections, on the eighteenth day of an attack of diphtheria he was 
seized with paralysis of the soft palate, and tachycardia appeared. There 
was absolute motor paralysis of the arms and legs with severe sensory dis- 
turbances, also impairment of motion in the lips, the tongue, and the muscles 
of mastication which are usually exempt from paralysis. The ocular muscles 
were also involved, and sensory disturbances appeared even in the region of 
the fifth nerve. Smell and taste were blunted. Similar severe cases have 
several times been reported by Baginsky. He maintains that there is no rule 
concerning the electric contractility of the implicated nerve, for contractility 
may be intact or wholly lost without furnishing a sign of special value in 
either a diagnostic or prognostic respect. But we must remember that in these 
ill children an accurate electrical test is scarcely ever possible. In cases much 
less severe we have found it necessary to desist from an electric examination, or 
to limit ourselves to superficial and brief exploration lest we do harm to these 
unruly patients. As a rule, the electro-diagnostic laws otherwise operative are 
here also binding. The functions of the bladder and rectum are usually un- 

i Deutsche med. IVocliensclir., 1900, Xr. 41. 



NEURITIS AND POLYNEURITIS 

disturbed, but exceptions have been reported. As rare symptoms I must men- 
tion that Eeman found auditory neuritis in diphtheria, and Bolton reported 
a case of optic neutritis. Therefore we note that the entire nervous system 
may finally be implicated. Notwithstanding these exceptions we must bear 
in mind the regular cases which are extraordinarily characteristic. 

The duration of the AFFECTION depends upon its extent. General 
paralysis runs as slow a course as in other forms of polyneuritis, hence the 
affection may persist for many months, but unless death intervenes complete 
restoration to health almost invariably follows. The course of post-diph- 
theritic ataxia is usually shorter — from a few weeks to months — and in local- 
ized paralysis it is even briefer. 

The prognosis depends mainly upon the involvement of definite nerve 
and muscle regions and the paralysis. Deglutition paralysis may become dan- 
gerous, especially if there is marked anesthesia and suspension of the laryngeal 
reflex; also when food or fluid enters the respiratory passages and thereby 
directly produces pneumonia, which may lead to death. Patients of this kind 
should be most cautiously fed by means of the stomach-tube; perhaps even 
rectal alimentation may be necessary. Implication of the phrenic nerve is 
always a serious symptom, but, as Epstein's case shows, hope must not be too 
soon abandoned, and artificial respiration, possibly inhalations of oxygen, 
should be resorted to. 

The state of the heart calls for special attention. Patients with cardiac 
disturbance, no matter whether myocarditic or neuritic (this cannot always 
be clinically differentiated), should be kept in bed, and in severe cases absolute 
rest should be enjoined. This should not be interrupted, not even for the 
administration of nourishment, or for the voidance of the dejecta, etc. Of 
course, the patient should be sedulously guarded from mental disquiet. The 
heart must be sustained by the ordinary stimulants, and its action kept regular 
and full. I cannot here discuss the other complications and dangers arising 
from diphtheria. The paralysis should be treated on the same general prin- 
ciples as other paralyses and will be described later. In localized paralysis 
of the soft palate and the muscles of deglutition, electricity sometimes gives 
immediate results (production of the galvanic deglutition reflex by direct 
stimulation of the soft palate by means of button-shaped electrodes) ; 
but, as has been stated, this form of paralysis is usually relieved spontane- 
ously. 

Diphtheritic paralyses occur in other than the pharyngeal forms of diph- 
theria; namely, in diphtheria of the intestine, of the vulva, and of the navel 
in the new-born. In a case of diphtheria of the umbilicus (Kussmaul) there 
was at first paralysis of the abdominal muscles, which was interesting in a 
pathogenetic sense. In a wound of the forearm infected by diphtheria, Op- 
penheim noted paralysis of accommodation before the general paralysis. I 
shall later discuss a few points relating to the pathogenesis. The question 
as to whether the foregoing and typical symptom-complex occurs without a 
preceding diphtheria is difficult to answer. In children who are not closely 
watched it sometimes happens that an affection of the throat is hardly noticed, 
and only the subsequent paralysis recalls the fact that the child had such an 
affection. This corroborates the opinion I formerly expressed that post- 
diphtheritic paralysis bears no relation to the severity of the original affec- 



POLYNEURITIS 809 

tion. Merklen and Broc x report a case in which there was intense stomatitis 
and angina tonsillaris which by bacteriologic investigation were positively 
proven to be of non-diphtheritic character, yet paralysis of the soft palate, 
tachycardia, paralysis of accommodation, and dyspnea followed. A few 
similar cases were previously published. Eisenlohr reports cases of infectious, 
multiple neuritis in which there was no diphtheria but an implication of the 
muscles of the palate and of deglutition which resembled diphtheritic paral- 
ysis; this is not usually observed in polyneuritis. Paralysis of the soft palate 
and of the muscles of the larynx has been observed even after erysipelas 
(Oppenheim), as well as after variola (Remak). 

Beri-beri or Kakke. — In the Dutch East Indies, Japan, and Brazil, a 
disease occurs endemically which in other regions is usually sporadic; this is 
designated as beri-beri or kakke, and, owing to its main features, it has been 
included among the forms of neuritis multiplex. The etiology of this affec- 
tion, which is still obscure, will be briefly sketched. Various theories have 
been suggested, some assuming the disease to be an infection, the pathogenic 
cause of which is a microorganism that lias apparently been demonstrated. 
The adherents of the infectious theory claim that the deleterious agent which 
acts upon the nerves is the toxin furnished by these bacteria; others regard 
it as an alimentary (a ptomaine) intoxication. Some incline to the view that 
the poison is generated in spoiled rice, or that a pathogenic bacillus developed 
in this food. Others attribute it to the consumption of fish which is so gen- 
eral in Japan. Still other hypotheses have been proposed; for instance, that 
the disease is due to the contamination of rice by arsenic, or that it depends 
upon malaria. Xo unanimity of opinion has been attained. The question 
of the etiology remains unsolved; even inoculation experiments (in apes, 
Wright, Brain, 1903, p. 488) have not enabled us to come to definite conclu- 
sions. The anatomical foundation has not been determined in all cases. It 
is certain that neuritic or polyneuritic changes are frequently found, but in 
autopsies recently performed in 5 fatal cases of beri-beri, Glogner 2 found 
chiefly a disease of the muscles. Although he does not deny that finer changes 
take place in the nerves supplying the muscles, he regards these as secondary 
and considers the entire affection to be a polymyositis. Rumpf and Luce 3 
declare that the affection is certainly a polyneuromyositis, since inflammatory 
processes are found in the nerves which supply the muscles. In the clinical 
picture the neuritic symptoms are most distinct: these are pain, paresthesia, 
sensitiveness of the nerve trunks to pressure, and hypesthesia. The tendon 
reflexes are at first increased, but later diminished or abolished. Paralysis 
primarily affects the peroneal region; the gait is the typical steppage gait. 
As the paralysis extends it reveals an ascending character. Except for the 
pneumogastric, the bulbar nerves are rarely attacked. The paralysis is often 
followed by atrophy, which is masked by edema. The electrical changes 
resemble those of polyneuritis. Bladder disturbances are rare. What gives 
to this form its special type is the marked involvement of the heart and vas- 
cular system. The patient soons complains of palpitation and pain in the 
cardiac region as well as dyspnea. Upon auscultation and percussion anom- 

1 Arch, generates de med., 1904, p. 1985. 

2 Glogner, Virchow's Arch., Bd. CLXLI, p. 389. 

s Runipf and Luce, Deutsche Zeitschr. f. Nervenheilk., XVIII, p. 63. 



810 NEURITIS AND POLYNEURITIS 

alous conditions are demonstrated in the heart. Edema is common, par- 
ticularly over the tibia and in the face; later it may appear in all the para- 
lyzed regions. Dropsy may be extreme. These cardio-vascular symptoms are 
attributable to changes in the pneumogastric and vasomotor nerves. A pri- 
mary myopathic change is considered by Rumpf and Luce to be the cause of 
the hard swelling and subsequent rigid contraction of the muscles. The 
course of the disease varies according to the form and the severity of the 
affection. If of the pernicious type death may occur in a few days, or, after 
the lapse of a few weeks or even months, the case may terminate either favor- 
ably or unfavorably. 

Leprous Neuritis. — I shall not here attempt a clinical description of 
leprous neuritis, the neuritic symptoms being only partial phenomena of the 
pathologic picture of leprosy. But the anatomical foundation of the leprous 
neuritis will be considered, since this represents a leading type of neuritis — 
namely, interstitial neuritis. 

Each variety of post-infectious polyneuritis which has been described in 
the foregoing pages possesses its own peculiar features. The other forms 
which occur in infectious diseases present only general symptoms of this 
affection, and their further description is unnecessary. Any infectious dis- 
ease may occasionally be the cause of polyneuritis, although the frequency 
with which this occurs varies. Polyneuritis is the most common after enteric 
fever and influenza. It has been observed also after scarlatina, pneumonia, 
whooping-cough, acute articular rheumatism, erysipelas, mumps, gonorrhea, 
and sepsis; in malarial regions even malaria plays an important role in its 
etiology. As has several times been shown by typical cases, localized neuritis 
may be the sequel of any of these infections. 

When Remak differentiates polyneuritis occurring during and after infec- 
tious diseases from those based upon a dyscrasia, he observes that this dis- 
tinction is by no means sharply defined. According to his view, among the 
forms which divide the two groups are those which appear in tuberculosis, in 
which it is questionable whether a toxemic-chemical virus or an autointoxi- 
cation is the cause. It is certain, however, that in the course of this disease 
degenerative neuritic processes frequently occur, which were first minutely 
described by Pitres and Vaillard, then by Joffroy, Striimpell and Oppenheim, 
and recently by Colella. 1 The cases of so-called latent neuritis with absence 
of clinical symptoms, in which only at the autopsy degeneration was found 
in the peripheral nervous system, do not interest us here; but there are a 
number of cases in which neuritic symptoms develop in a pronounced form 
intra vitam. It is often doubtful in how far the appearance of pain and 
hyperesthesia in the pathologic picture may be ascribed to neuritis or poly- 
neuritis. Here the diagnosis is positive only if, to the previously mentioned 
symptoms, there are added those due to absence of sensory or motor func- 
tion. The last named forms of amyotrophic polyneuritis sufficiently corre- 
spond to the well known classical picture of the affection. The weakness 
begins usually in the lower extremities, affecting the muscles supplied by the 
peroneal nerve, at times increasing to complete paraplegia. In the upper 

i Colella, " Studio sulla polinevrite tuberculare." Annali delta Clinica della mal. 
mente nervose, Palermo, 1903, p. 1. 



POLYNEURITIS 811 

extremity the small muscles of the hand are most frequently attacked. Of 
the bulbar nerves occasionally the pneumogastric, the facial and the phrenic 
are implicated. Paralysis of the ocular muscles is not observed. The sphinc- 
ters are, as a rule, intact. Subjective sensory disturbances are extraordinarily 
frequent, nor are those of an objective nature often absent (Francotte, Pal). 
The tendon reflexes are seldom produced. Colella expressly states that in 
his cases, as in other forms of polyneuritis, the patella reflex was absent or 
was so feeble that after 5 to 7 taps it ceased. In a few instances the patella 
reflexes were increased (Lesage, Decroly). Vasomotor, secretory, and trophic 
disturbances were frequent (cyanosis of the hands, edema, sweating, changes 
in the nails, etc.). The onset was usually chronic; only in very rare cases 
of pure tuberculous origin was it acute. This exclusive tuberculous etiology 
was present only in the minority of the cases; in the majority, as Eemak 
emphasizes, there was also alcoholism. It must be remembered that with a 
very insidious development of tuberculosis, polyneuritis occurs as a late com- 
plication. An excellent example of polyneuritis due to autointoxication is 
that form arising in diabetes. The following is a typical case : 

Case 27. — A man, aged 50, reported that for three years he had suffered from the 
symptoms of well developed diabetes (glycosuria of moderate grade, polydipsia, loss of 
weight, etc.). For a year there has been weakness of the fingers, which are constantly 
used in his occupation, and this condition has increased within the last three months, 
accompanied by numbness and pain in the legs. The gait has become halting and 
uncertain, with the legs wide apart, and the feet, particularly the external borders, 
droop. The nerves of the lower extremity are distinctly painful to pressure. The 
sciatic phenomenon is well developed bilaterally; both patella reflexes are very indis- 
tinct; the Achilles tendon reflexes are absent. There is decided weakness of the mus- 
cles supplied by the peroneal nerve, more so in the peroneus than in the tibialis anticus. 
Partial DeR can be electrically demonstrated in the peroneal muscles. Also, there are 
well developed contact anesthesia, painful irritation and changes of position in the 
peroneal region ; even in the recumbent posture a certain degree of ataxia can be 
determined. There is distinct swaying on closure of the eyes. The radial and ulnar 
nerves are somewhat sensitive to pressure, the interossei are markedly emaciated ; the 
ball of the thumb is flattened, and there is typical claw position of the left hand. 
Only slight power is furnished by the small muscles of the hand. A coarser sensory 
disturbance in the hands cannot be demonstrated. Here also there is partial DeR. 
The patient formerly drank a great deal of beer but has abstained from such beverages 
in recent years. 

This is a typical picture of amyotrophic polyneuritis, combined with well 
marked diabetes mellitus to which we may undoubtedly attach the greatest 
importance in the development of the affection, as the previous alcoholism 
does not play an immediate role. Besides the conditions occurring in poly- 
neuritis there are also other pathologic pictures due to diabetes which may 
be referred to an affection of the peripheral nervous system: primarily, true 
neuralgias, diabetic sciatica, and far more frequently than otherwise, neu- 
ralgia of the crural and obturator nerves. Further there is diabetic mono- 
neuritis, and even polyneuritis which may assume various forms. The signs 
of trophic degenerative paralysis are not always so prominent as in the case 
just described. Sometimes there are only sensory, irritative phenomena and 
those due to loss of function, perhaps also absence of the tendon reflexes. 
The following case is an illustration of such a form, and also shows the fully 
developed symptom-complex of diabetes. 



812 NEURITIS AND POLYNEURITIS 

Case 28. — The patient complains of paresthesia in the tips of the fingers, lancinat- 
ing pain, and a somewhat uncertain gait. There are no urinary complications. The 
Achilles tendon reflex is absent; the knee-jerk may be imperfectly produced with Jen- 
drdssik's grasp. The patient sways upon closure of the eyes. The nerves and muscles 
of the legs are not sensitive to pressure ; there is no atrophy, no paralysis ; but numb- 
ness and distinct analgesia are present in the distal portions of the leg. There is no 
anesthesia of the trunk. The pupillary reaction, the eye-ground, and all other con- 
ditions relating to the nerves of special sense are normal. 

At first glance there is a conspicuous similarity of this clinical picture 
with that of tabes. The differentiation of this pseudotabes diabetica from 
true tabes is often difficult, and sometimes impossible, especially if we remem- 
ber that melituria may be a symptom of tabes. As distinguishing factors the 
absence of pupillary rigidity, of bladder disturbance, of anesthesia of the 
trunk, and perhaps also of crises in this so-called pseudotabes diabetica, may 
be mentioned, while perforating ulcer of the foot occurs also in diabetes. 
Finally, there may be a combination of tabes and diabetic neuritis. Of the 
greatest diagnostic importance is the fact that in diabetes the knee-jerk may 
reappear with an improvement in the general condition, and it may also be 
absent without the occurrence of other neuritic symptoms. Therapeutically, 
anti-diabetic diet, which has frequently produced good results, is first to be 
considered. 

1 have already referred to the forms of polyneuritis developing in the 
course of syphilis, as well as to puerperal and parturitional neuritis. Gout 
probably, as a rule, gives rise only to neuralgia, rarely to mononeuritis. 
Remak, Epstein, and Grube observed the latter condition in the course of 
the brachial nerves. True polyneuritis, as emphasized by Remak, is exceed- 
ingly rare. Occasionally polyneuritis occurs as the result of cancerous 
cachexia, such cases having been reported by Oppenheim, Miura, and Fran- 
cotte. A case reported by Oberthiir * requires especial mention. Here the 
symptom-picture of an atypical peripheral neuritis resulted from carcinosis 
of the peripheral nerve terminations and muscles, and not, as is usual, from 
a toxicant. Oppenheim 2 first called attention to senile polyneuritis, a form 
of neuritis occurring in the aged. In a patient over 70 years of age there 
were gradually increasing disturbances which implicated sensation and move- 
ment of the hands and feet. Pain was absent or slight, but paresthesia was 
very conspicuous. The paralysis attained a high grade. The cerebral nerves 
were not involved. These symptoms are not observed in all cases of senile 
neuritis. In the cases reported by Stein, pain and hyperesthesia were most 
prominent; exceptionally, bladder disturbance and paralysis of the cerebral 
nerves have also been noted. The course may be favorable, although the 
danger of arteriosclerosis must always be borne in mind — a condition which 
is of decisive importance in the development of neuritic changes. It must also 
be remembered that the inverse condition may result from arteriosclerosis 
due to degeneration of the nerves (Lapinsky) — a point which we shall 
consider under pathological anatomy. On the other hand, neuritic changes 
may produce not only senile arteriosclerosis but also other forms of this dis- 

i Revue neurolog., 1902, p. 485. 

2 Oppenheim, " Ueber die senile Form der multiplen Neuritis." Berliner klin. 
Wochenschrift, 1895, p. 289. 



POLYNEURITIS 813 

ease. In the case of Schlesinger's there was an endarteritis obliterans of the 
vasa nervosum in a man aged 69 ; in a second case, a man aged 23, endarteritis 
obliterans and endarteritic gangrene occurred. Similar conditions have been 
reported by Joffroy, Achard, Dutil, and Lamy; and periarteritis nodosa was 
present in two cases of Kussmaul and Maier. In these patients positive 
neuritic symptoms were not always present. A case of polyneuritis due to 
endarteritis obliterans has recently been reported by Franceschi. 1 

Pathological Anatomy. — The pathological anatomy of multiple neuritis 
has for its object the demonstration of changes common to all the forms, be- 
sides showing the deviations in the numerous varieties of this disease. The 
most extensive material for investigation is contributed by alcoholic polyneu- 
ritis, from which we shall start, using the conditions for a general descrip- 
tion. Macroscopic examination of the peripheral nervous system at the 
autopsy reveals nothing of note. Rarely can we observe swelling or redness 
of individual nerves — a hyperemia — and this most readily in the acute in- 
fectious cases. In leprous neuritis there are spindle-shaped or globular swell- 
ings of the nerve which may frequently be seen with the naked eye, and may 
also be palpated intra vitam. Microscopically we observe mainly atrophy of 
the nerve fibers. These were first determined by Thomson and Lancereaux. 
Moeli, Oppenheim, Siemerling, Dejerine, and others have since increased 
our knowledge of these conditions. The atrophic processes in the nerve fiber 
implicate the axis cylinder as well as the medullary sheath. They are of 
varying intensity: from the mildest grades of a reduction in the medullary 
sheath they lead to almost complete atrophy and destruction of this substance 
and of the axis cylinder. According to the acuity and the stage of the proc- 
ess, alterations in the destroyed medullary sheath will best be seen by the 
Muller osmium method of staining, which gives a black tinge; at other times 
Weigerfs medullary stain or van Gieson's stain will produce better results. 
A 1 per cent, osmic acid solution is valuable to demonstrate these alterations. 
The histologic conditions do not differ from those degenerative processes de- 
scribed in mononeuritis, but the division of these changes is usually character- 
istic. As a rule, the peripheral muscular tranches are most severely implicated ; 
and here again, corresponding to the clinical symptoms, the peroneal nerve 
is largely affected. While, here and in the peripheral branches of the tibial 
nerve, in the large saphenous, and perhaps also in the radial and ulnar, the 
atrophy may be very marked, we find in the nerve trunks proximally situated 
numerous column fibers besides those that have degenerated. These differ- 
ences have been frequently demonstrated in the same nerve and have also been 
found in the cutaneous nerves, often with decided intensity. That the per- 
ipheral branches are most severely implicated is a phenomenon peculiar to all 
forms of polyneuritis. Various explanations have been attempted. It has 
been assumed that, on account of being farthest from the trophic center, 
their constituents suffer most quickly from any nutritive damage; also that 
they furnish the best point of attack for toxic, infectious, or rheumatic 
damage. 

In contrast to the atrophic-parenchymatous processes the changes in the 

i Franceschi, " Polynevrite ed arteriosclerosi del sist. nerv. centr. e perif." Riv. d. 
patol. nerv. e merit., 1903, 5. 



814 NEURITIS AND POLYNEURITIS 

connective tissue are secondary, particularly in neuritis due to alcohol and 
similar toxic agents, and only exceptionally do we find a marked increase 
of the perineurium (Oppenheim and Siemerling), which is then usually lim- 
ited to a few nerves. There is also an increase of the nuclei of the connective 
tissue, and even small hemorrhages, changes of the vascular walls, and exu- 
dates around the vessels occur (Eichhorst, Flemming). Figure 5, Plate III, 
from a patient who succumbed to a polyneuritis in the chronic stage, shows 
these atrophic processes distinctly. Nowhere are there signs of a fresh inflam- 
mation. Besides numerous normal nerve fibers — the preparation, taken from 
the trunk of the sciatic, was stained according to Pal with van Gieson's stain — 
we find the greater number of the medullary fibers severely damaged, a por- 
tion of these fibers and their axis cylinders having disappeared; of the re- 
mainder a greater magnification shows the nerve sheaths to be incomplete, 
thin, pale, partially destroyed, and the axis cylinders also changed, unevenly 
thinned and thickened. Figures 3 and 4, previously described, show these 
conditions distinctly. The endoneural and perineural connective tissues 
are moderately increased. In some transverse sections a greater richness in 
nuclei is simulated by a decrease in the nerve bundle due to connective tissue 
contraction. 

Changes are frequently found in the muscles. They need not be described 
as they are identical with those observed in mononeuritis. Their distribu- 
tion corresponds to that of the neuritic changes and thus conforms with clin- 
ical observation. The parallelism between clinical and neuritic or neuromyo- 
sitic phenomena is not always complete: For example, if paralysis of the 
diaphragm has been the cause of death the phrenic nerve may be found nor- 
mal, although there had been sufficient time for the development of neuritic 
changes. 

In paralysis due to alcohol the changes in the peripheral nervous system 
are the most prominent, but are not always solitary. The spinal cord is often 
implicated. In 43 cases Gudden found spinal changes 15 times. With the 
development of the more delicate methods of investigation this ratio has been 
increased. Nevertheless it must be distinctly emphasized that occasionally 
spinal lesions cannot he demonstrated even with the employment of Marchi's 
and Mssl's methods. In different cases very dissimilar changes may be de- 
termined. The intramedullary anterior and posterior roots are frequently 
degenerated, usually to a slight degree, this being best determined by the 
Marchi method, but sometimes atrophy is so pronounced that Weigert's medul- 
lary sheath staining is sufficient to demonstrate the change. On the other 
hand, the extramedullary roots often escape degeneration, 1 even when the 
intramedullary roots are damaged. The posterior columns of the spinal cord 
often show more or less severe degeneration, usually analogous to the changes 
of the posterior roots, while, less frequently, circumscribed inflammatory foci 
can be demonstrated. The remaining white substance is only exceptionally 
the seat of these changes (the cases of Cole). 2 The alterations which are 
found mainly in the anterior horn cells are usually slight, as a rule, showing 
changes of NissFs bodies, or, more rarely, vacuole formation in the cells. 

i Heilbronner, " Riickenmarksveranderungen bei der multiplen Neuritis der Trinker." 
Monatsschr. f. Psych., IV, pp. 1 and 81 and others. 

2 Cole, " Central Changes in Alcohol Neuritis." Brain, 1902, p. 326. 



POLYNEURITIS 815 

The cells of the spinal ganglion (Cole, I. c, Philippe and Eide), 1 and those 
of the corresponding cerebral ganglia (Burr) 2 have also been found changed. 
These are not all of the lesions found in the central nervous system. Bon- 
hoeffer found them in the radiating fibers which enter the cortex of the cere- 
brum; Cole in the fronto-thalamic fibers, in Betz's giant pyramids, etc.; 
inflammatory lesions have been demonstrated in the brain trunk (superior 
hemorrhagic polioencephalitis). In the clinical description we observed that 
symptoms may appear which extend beyond the picture of neuritis (psychotic 
phenomena, paralysis of sight). A portion of the central changes may ex- 
plain these signs, but it now appears certain that the majority of symptoms 
owe their origin mainly to alterations in the peripheral nervous system, which 
are the only constant ones; all others vary in intensity and extent and may 
be absent. 

The pathologico-anatomical foundation of other forms of polyneuritis due 
to chronic poisoning from metals is less definite and clear than that of alco- 
holic neuritis. In arsenical poisoning, investigations conducted singly 
(Henschen, Erlicki, and Rybalkin) have shown degeneration in the radial 
and peroneal nerves, and also in the anterior horn cells and in GolPs columns. 
In poisoning with mercury there is no anatomical basis. Experimental in- 
vestigations (Letulle and Heller), which have, however, been contradicted, 
would appear to confirm the clinical phenomena, in that they assume a neu- 
ritic foundation. C0 2 and carbon bisulphid poisoning associated with paral- 
ysis have not been satisfactorily explained by the pathological lesions. In 
lead poisoning, however, the lesions are better understood. Here the investi- 
gations at least permit a positive opinion in points of the greatest importance. 
As is clinically confirmed the changes in the peripheral nerves are usually most 
marked in the radial, but the previously mentioned atrophic changes are also 
found in other peripheral nerves. The peripheral branches are most severely 
injured but proximally the lesions rapidly decrease. Gombault and others 
have demonstrated, particularly in lead paralysis, a segmental periaxial neu- 
ritis, which has also been marked in other toxic and infectious forms. The 
spinal cord, as a rule, is intact but Oppenheim found changes in the anterior 
horns. Here the anterior horn cells as well as the fiber-work were atrophic 
together with interstitial changes, enlargement of the vessels and the ap- 
pearance of spindle cells. Lesions of the anterior cells have also been deter- 
mined by experimental researches (Stieglitz, Vulpian). In a case recently 
reported by Philippe and Gothard 3 which was anatomically investigated, 
the authors found a subacute anterior poliomyelitis as the cause of the exten- 
sive suturnine paralysis, in comparison with which the changes in the periph- 
eral nerves were only secondary. In such cases which are, however, excep- 
tional the lesions in the anterior horns cannot be regarded as secondary to 
those of the peripheral nerves, as was assumed by Warrington in his case. 
Here they evidently have a substantive importance and it appears that lead 
may seriously damage the peripheral, as well as the cerebral nervous system; 
this is further confirmed by our knowledge of encephalopathia saturnina. This 

i Neurol. Centralbl., 1003, p. 02. 

2 Revue neurolog., 1002, p. 507. ( " Ver&nderangen im Ganglion Gasseri.") 
s Lev. neurolog., 1003, p. 117. 



816 NEURITIS AND POLYNEURITIS 

view that various poisons do not constantly implicate a definite nervous area, 
may be generally accepted and particularly for the conditions observed in 
alcoholic polyneuritis, in which there have been spinaland cerebral changes. 
These may, therefore, be coordinate to the peripheral lesions. That in one 
or the other case they may be regarded partially as the consequence of periph- 
eral changes (as a retrogressive degeneration, etc.) must be admitted; but 
this view is not generally accepted. Even the alteration observed in the 
muscles may not at once be regarded as dependent upon neuritic changes, for 
clinically (myopathia saturnina, neuromyositis) as well as anatomically they 
show a certain substantive position. 

These views are applicable not only to toxic but also to other forms of 
infectious neuritis. Of the latter the diphtheritic variety has been most 
closely studied, and here the changes in the peripheral nerves are the most 
constant. These conform to the clinical symptoms and are earliest and most 
distinctly present in the nerves immediately adjoining the infectious focus: 
therefore in the nerves of the palate. The local effect of the poison may be 
noted in KussmauFs case previously mentioned, in which after diphtheria 
of the umbilical cord there was first paralysis of the abdominal muscles. 
Trousseau also found that in cutaneous diphtheria, paralysis first appeared 
in the extremities. Naturally there are exceptions to this rule. Thus in 
Griesinger's case paresis of accommodation appeared after intestinal diph- 
theria, and in a patient of Oppenheim's, following cutaneous diphtheria, the 
same sequel was noted. Besides the neuritic symptoms occurring in continuo 
others appear in many spinal, and especially also in the cerebral, nerves which 
may naturally be referred to the general effect of a circulating poison. 

The lesions may be very severe. The terminal muscular and cutaneous 
tranches are sorely involved and the destruction of the parenchyma may be 
complete. Proximally the degeneration decreases in intensity. The connective 
tissue apparently more often takes part in the changes than in toxic neuritis. 
This is often microscopically demonstrated by hyperemia and swelling of 
the nerves (Stewart), and even by decided nodular formation (in the nerves 
of the abdominal wall, the phrenic nerve, more rarely in those of the ex- 
tremities). Microscopically there are swelling and proliferation of the fibers 
and especially of the nuclei of the connective tissue; besides an inflammatory 
exudate and the migration of red and white corpuscles. The signs of true 
inflammation are therefore distinct. Streptococci are found occasionally in 
the nerve sheaths (Moos, Buhl). In numerous cases lesions could also be dem- 
onstrated in the central organs, the importance of which is not easy to esti- 
mate. Katz refers to an accumulation of black granules in the ganglion 
cells (investigated with Marchi's method) but no pathologic importance is 
to be attributed to this finding. Dejerine found marked cellular alteration 
in the anterior horns as well as in the anterior roots. Other authors have 
confirmed this and further observed degeneration in the posterior roots 
and posterior columns. Myelitic foci have been present, also hemorrhages 
in the spinal cord and spinal ganglia, meningitic changes, and lesions in the 
cardiac ganglia. Severe alterations have also been observed in the muscles, 
exceptionally even as the only finding. Occasionally no lesions have been 
demonstrated. 

The foregoing explanation serves to explain the different lesions. The 



POLYNEURITIS 817 

toxin of diphtheria finally implicates and damages all parts of the nervous 
system; those mediate as well as immediate (by fever, cachexia, secondary 
infections, sepsis, etc.) but the peripheral nervous system apparently is first 
and most severely involved. The experimental investigations (Babonneix 1 is 
the latest observer in this direction) lead to different results, showing a 
variability in the point of attack, but prove that the peripheral nervous 
system is the one most seriously damaged. In other forms of infectious 
neuritis there is by no means so great a collection of anatomical material. 
Some few cases of post-typhoid polyneuritis show the severest changes in the 
nerves of the lumbar plexus. Typhoid bacilli have been found in the nerve 
sheaths. In puerperal polyneuritis (Ivorsakow and Serbski, Pribytkow, 
Mader) 2 there is primarily parenchymatous degeneration and in part also 
periaxial neuritis. Malarial polyneuritis has been anatomically investigated 
in a few instances (Luzzato 3 and others); in a case of Eichhorst's, which 
must be included, there was principally an interstitial neuritis, likewise severe 
parenchymatous degeneration, which could also be partially demonstrated in 
the central organ. In a case of polyneuritis after follicular tonsillitis Kast 
found widely distributed parenchymatous and interstitial changes. 

Two forms of polyneuritis of well defined anatomical type require special 
description, although as I previously emphasized, with us they are rare affec- 
tions. The first is beri-beri. In this disease we find severe parenchymatous 
and interstitial lesions, of which the latter, especially in chronic cases, may 
be very decided. There may be enormous proliferation, and thickening of 
the epineurium and perineurium ; the individual fiber bundles then being 
separated from one another by broad connective tissue bands. The endoneural 
tissue is not markedly increased and a nuclear proliferation of the interstitial 
tissue can be demonstrated only in different areas to a limited extent. In 
a case reported by Rumpf and Luce, there was in particular a distinct in- 
crease of the epineural fatty tissue, so that these authors refer directly to a 
chronic interstitial lipomatous neuritis. Besides, there is quite a decided dis- 
appearance of medullary fibers. In this variety of polyneuritis, as well as 
elsewhere, peculiar structures known as Renaut's bodies have been seen. 
These consist of connective tissue bands, sometimes resembling onion peels, 
layered one above another, containing numerous large nuclei. Centrally in 
most of these bodies, there is a more or less well retained nerve fiber, usually 
with a destroyed medullary sheath. These structures apparently originate 
from the epineurium. Some writers believe that they come from the vessels. 
They do not appear to characterize any special form of the disease (Oppen- 
heim and Siemerling, Pick, Nawratzki, Kiistermann, Okada, Westphal). 
While in beri-beri the spinal cord usually shows only insignificant lesions, 
which are found in other conditions, there are almost always severe changes 
in the muscles, which may also be demonstrated clinically. In cases of acute 
onset there is a parenchymatous, fatty, occasionally colloid, degeneration of 
the muscle fibers; atrophic and hypertrophic fibers are found, the muscle 
nuclei are increased; in the chronic forms besides the atrophy of the fibers 
there is a profuse interfibrillary development of connective tissue. 

i Babonneix, Arch, general, d. med., 1903, p. 1201. 

2 See Lugaro, " Handb. d. pathol. Anat. d. Nervensystems," II, p. 1140. 

3 Luzzato, Berlin, klin. YS^ochenschr., 1902. 

53 



818 NEURITIS AND POLYNEURITIS 

The lesions in leprous polyneuritis have much in common with those of 
beri-beri, but others again deviate greatly. Here alterations of an inflamma- 
tory nature occur alongside of those which must be regarded as proliferation, 
and it is not easy to differentiate between these varieties of pathologic change. 
As may readily be seen (compare Figures 6-8, plates III and IV) the inter- 
stitial tissue is here also markedly implicated; in part there is a formation 
of small nodules which may be macroscopically recognized and palpated and 
which are of well known diagnostic importance. The interstitial changes in- 
volve especially the epineurium and perineurium, the endoneurium being less 
affected. Besides the proliferation of the connective tissue there is small cell 
infiltration which I believe does not always indicate the preliminary stage of 
connective tissue hyperplasia. On the contrary it may completely characterize 
a small cell granulation tumor. The small cell infiltrations may reach an ex- 
traordinary degree, as is seen by the illustration. The cell infiltrate is situated 
partly external to the nerve fiber bundle, and in the connective tissue structures 
between the perineurium and epineurium; such a condition is seen in Fig. 6, 
marked a. Further infiltrates force themselves between the dense or fine 
strands of the nerve fiber bundle, following the fibers of the endoneurium. In 
Fig. 7, showing greater magnification, which like Fig. 6, represents a prepara- 
tion stained according to van Gieson, these finer relations are shown distinctly. 
Besides these changes and the frequently observed thickening of the vessels, 
there are also alterations of the nerve fibers themselves which lead to their 
destruction in the manner frequently mentioned. How great this may be is 
seen in Fig. 8, • a preparation stained according to van Gieson, showing the 
scant nerve fibers which are still retained (Nf). It is here to be noted that 
the interstitial connective tissue increase of the epineurium and perineurium 
is somewhat less marked, although the previously described distinct small 
cell infiltrations are clearly recognizable at different points (a). In Fig. 7, 
the condition of the nerve fibers can be somewhat more closely studied, in 
contrast with the small cell infiltration and connective tissue increase. The 
specimen was from a nerve with moderately severe lesion in which some of 
the nerve fibers (Nf) are yet intact. In leprous polyneuritis Renaut's bodies 
are also found. 

A phenomenon, having general importance in the pathology of neuritis, 
observed by earlier investigators, may be distinctly demonstrated in these 
preparations. This is the discontinuity of the lesions. If I had examined 
above and below the points of greatest change, from which my illustrations 
were taken, but very slight alteration would be found and only at distant 
points of the same nerves are renewed severe changes of the same character 
demonstrated. For example, between two diseased foci of the right ulnar 
nerve at the elbow and wrist a large portion of the nerve showed but very few 
changes and peripherally from the wrist- joint better retained portions could 
be found. Thus Fig. 7 was taken from a peripheral branch of the right ulnar 
nerve in the hand, while Fig. 6 shows a portion of the same nerve from im- 
mediately above the wrist-joint. Nonne 1 demonstrated similar conditions. 
It is almost always possible to distinguish the bacilli of leprosy in the per- 
ipheral nerve trunks, although their number is usually small. In my case 

i Nonne, " Ueber Lepra tuberosa." Neurol. Centralbl., 1892, p. 454. 



POLYNEURITIS 819 

I was able to find bacilli in those portions shown in illustrations 6 and 7. 
The)' have been demonstrated even in the ganglion cells of the spinal ganglia 
and in the anterior horns of the spinal cord. 

The changes of the central organs are usually not very marked. They 
have been found in the posterior roots and posterior columns; but all spinal 
alterations may be absent, as in my case. 

Of the forms of polyneuritis developing from dyscrasias those of a tuber- 
cular origin have been most frequently investigated. Here interstitial and 
parenchymatous changes have been noted (Oppenheim and Siemerling). 
Pryce and Lapinsky found in diabetic paralysis degeneration of the nerve 
fibers. The same is true of paralysis due to carcinomatosis, inanition, etc. 
In cachectic individuals degenerative and atrophic changes have been found 
in the nerve fibers, often without previous clinical symptoms. These condi- 
tions were regarded as latent polyneuritis. The knowledge of this fact is im- 
portant; it cautions us not to develop relations between clinical and anatom- 
ical findings too rapidly; it forces us to appreciate a phenomenon with which 
we constantly meet in clinical descriptions; namely, that in such individuals 
an insignificant damage, a brief pressure or a mild infection, is sufficient to 
produce a neuritis. The lesions in senile polyneuritis, particularly as regards 
the peripheral nerves^ do not deviate from those that have been described. 
Alongside of these we find signs of arteriosclerosis in the vessels. The ques- 
tion how vascular changes are to be regarded in polyneuritis can, however, 
not always be satisfactorily answered. While in cases of senile polyneuritis, 
and also in those originating from an endarteritis obliterans, there can be 
no doubt of the causal connection of the vascular changes, in some instances 
at least these appear to be conjoined with neuritic ones. In other cases 
the question may well be discussed whether the vascular changes have not 
arisen secondarily in consequence of neuritis (neurotrophic changes of the 
vessels). 

It was previously stated that bacilli have been discovered in the diseased 
nerves. Apart from leprous neuritis observations of this nature are very 
scant, but Glogner, Wochenius, Centanni, Eisenlohr, Moos and Bunl have 
reported such findings. In these cases the microorganisms were by no means 
identical, for the most dissimilar varieties were observed (staphylococcus 
pyogenes aureus, staphylococcus pyogenes albus and a special bacillus by 
Centanni). From this it may be concluded that not the bacilli themselves 
but their products of metabolism are responsible for the damage to the nerves. 
The negative finding in numerous other cases of polyneuritis as well as the 
fact that many other poisons introduced into or present in the body cause 
similar phenomena, is operative in the same sense. Thus the view that 
neuritic changes are due to toxemia is very prevalent. That the toxins which 
are produced may give rise to such diverse clinical pictures, these being so 
characteristic in their individual factors, as is the case for instance with 
diphtheria-toxin, is readily understood on account of the specific affinity of 
the poison, a fact that has been demonstrated by numerous observations. 
Experimental investigation, to a great extent, is calculated to favor this view. 

In a comprehensive review of the anatomical lesions in polyneuritis as the 
primary and special seat of the changes, the peripheral portions of mixed 
nerves must be recognized. We have seen that in general the further we ad- 



820 NEURITIS AND POLYNEURITIS 

vance from the peripheral region of distribution toward the central organ, 
the less marked the changes become, without, however, the decrease in the 
process being uniform. On the contrary, it appears that special central, 
spinal or bulbar, areas are implicated with a certain degree of regularity. 
These are the intramedullary anterior and posterior roots, the anterior horn 
cells and the continuation of the posterior roots, the posterior columns. We 
must not, in order to advance a particular theory, regard the entire peripheral 
sensory and motor " neuron " as uniformly diseased ; the assumption of such 
a " neuronitis " 1 by the enthusiastic adherents of the neuron theory does 
not find sufficient support in the anatomical lesions. 

The motor nerve divisions, from the cell in the anterior horn to the ter- 
minal distribution in the muscle and in fact even this structure itself, on the 
one hand is most affected — and on the other hand, the sensory nerve portions 
from the periphery to the spinal ganglion cell and thence to the spinal cord. 
From this localization it follows, necessarily, that the differentiation of 
polyneuritis from two diseases, poliomyelitis and tabes, must be exceedingly 
difficult. The former is a malady which especially involves the first named 
motor, the latter a disease which most severely damages the last named 
sensory, division. The differential factors show, in the first place, that in 
polyneuritis motor and sensory nerves are simultaneously attacked (we have 
seen that there are exceptions to this rule) and secondly the differential points 
may be deduced from the varying nature of the disease. 

The following points differentiate the affection from poliomyelitis: Poly- 
neuritis develops in immediate connection with an infectious disease, or the 
affection in its first stages bears such a character, while in poliomyelitis these 
general phenomena are usually slight, and even when they are more prominent 
they are of brief duration and do not play an essential part. That polyneuritis 
is more frequent in adults is true, but as acute poliomyelitis also occurs in 
adults this aids but little in the decision. The development of nervous symp- 
toms in polyneuritis is mostly gradual. As a rule sensory irritative symptoms 
precede and the paralysis follows in the manner previously described. In the 
spinal affection paralysis develops rapidly, showing its maximum extent 
within a few days and gradually ameliorates. Nor do relapses occur in the 
further course as is sometimes the case in polyneuritis. There is, however, 
one form of neuritis in which all symptoms, particularly the paralysis, appear 
suddenly. This is a malady designated neuritis apoplectica and described 
by Dejerine, Remak, Westphal and others. It is usually not a true polyneu- 
ritis but a plexus neuritis. I have seen two cases of this kind, in both of 
which there was partial plexus paralysis. The differentiation from polyneu- 
ritis may give rise to great difficulty as the differential factor soon to be de- 
scribed, particularly in these cases, often leaves us in the lurch. In my cases 
the sensory, irritative and paralytic symptoms, were the ones which made the 
diagnosis of neuritis possible. The nature of the paralysis, as well as the 
localization, quite apart from the difference in development just men- 
tioned, is not the same. In polyneuritis the distal portions are most 
severely damaged. Only very rarely does complete paralysis implicate exten- 
sive muscle areas. In the early stages of poliomyelitis total paralysis may in- 

i Teitelbaum refers directly to neuronites motrices inferieures. 



POLYNEURITIS 821 

volve both legs and even all four extremities. If in the further course the 
paralysis is limited to a circumscribed region the arrangement corresponds 
to the spinal type. It is often asymmetric and does not so exclusively affect the 
distal muscle groups. The involvement of cerebral nerves, which is not rare 
in severe polyneuritis, is exceptional in poliomyelitis ; perhaps most often dis- 
ease of the facial nerve takes place, due to implication of a poliomyelitis 
bulbi. On the other hand paralyses of the ocular muscles only rarely occur 
in acute poliomyelitis but they are sometimes seen in the rare malady known 
as polioencephalomyelitis. Upon minute examination we also discern differ- 
ences in the electric contractility. There exists here between the degree of 
paralysis and the degree of alteration in the electric contractility by no means 
such a parallelism as in poliomyelitis. Severe paralyses may only present 
slight DeR or even only quantitative diminution. In areas that are only 
slightly paretic, conspicuous alterations of electric contractility may be 
present. 1 The same is true of muscular atrophy; here also differences be- 
tween function and nutrition are the rule in polyneuritis while they are absent 
in poliomyelitis. 

Fibrillary contractions are mentioned as differential factors, as they are 
said only to occur in poliomyelitis. According to my experience, however, 
in the most common form of poliomyelitis, acute infantile paralysis, they 
play no part, while in the subacute forms of adults they are more common 
and they do occur in polyneuritis. 

Among the non-motor disturbances «the most important diagnostically in 
polyneuritis are the sensory irritative symptoms and those due to absence of 
function. Pain is not necessarily absent in the first stage of poliomyelitis. In 
a case reported by Striimpell and Bartelmes, there were severe sacral pains 
for three weeks. In my experience this is not even rare in children, at least 
this symptom has been reported frequently enough. 2 But upon the whole 
the pain is of a different character in polyneuritis; it radiates to the distal 
regions often following the course of the nerves, while in poliomyelitis the 
pain is mostly localized around the focus of the disease. Marked pain upon 
pressure of the nerves and muscles, is the case in polyneuritis, in which there 
may also be only paresthesia and finally objective sensory disturbances. 
[The principal differences in regard to the sensory symptoms as between 
poliomyelitis and polyneuritis seem to be that in poliomyelitis there is diffuse 
tenderness over the limbs and along the spine. The pains are rarely neuralgic 
but commonly steady and of medium intensity. The skin is sometimes 
superficially sensitive. There is no particular sensitiveness of palpable nerve 
trunks. The pain and tenderness come on immediately with the onset of 
other symptoms, and usually subside within a week. In polyneuritis the sen- 
sory disturbances are almost invariably initiated by paresthesias, such as creep- 
ing, crawling numbness, tingling, burning, etc., and almost invariably in the 
terminal distribution of the nerves and in symmetrical areas. These hyperes- 
thesias are not transient but are characterized by their constancy, and such 
pains as occur are distinctly neuralgic. Tenderness of muscle masses and 

i See Raymond, " Polynevrite et Poliomyglite. Nouv. Iconogr. de la SalpetriSre," 
1899, p. 1. 

2 See Oppenheim, " Lehrbuch," p. 215. 



822 NEURITIS AND POLYNEURITIS 

of nerve trunks is pronounced, and when these sensory disturbances are once 
established they tend to persist for weeks, undergoing modifications that 
parallel the condition of degeneration or regeneration in the nerves that are 
affected. — Ed.] Some symptoms are entirely missing in poliomyelitis. 
Among these is the ataxia which we have learned to recognize as a very 
prominent symptom in polyneuritis. Nor does optic atrophy occur in polio- 
myelitis nor is there a symptom-picture which corresponds to the " polyneu- 
ritic psychosis." The trophic signs, in general, are somewhat different in 
polyneuritis than in poliomyelitis. Glossy skin particularly is not seen in 
poliomyelitis, in which there is extensive atrophy, cyanosis and coldness, rarely 
true hyperplasia of the fatty tissue, or much edema. The differentiation of 
these two diseases, to which so much labor has been devoted, is very important 
as the prognosis in each is very different. Finally if doubt still exists, we are 
able to form an opinion from the termination of the affection. Poliomyelitis 
never recovers entirely. Improvement, which regularly occurs at the onset, 
finally halts; a stationary pronounced limited paralysis remains, a condition 
which is exceedingly rare in polyneuritis. In polyneuritis improvement takes 
place later, it is slower, but finally leads to almost complete recovery; and 
even where paresis remains there is not such complete absence of function 
of the affected muscles and such high-grade atrophy as in poliomyelitis. 
While we may assume that after 9-12 months there is a stationary condition 
in poliomyelitis, improvement may even occur in polyneuritis after this 
period. 

For the classical form of polyneuritis these points of support will mostly 
suffice but some atypical cases may give rise to considerable difficulty. 
Neuritis apoplectica has already been mentioned. Further there are cases 
with a deviating type of localization as well as with marked decrease of sen- 
sory symptoms. Thus in the following case the diagnosis was in doubt for 
some time. 

Case 29. — A laborer's wife, aged 56, does not use alcohol and has never been seri- 
ously ill. Early in October she was attacked with very severe pain which had its main 
seat in the region of the left shoulder, radiating to the back and arms. With this 
there was formication and numbness in the left arm and hand. Fever was the only 
constitutional symptom mentioned by the patient and even in regard to this her reports 
were meagre. 

Examination middle of November, 1904, reveals passive limitation in movement of 
the left shoulder joint, marked sensitiveness on pressure of the brachial plexus, of the 
ulnar, median, and radial nerves. The pressure produces radiating pain. The triceps 
reflex is absent but the supinator reflex is marked. Of the muscles of the left upper 
extremity Erb's muscles are normal in the main, only the deltoid is implicated in its 
activity by disease of the shoulder joint. All other muscles are more or less paretic, 
the debility increasing distally, being therefore most marked in the small muscles of 
the hand. 

Electric contractility in the paretic and atrophic muscles shows at times severe 
at other times mild disturbance. A positive, objective sensory lesion was not present; 
the continuous paresthesias were mainly referred to the ulnar region. There were no 
oculo-pupillary symptoms, none on the part of the legs, bladder, or cerebral nerves. 
Continued observation of the case showed constant improvement. 

At first the pain as well as the sensitiveness to pressure disappeared. The move- 
ments in the shoulder joint, passively, soon became free. The paralysis of the extensors 
and flexors of the hands recovered completely but in the long flexors of the fingers 
there was still (March, 1905) a slight degree of weakness; severe disturbance and 



POLYNEURITIS 823 

marked atrophy are still present in the small muscles of the hand. Here there is also 
great change in electric contractility. Decided coolness or cyanosis is not present; the 
tendon reflexes have reappeared. 

In some respects this case differs from ordinary polyneuritis. From its 
localization, a spinal process involving the 7th and 8th cervical and first 
dorsal segments might be considered. The complete absence of objective, 
sensory symptoms, at least when the patient was first examined, is remark- 
able. The points, however, which favored a polyneuritis were the early, 
marked, sensory, irritative symptoms, their prolongation, the pain on pressure 
over the nerves and muscles and finally the course of the disease which tended 
toward complete recovery. We, therefore, regard the case as an amyotrophic 
plexus neuritis, the elucidation of which we owe to Remak. 

Similar cases have lately been reported by different authors. A position 
midway between polyneuritis and poliomyelitis has been assigned to them, the 
affection being regarded as a neuron disease (Barnes, 1 Williamson, 2 Brissaud 
et Bauer). 3 That polyneuritis may, however, occur without sensory symp- 
toms and without minutely corresponding to the localization and distribution 
of the peripheral nerves, is shown by lead paralysis, so that for the first men- 
tioned variety there is no decisive reason for separating it from polyneuritis; 
at all events before a final classification is attempted sufficient anatomical evi- 
dence must be produced. 

Tabes is the second disease which must be considered in a differentio- 
diagnostic respect. The decisive factor here is the development of the malady, 
for recent investigations have shown that no single symptom is pathognomonic. 
Tabes is a chronic, polyneuritis an acute, disease. Naturally there are excep- 
tions from both points of view: tabes with acute development of the main 
symptoms; polyneuritis with slow progression. The etiology deserves con- 
sideration. Polyneuritis is favored by the demonstration of a toxic or in- 
fectious foundation ; but naturally a person saturated with lead or alcohol may 
acquire tabes. 

In cases of multiple neuritis in which ataxia and sensory disturbances are 
very prominent and in contrast the paralytic phenomena are less marked, 
the diagnosis is very difficult. These are the cases of ncnrotabes peripherica 
(alcoholica, diabetica, diphth erica, etc.). They have already been referred 
to and their differentiation from tabes has been described. Marked sensitive- 
ness to pressure of the nerves and muscles favors polyneuritis or at least a 
polyneuritic component. Bladder symptoms, in polyneuritis, are slight, less 
intense and less continuous. 

Reflex pupillary rigidity in the form of arrested reaction to light with 
retained convergence may be regarded the most important sign, especially 
if it be permanent, in which case tabes is extremely likely. Transitory altera- 
tion of the pupillary reflexes, especially a combination of disturbance of the 
light reflex with that of accommodation and convergence, are not of equal im- 
portance. Crises do not appear to occur in polyneuritis, nor do arthropathies 

1 Barnes, " Toxic Degeneration of the Lower Neurons simulating Peripheral Neuri- 
tis." Brain, 1902, page 479. 

2 Williamson, Brain, 1903, page 206. 

3 Brissaud et Bauer, "' Poliomyelite anter. subaigue ou polynevrite motrice." Rev, 
neurol., 1904, p. 1226. 



824 NEURITIS AND POLYNEURITIS 

and other trophic changes. Perforating ulcer of the foot has been observed 
in alcoholic polyneuritis. Korsakow's psychosis does not belong to tabes but 
in tabo-paresis similar psychopathic conditions may be seen. Steady progres- 
sion naturally favors a spinal disease. 

There is a form of tabes designated acute spinal ataxia which develops 
from disseminated myeloencephalitis. The acute development of ataxia, as 
well as the intimate association with an infectious disease, may closely simu- 
late polyneuritic ataxia; but all other neuritic symptoms, pain upon pressure 
of the nerve trunks, sensory symptoms, and paralyses of peripheral character, 
are absent. For the most part cerebral and cerebellar symptoms are present, 
so that the differential diagnosis does not actually cause serious perplexity. 

In the symptomatology I referred to a malady called by Senator, neu- 
romyositis. Its main features are the early and marked prominence of myo- 
pathic symptoms (great pain on pressure of the muscles, edema of the muscles 
and perhaps of the skin, myopathic paralysis). The differentiation of neu- 
romyositis from neuritis, as is obvious, is not always possible, nor has this 
any great scientific or practical value. 

Landry's Paralysis. — It still remains to discuss the relation of polyneuritis 
to Landry's paralysis and to describe the differential factors. Here we meet 
with difficulties as the uniformity and substantive character of Landry's 
paralysis have, by no means, been solved. Landry gives the following de- 
scription of the symptom-complex : In persons previously healthy there devel- 
ops, after mild prodromes, subjective phenomena such as general malaise, 
paresthesia in the legs, etc., a flaccid paralysis of the legs, usually first in one, 
but rapidly, in the course of a few hours or a day, also in the other leg. 
After complete paralysis of the legs, the muscles of the trunk are involved 
and in a few days also those of the arms. Following this, the muscles of 
deglutition, articulation, and respiration are implicated, and with symptoms 
of suffocation, in a few days or in from one to two weeks, the lethal termina- 
tion occurs. In only a very few cases does improvement take place in the 
manner that the muscles last paralyzed first regain their function. Mild 
sensory symptoms occur but muscular atrophy and alterations of electric con- 
tractility are absent. Autopsy findings have so far been negative. The 
sequence of the paralytic involvement is reversed under rare circumstances, 
so that bulbar symptoms are the first, followed by an implication of the arms 
and legs. 1 

Recent experience has familiarized us with a number of cases which, in 
the main, correspond to the clinical picture just sketched and thus permit 
of a somewhat accurate clinical diagnosis. On the other hand cases have been 
reported which deviate in one point or another and these changes concern 
symptoms which were supposed at first to be absolutely necessary to a 
diagnosis. 

In fact, finally, but one characteristic remained: the rapid ascending, or 
more rarely descending, paralysis involving the entire muscular system, ap- 
pearing to be the consequence of an infectious or toxic cause. That uncer- 
tainty was bound to appear in a diagnosis which seemed to be founded upon 
the acuteness or the intensity of the pathologic process is obvious, so that 

i Oppenheim, " Lehrbuch," p. 553. 



POLYNEURITIS 825 

subdivisions and differentiations were often attempted without, however, clear- 
ing the situation. 

The course of the later reported cases revealed that they were usually 
afebrile. The paralysis began in the toes and proceeded upward rapidly, so 
that in the course of a few days there was total muscular paralysis. The 
bulbar muscles were attacked usually even before complete paralysis of the 
extremities. Difficulty in deglutition, as well as in coughing, in speech, and 
in respiration appeared. Attacks of suffocation, due to paresis of the dia- 
phragm, were the cause of death. The entire disease may last 2 or 3 days but 
often this was somewhat longer up to 2 or 3 weeks; with a bulbar onset the 
lethal termination has been reported after 29 hours. Electric examination 
in some of the classic cases reveals normal conditions. If the course of the 
disease is very rapid the explanation may be that even with the seat of the 
lesion in the peripheral nervous system there has been no time for the occur- 
rence of electric changes (as well as of atrophy). But there are cases in 
which this explanation is not sufficient, in which, in spite of a prolonged dura- 
tion of the malady, the electric reactions remain normal. On the other hand 
there are quite typical cases in which quantitative and qualitative electric 
changes are observed. In how far these differences are connected with the 
localization of the process will soon occupy our attention. 

Pain is absent or at least not very pronounced, and then appears only 
upon pressure or passive movements; but there are also cases in which pain 
is not so insignificant. Among the objective, sensory signs slight hypesthesia 
is not rare; slowing of sensory conduction is also noted. Severe bladder 
symptoms are absent, although there are exceptions to this rule. While bulbar 
symptoms are frequent, paralysis of the ocular muscles is very exceptional. 
The mind remains clear and only under the influence of high, septic fever 
may unconsciousness occur. 

These cases give us the impression that we are concerned with a severe 
infection or intoxication. Enlargement of the spleen and tumid glands often 
point to such an explanation. Accurate investigation has shown that the dis- 
ease may follow in the course of all possible infections and intoxications: 
diphtheria, enteric fever, variola, influenza, tuberculosis, sepsis, anthrax and 
many others. One point is certain namely, that there is no one definite virus 
which produces the disease; this is also in consonance with the fact that in 
a number of cases bacilli have been found in the blood, in the peripheral ner- 
vous system, and that these microorganisms were by no means of a uniform 
character. 

The patliologico-anatomical lesions are just as varied as the etiologic 
factors. There are cases in which nothing essential has been found although 
the investigations have been conducted with the most improved methods. 
Buzzard 1 only recently found insignificant changes. There are cases in 
which spinal and bulbar lesions, in the form of disseminated foci, have been 
demonstrated, and finally there is another group in which lesions were found 
exclusively, or at least principally, in the peripheral nervous system, closely 
resembling those of severe polyneuritis. 

i Buzzard, " On the Pathology and Bacteriology of Landry's Paralysis." Brain, 
1904, page 94. 
54 



826 NEURITIS AND POLYNEURITIS 

Thus it is seen how difficult it is to gain a firm foot-hold upon such an 
unsteady foundation. The cases in which there are severe pain, marked ob- 
jective, sensory disturbance, decided changes of electric contractility, closely 
resemble the severest types of polyneuritis, and I do not believe it possible 
to differentiate these diseases from the t rapidity of their course. The acute- 
ness of a process is not a sufficient differential factor in pathology. Nor does 
the impression of a severe intoxication, which certainly exists, serve to char- 
acterize the disease more definitely. On the other hand, it must be noted that 
by no means in all cases of Landry's paralysis is it correct to include them 
under the group of polyneuritis ; on the contrary there are cases which might 
more properly be included as acute disseminated my elo encephalitis. Among 
and between these there are again cases in which in one respect or the other 
the clinical picture is not sufficiently defined, and finally again such in which 
the intoxication appears to overwhelm and the nervous symptoms appear un- 
important as may well be the case in any other intoxication, such as scarla- 
tina, etc. It is, therefore, as yet impossible to systematize this group of cases. 
Common to all are the severity of the infection or intoxication and the rapidity 
of the resulting course, but the special etiology is as varying as the special 
pathology, and even the symptomatology, although apparently well defined 
and producing the appearance of clinical unity, is by no means always con- 
clusive. 

Treatment. — The management of polyneuritis, in the main, is identical 
with that of neuritis and I may, therefore, refer to that division of the 
subject. The prophylaxis consists in the avoidance and prevention of the 
many infections and intoxications on account of their intimate connection 
with polyneuritis. In this respect the advice of the physician is but little 
heeded. The prevention of occupation poisoning, especially of lead, depends 
upon general and individual measures and scrupulous cleanliness is the first 
consideration. 

As alcoholic polyneuritis is mainly due to chronic and immoderate abuse 
of spirits, while beer is much less harmful, the avoidance of alcoholic beverages 
will decidedly limit this form of polyneuritis. To prevent the arsenical form, 
among other precautions the physician must observe care in the medical em- 
ployment of arsenic. 

We are hardly in a position to maintain a proper prophylaxis of the 
infectious varieties of polyneuritis. We should see to it that the patient does 
not over-exert himself and that he have a sufficiently long period of rest after 
an infectious disease, and prevent inanition and cachexia. In cachectic, 
dyscrasic polyneuritis the prophylaxis is the same as the treatment. In the rare 
form of syphilitic polyneuritis mercury, as previously stated, is sometimes 
contra-indicated ; in malarial neuritis quinin is of great benefit. 

After the disease has become manifest absolute rest in bed is imperative 
especially for the severe cases, so soon as the neuritic symptoms advance be- 
yond the lower extremities. A bed-pan must be used by the patient ; he should 
be fed and not permitted to alter his position without aid. This absolute rest 
may only be modified when there is an obvious improvement in the paralysis. 
To avoid deformities certain measures should be instituted at the onset : foot- 
drop is hindered by properly placed sandbags, and the pressure of the bed 
clothes can be removed by a wire hoop. 



EXPLANATION OF THE COLORED PLATES 

Fig. 1. — Sciatic nerve of a rabbit, peripheral portion; 10 days after severing the trunk. 
Marchi preparation. 

Fig. 2. — Nerve cicatrix from the brachial plexus after an incised wound. Van Gieson- 
alum-hematoxylin preparation. Zeiss ocular 2, obj. AA. 

Fig. 3. — Ulnar nerve in parenchymatous neuritis. Osinic acid preparation stained after 
van Gieson. Ocular 2, obj. AA. 

Fig. 4.— The same. Ocular 2, obj. DD. 

Fig. 5. — Sciatic nerve in acute polyneuritis. Weigert-Pal preparation stained after van 
Gieson. Ocular 2, obj. AA. 

Fig. 8. — Ulnar nerve iu polyneuritis leprosa. Van Gieson-alum-hematoxylin preparation. 
Ocular 2, obj. A A. 

Fig. 7. — Ulnar nerve in polyneuritis leprosa ; different view than Fig. 6. Same stain. 
Ocular 2, obj. DD. 

Fig. 8. — Ulnar nerve in polyneuritis leprosa; the same field as Fig. 6. Weigert-Pal 
preparation with van Gieson stain. Ocular 2, obj. A A. 



PLATE II 
Fig. 1 



,% •'&£'£& ► J! 




MfM 







■0> 



\m. 









m 



Fig. 2 





Fig. 4 




PLATE IV 
Fig. 5 











PLATE V 

Fig. 7 








" n.f. 



POLYNEURITIS 827 

The diet is very important. When at all possible it should be plentiful 
and very nutritious, which in the early stages, especially of infectious poly- 
neuritis is not always possible. Milk is advised by Edinger, 1 as well as but- 
ter, bacon, lipanin, in fact a diet rich in fat. Sometimes nourishment by 
means of the stomach tube may be necessary as in diphtheritic deglutition- 
paralysis. In the diabetic form, a diet to combat the underlying malady often 
gives marvelous results. 

Oppenheim also advises diaphoresis in polyneuritis the same as in neuritis. 
In robust patients hot baths should be used; in others hot packs, or heated 
air with hot drinks, and perhaps by the administration of diphoretics such 
as aspirin. Such diaphoresis may be employed daily for from 1-2 hours. 
Even in severe and chronic cases Oppenheim has obtained brilliant results by 
these means. Naturally watchfulness, in this method of treatment is neces- 
sary. It is supposed to remove, by excretion, the toxic products formed or in- 
troduced into the organism, in a speedy manner. We possess no specific for 
the disease but there is sufficient opportunity for the physician in the relief 
of the special symptoms. 

The heart often requires attention but alcohol had better not be used; 
when stimulants become necessary, camphor or caffein may be administered; 
also sinapisms and faradization of the cardiac region, etc. Respiratory dif- 
ficulties, which may directly endanger life, require artificial respiration, fara- 
dization of the phrenic nerve and cutaneous irritants. Eaymond and Leyden 
have particularly advised strychnin for this purpose. Difficulty in deglutition 
may necessitate artificial nutrition. 

Pain may be relieved by some of the newer analgesics, and on account 
of their great variety it will be possible to dispense with morphin. Hot com- 
presses or Priessnitz's packs are useful. 

Paralysis should not be treated during the early course of the disease. 
Only after the irritative signs have disappeared should electricity be used, 
as well as mechanical therapy. In regard to the special indications I may 
refer to what was stated under mononeuritis. In the later stages besides 
electricity and massage, exercises are important, but overexertion must always 
be prevented. Spa treatment may be advised with advantage. The sodium 
chlorid springs and peat baths (Baden-Baden, Wiesbaden, Nauheim, Wildbad, 
Eagaz, Teplitz, Marienbad, etc.) are useful. 

Convalescence, which is prolonged, is often hastened by climatic treatment ; 
a warm climate, perhaps even the sea air is strongly advised by Edinger. 
But here caution is necessary, the patient must avoid all rheumatic influences 
as the danger of relapse is not remote. 

In cases in which complete cure does not occur, orthopedic or surgical 
treatment must be used, the necessity for which has been previously detailed. 

The treatment of polyneuritis is one of the most gratifying in the entire 
realm of neurology; with a careful and attentive choice of remedies it brings 
about good results even in instances in which the affection is a very serious 
one. 

i " Behandlung der Polyneuritis." Penzoldt-Stintzing's Eandbuch, Bd. V, p. 679. 



NEURALGIA 

By H. EICHHORST, Zurich 

In the description of neuralgia, we shall utilize the clinical history of four 
patients in whom a diagnosis of neuralgia has been made. If a layman were 
to draw from this the conclusion that the subject of our present consideration 
(neuralgia) presents a uniform pathological picture (since the name cer- 
tainly implies that all were suffering from the same malady), undoubtedly we 
should object strongly to so erroneous a conclusion. In fact, each of these 
four patients presented a group of highly interesting and peculiar symptoms. 
In the course of our description we will find abundant opportunity to revert 
to this. 

In the great majority of cases the recognition of neuralgia causes the 
physician no great perplexity, and even a tyro in medicine can usually diag- 
nosticate it without difficulty. Let us now consider what manifestations of 
the disease were particularly prominent in our four cases, so that we may 
be absolutely convinced of the correctness of the diagnosis. 

SYMPTOMS 

The chief symptom — of this there is obviously no doubt — was pain, but 
pain of a very peculiar character. Although it is true that the word neural- 
gia in itself means merely nerve pain, and since we can scarcely imagine 
pain which does not involve the nerves, every painful sensation might prop- 
erly be called neuralgia; but the word has a definite, clinical meaning, and 
is applied only to pain of a certain kind. In the first place, pain which is to 
be designated neuralgia must be confined to the anatomical distribution of a 
sensory nerve tract. Besides this, however, a paroxysmal character is typical 
of neuralgic pain. It must be borne in mind that all of our four patients 
presented these symptoms which are decisive in a diagnosis of neuralgia. 

Considering the importance of the subject, a closer consideration of the 
nature of neuralgic pain is quite justifiable. In many cases the patient is 
able to give such accurate and reliable information as to the seat of his pain 
that no doubt can exist as to the exact nerve tract which is affected. For 
example, our first patient asserted positively that he suffered from severe pain 
in the left leg, which began at the back of the thigh and thence passed 
through the leg to the sole of the foot and the toes. At our request he traced 
with his finger the area of distribution of the pain, and we are quite certain 
that no one could better or more accurately outline the course of the sciatic 
nerve in the living body. We cannot for a moment doubt that in this patient 
828 



SYMPTOMS 829 

the left sciatic nerve was the seat of the neuralgia, and that this case revealed 
the diseased condition known as sciatica. 

In the second patient also it was easy to determine which nerve tract 
was the seat of neuralgia. Here the patient was a man of 62 ; he was attacked 
nearly six months ago by herpes zoster in the area of distribution of the left 
fifth and sixth intercostal nerves. Although we did not see the patient at that 
time, we cannot doubt his account, because we still observe upon his skin five, 
oval, brown spots, speckled with white, and almost as large as the palm of 
the hand, which follow the course of the fifth and sixth intercostal spaces 
from the left side of the spine to the sternum, and which form indubitable 
proof for any one of experience that zoster must have existed at those points 
only a short time previously. We learned from the patient that the develop- 
ment of herpes was preceded for several days by violent pain in those areas 
of the skin in which subsequently groups of herpetic vesicles appeared. The 
pain did not absolutely cease with the development of the vesicles but amelio- 
rated considerably, and led the patient to hope that with the disappearance 
of the eruption the pain also would be arrested. Unfortunately he was 
doomed to disappointment. In the course of the second week the herpetic 
vesicles dried up and the crusts thus produced desquamated; but this had 
hardly occurred when the pain became more violent than ever, and for six 
months persisted with undiminished intensity. In our examination we were 
convinced that the patient indicated the left fifth and sixth intercostal spaces 
as the seat of pain for, in order to point it out, he drew the thumb of his 
left hand along the fifth and then the sixth intercostal space from the verte- 
bral column to the left border of the sternum. After such unequivocal sign- 
language, we could not for a moment hesitate to assume intercostal neuralgia 
of the fifth and sixth intercostal nerves on the left side. 

The localization of the pain is not always so clearly indicated as in these 
two patients. In our third case, a woman, 43 years of age, we made a diag- 
nosis of neuralgia of the second branch of the trigeminal nerve (fifth nerve). 
This patient asserted positively that the paroxysmal pain did not, as a rule, 
spread longitudinally, but was generally limited to a deep circumscribed 
point. From time to time, however, she suffered from attacks characterized 
by radiating pain. 

Moreover, we must remember that not only the peripheral sensory nerves are 
affected by neuralgia, but also occasionally the sensory nerves of the internal 
organs, in which case the picture of visceral neuralgia is produced.. We are 
forced to admit that but little is known of this form of the disease, and that, 
for example, many reports of neuralgia of the liver, of the kidneys, and of 
the stomach admit of a different clinical interpretation. It is well known 
that the joints may be the seat of neuralgia; and some physicians even in- 
clude migraine and hemicrania among the neuralgias. In all instances of 
visceral neuralgia the patient feels paroxysmal pain in the affected organs, but, 
as a rule, is unable to locate it exactly. 

We have pointed out as characteristic of neuralgia not only its seat and 
distribution, but also its paroxysmal appearance. All our patients agreed that 
the pain was unbearable only at times. Three of them were absolutely free 
from pain in the intervals between the paroxysms ; the man with sciatica, how- 
ever, asserted (and this agreed perfectly with other experiences) that he was 



830 NEURALGIA 

never entirely free from pain. He felt constantly a dull but endurable pain 
along the diseased nerve, and at intervals there were paroxysms of short 
duration that were almost unbearable. It is customary, therefore, to dis- 
tinguish between intermittent and remittent neuralgia. Cases like the one 
just described belong, of course, to the remittent form. 

The number and the duration of the paroxysms reveal astonishing varia- 
tions. In some cases they follow each other at intervals of a few minutes, 
even a few seconds ; in others they may appear only a few times in the course 
of a day. At times there is a lapse of several days between the distinct 
attacks, or there may be many weeks. Even in the same patient there are 
variations, and it may be stated as a general rule that at the beginning and 
the end of the illness the paroxysms are usually less frequent than at its 
height. 

The duration of a paroxysm varies from a few seconds to several minutes. 
When we hear of a paroxysm lasting an hour or even several hours, closer 
questioning will usually reveal the fact that there was previously a series of 
paroxysms separated from each other by such brief intervals of freedom from 
pain that the patient considered them to be one continuous paroxysm. 

It is often necessary to discriminate between fully developed and abortive 
attacks. The latter generally appear as lightning flashes of pain which dis- 
appear as quickly as they come, and often visibly startle the patient and cause 
him to shudder. 

The diameter of the pain is very differently described by different pa- 
tients. Most often it is designated as burning, piercing, or boring. Some 
patients say that a paroxysm produces a sensation which can only be compared 
to that of the glowing iron being bored into them; others, that the affected 
nerve seems to be slowly pulled with tongs. Moreover, the character of the 
pain varies in different attacks, and it sometimes happens that a paroxysm 
may begin with a boring pain and subsequently change to a sensation of violent 
burning. 

At times the pain is confined to a certain point, but in the majority of 
cases it radiates through large areas. In many cases the patients assert posi- 
tively that the pain shoots from the center to the periphery; whereas, in my 
experience, a distribution of the pain in the opposite direction is more rare. 
This has resulted in the frequent distinction between neuralgia ascendens and 
neuralgia descendens, but I do not ascribe much importance to this differen- 
tiation since, in many patients, ascending and descending pains may alter- 
nate, or the pain may simultaneously shoot from a certain point both to the 
center and to the periphery. 

Although the peculiar pain above described is sufficient for the recogni- 
tion of neuralgia, in many cases there are still other manifestations of the 
disease which may be grouped together under the generic title of incidental 
neuralgic findings. Sometimes these are local changes in the nerves, sometimes 
reflex disturbances. 

Among these local secondary findings, the circumscribed pressure or pain 
points must be given due importance. They are also called Valleix's pressure 
points (points douloureaux) because Valleix, 1 in 1841, first described them 

i Valleix, " Traite des neuralgies," etc., Paris, 1841. 



SYMPTOMS 831 

in detail. If we trace with the finger the accessible peripheral course of a 
nerve affected by neuralgia, we will find in many cases that the entire course 
of the diseased nerve is more or less sensitive to moderate pressure. This is 
especially noticeable when we outline in a similar manner the corresponding 
nerves of the well side of the body. In many cases, moreover, we will find 
the nerves particularly sensitive to pressure at different points which, in fact, 
represent the painful points of Valleix. By pressing with a single finger-tip 
we can readily ascertain that the extent of a single area is very limited. 

Brenner x called attention to the fact that it is occasionally possible by 
means of the galvanic current to discover painful points in a nerve affected 
by neuralgia when pressure with the finger does not reveal them. For this 
purpose we use a galvanic current sufficiently strong to produce sensation in 
the skin. The anode (positive or copper pole) is placed upon any part of the 
body as the indifferent electrode, and the cathode (negative or zinc pole) is 
slowly passed along the course of the nerve. Painful points, formerly obscure, 
are indicated by the fact that the patient feels pain at circumscribed points 
when the cathode passes over them. 

The number of painful points and their clinical significance has undoubt- 
edly been overestimated by Valleix ; nevertheless they are of the greatest diag- 
nostic and therapeutic importance. 

Each nerve has its specially painful points. Our patient with trigeminal 
neuralgia exhibited an easily recognizable, painful point directly below the 
infraorbital margin, corresponding to the position of the infraorbital foramen, 
from which the second branch of the trigeminal nerve finds its exit. In the 
patient with intercostal neuralgia, three painful points were revealed: One 
near the spinous processes of the vertebral column, a second about in the 
midaxillary line, and a third just behind the left margin of the sternum. In 
the two patients with sciatica the locations varied. The first showed only two 
pressure points of which the first was situated just below the lower margin 
of the gluteus maximus, the second, in the popliteal space. In the other case 
we discerned six pressure points as follows : One near the anterior superior 
spine of the ilium ; one on the lower margin of the gluteus maximus ; one in 
the center of the posterior surface of the thigh; one in the popliteal space; 
one in the middle calf of the leg; and one under the head of the fibula. We 
note, therefore, from our clinical observations, that the painful points in any 
nerve may vary in number, and that in neuralgia confined to one nerve all 
of the areas known to be painful points are not necessarily involved. Con- 
tinued observation of patients suffering from neuralgia would probably soon 
convince us that there are certain susceptible points in every nerve. 

The scat of pain in a nerve is usually to be sought just below the surface, 
or where the nerve emerges from bony canals, muscles, or fasciae, where it is 
superficial over bones, and where it gives off lateral branches. To refer once 
more to our patients, we see that the pressure point in infraorbital neuralgia 
is an instance of an affected nerve emerging from a bony canal and being 
superficial, situated upon an osseous surface. The three pressure points men- 
tioned in the case of intercostal neuralgia are of varying origin. The inter- 

i Brenner, " Ueber die Auffindung von Schmerzpunkten langs der Wirbelsaule und 
der Nervenstamme." Berliner klin. WoeJienschrift, 1880, Nr. 21. 



832 NEURALGIA 

costal nerve issues from the spinal canal just as it leaves the intervertebral 
space close to the vertebral column. The perforans lateralis branches from 
it on the side toward the thorax, and the perforans anterior emerges from 
under the skin close to the margin of the sternum. From these two examples 
it is easy to understand the anatomical distribution of painful points in other 
neuralgias, as in our two cases of sciatica. 

There are no absolutely reliable data concerning the origin of painful 
points. The hypothesis that they can be explained in a purely mechanical 
way, in that the diseased nerve from its superficial position is susceptible to 
pressure at these points, must be discarded as incorrect; since, in the same 
anatomical positions, only circumscribed nerve areas give any indication of 
being painful points, while adjacent areas, with the same anatomical situa- 
tion, do not. Whether, however, these painful points bear any relation to 
the still very little known nervi nervorum, or to the so-called nervi recur- 
rentes, or to peculiar vascular distributions, is a question which has not yet 
been answered. 

Trousseau deserves credit for having added materially to our knowledge 
of painful points by drawing attention to the occurrence of distant painful 
points in neuralgia, or, as he called them, points douloureaux apophysaires. 
In many cases of neuralgia it appears that not only portions of the diseased 
nerves but also certain spinous processes of the vertebral column are ex- 
traordinarily sensitive; these correspond to the points apophysaires of Trous- 
seau. In the case of neuralgia of the nerves of the trunk or of the extremities, 
we frequently recognize that only the spinous processes of those vertebra? are 
sensitive to pressure between which the branches of the roots of the affected 
nerves leave the vertebral canal. But such points apophysaires may also be 
found with relative frequency in neuralgia of the cranial nerves; that is, in 
trigeminal neuralgia in the region of the middle cervical vertebrae. 

In exceptional cases, painful points may also be found in distant areas 
of the extremities; for instance, at the head of the radius or the ulna, and 
it is this circumstance which leads me to prefer the title, distant pressure 
points, to that chosen by Trousseau. 

A very limited experience makes it apparent that the painful points in 
neuralgia may vary greatly. For instance, in some patients painful points 
can be recognized only at the time of the paroxysm, while in others they exist 
also at other times and merely become more painful during the paroxysm. 
In another respect also a noteworthy difference appears; in one case pressure 
on the painful points may produce an attach of pain ; in another it causes the 
disappearance of the paroxysm then present. At times the effect may be 
lessened or intensified according to whether the pressure upon the painful 
point is light or heavy. The former is likely to produce a paroxysm, while 
the latter may cause its disappearance. 

Speedy and astonishingly favorable results have been obtained from the 
treatment of painful points by means of the galvanic current. The use of 
the positive electrode (anode) has rapidly cured cases of neuralgia of long 
standing. This is true of the far distant as well as of the local painful points ; 
stubborn trigeminal neuralgia (fifth nerve), for example, has often been 
speedily relieved by treating the painful vertebral points with the anode after 
all other remedial measures were unavailing. 



DISTURBANCES OF TACTILE SENSE 833 

Nervous disturbances, produced reflexly, affect sometimes sensory, some- 
times trophic, secretory, or vasomotor nerve tracts. 

DISTURBANCES OF TACTILE SENSE 

In the majority of cases we find abnormality of sensation. Frequently 
this is at first indicated by hyperalgesia of the skin, only at the time of the 
paroxysm — mere contact with the point of a needle causing excessive pain — 
while at the same time there may be hyperesthesia — a blunt object, a camel's 
hair brush, for instance, may be passed over the skin without producing any 
sensation. In neuralgia of long standing, hyperalgesia and hyperesthesia are 
often most persistent. After a while hyperalgesia may disappear and be fol- 
lowed by a steadily increasing loss of sensation. In neuralgia of long stand- 
ing, hyperalgesia and hyperesthesia are often, though by no means invariably, 
found in the distribution of the affected nerve. On the other hand, complete 
anesthesia or analgesia is very rare. 

In many cases these sensory disturbances are of purely peripheral origin, 
especially when a peripheral nerve trunk shows marked change. If, for in- 
stance, a nerve trunk is being pressed upon by a tumor, a complete interruption 
of the conduction of the nerves may readily occur, and the area of skin ap- 
pertaining to it will lose all sensibility. On the other hand, irritation of 
the central pressure point may cause violent paroxysms, which the patient, 
in accordance with the law of eccentric conduction, will refer to the anesthetic 
region of the skin. This condition, called anesthesia dolorosa, is often asso- 
ciated with cancer of the spine, if the cancer presses upon and irritates the 
sensory nerves issuing from the intervertebral spaces. 

It would scarcely be in conformity with the actual conditions if we sought 
to trace all sensory disturbances which appear in the course of neuralgia to 
an impairment of conduction within the peripheral nerve tract. Undoubt- 
edly, in many cases an obstruction or a dissolution of continuity in the con- 
duction of sensory impulses in the nerve centers is caused reflexly through 
a neuralgic irritative condition, especially in the nerve centers of the spinal 
cord. This condition is indicated whenever the sensory disturbances extend 
beyond the area of distribution of the affected nerve. As long as they are 
limited to the diseased nerve, and unless there are changes in the peripheral 
nerve trunk which point directly to a peripheral stoppage of conduction, it 
is sometimes impossible to decide whether the cause is peripheral or central. 

The same conditions prevail in other disturbances yet to be mentioned. 
These may be either of peripheral or central-reflex origin, and must be con- 
sidered individually from the standpoint just discussed. 

Vasomotor disturbances appear frequently in trigeminal neuralgia whereas 
they are rare in other forms; for instance, in sciatica. Our patient with 
trigeminal neuralgia displayed these so markedly during attacks of pain that 
one could deduce the onset of a paroxysm merely from the facial appearance. 
In one case in which I had the opportunity of witnessing the beginning of 
a paroxysm, my attention was attracted by the sudden pallor of the affected 
side of the face, this being succeeded in a short time by a still more conspicu- 
ous flushing. Obviously the pain had first produced a contraction of the vaso- 
motor nerves which suddenly changed to a paralytic condition of the vessels 



834 NEURALGIA 

supplying the muscles. In neuralgia which has existed for a long time this 
spasmodic action of the vessels does not appear, and from the beginning there 
is intense flushing caused by their paralyzed condition. In the case cited I 
believe there can be no doubt that a reflex change had taken place in the 
activity of the vasomotors, since evidently only the second branch of the fifth 
nerve was affected by neuralgia, whereas the vascular change was apparent 
over the entire side of the face. Moreover, turgidity and increased pulsation 
of the temporal artery on the affected side of the head were observed during 
the period of flushing. As was to be expected, the flushed side of the face 
was abnormally warm to the touch. 

Secretory and Trophic Disturbances. — But our patient demonstrated to 
us that besides vasomotor disturbances there may also be secretory and trophic 
changes in neuralgia. We noticed that as the flush deepened upon the af- 
fected side of her face the skin became glossy, and numerous tiny drops of 
perspiration soon appeared. At the same time the conjunctival sac filled 
with tears which overflowed upon the cheek of the involved side. Trigeminal 
neuralgia is characterized by the relative frequency with which secretory dis- 
turbances appear, and these are not always limited to increased sweating and 
the secretion of tears. Some patients show profuse nasal secretion* or saliva- 
tion on the affected side, and the nasal secretion occasionally contains blood. 

Occasionally there are reflex, secretory disturbances in remote organs. 
For example, some patients pass large quantities of pale urine during a 
paroxysm; and these are not hysterical patients, with whom such occurrences 
are common even though they do not suffer from neuralgia.. 

To return to our case of trigeminal neuralgia: I wish to call atten- 
tion to the presence of trophic disturbances as well as those which are vaso- 
motor and secretory. A trained eye will readily perceive that the skin on 
the affected side of the face is thicker than on the other; and if we pinch 
up a fold of the skin between two fingers we are thoroughly convinced that 
the skin is thicker and coarser over the entire affected side. Moreover, experi- 
ence has demonstrated that at the time of the paroxysm there is swelling of 
the side implicated, especially when the paroxysm is of unusual violence and 
duration. Naturally I look upon this not as a trophic, but as a vasomotor 
disturbance, in fact as a transitory cutaneous edema. 

In a few cases of trigeminal neuralgia the exact opposite of this condition 
has been observed; namely, a unilateral facial atrophy. It has also been 
reported that the hair on the affected side of the head becomes gray, rough 
or coarse, or falls out. Occasionally we observe the so-called ringed hair 
(leukotrichia), in which condition pigmented and non-pigmented areas alter- 
nate in a single hair and cause these points to appear as white spots. 

In neuralgia affecting various nerves it is not unusual for trophic lesions 
to appear upon the skin. As such we may cite thickening of the epidermis, an 
excessively active desquamation, conspicuously profuse growth of the hair, and 
erythematous and vesicular eruptions which either form large, single vesicles 
known as pemphigus or coalescent groups of pin-head vesicles called herpes. 

Trophic changes in the bones and joints occur less frequently. They usually 
represent thickening, and must not be confounded with inflammatory changes 
which are often not the result of neuralgia, but its cause. Painful lesions 
would, in any case, contraindicate a purely trophic origin. 



DIAGNOSIS 835 

Atrophy of the muscles in an area affected by neuralgia is not rare, neither 
is it by any means invariable. It would, however, be incorrect always to in- 
terpret such muscular lesions as purely trophic. Often they are a natural 
result of the fact that, on account of the pain, the muscles were used too 
little and therefore gradually decreased in size. Such inactivity atrophy 
would seem to be indicated when the atrophy of a muscle progresses slowly 
but continuously for many weeks, yet in spite of this does not become extreme. 
A pure, trophic, muscular atrophy develops rapidly, is often distinctly notice- 
able in the second week, frequently becomes extensive, and disappears but 
slowly after the neuralgia is cured. Our two patients with sciatica presented 
instructive illustrations of both these forms of muscular atrophy ; one of them 
suffered from sciatica only four weeks, yet the muscles of the thigh and leg 
shrunk considerably. This was apparent on inspection, but measurements 
showed that the circumference of the middle of the thigh and leg of the 
affected right side was 4 cm. less than that of the healthy left side. On the 
other hand the second patient was ill for nearly three months. In his case also 
the muscles of the affected leg were shrunken, but, in contrast to the first 
patient, he himself had not noticed this, and measurement revealed a differ- 
ence of not quite one cm. It appears justifiable, therefore, to consider the 
first case purely trophic, the atrophy in the second as due to inactivity of the 
muscles. Eeflex causes may be active in the case of muscular lesions also. 
For instance, neuralgic changes in the sensory area of the spinal cord may 
produce an inhibition of the trophic cells, possibly also of the large ganglion 
cells in the anterior horns of the spinal cord. Since, however, such muscular 
atrophy is usually unaccompanied by paralysis but merely produces weakness 
in the muscles due to their diminution, it appears more logical to seek the 
cause of purely trophic muscular atrophy in disturbances of the peripheral 
nerve tract; in fact, we are warranted in supposing that it is caused by 
neuritic lesions, and is indicative of these. 

DIAGNOSIS 

All objectively demonstrable nervous disturbances are of the greatest im- 
portance in the diagnosis of neuralgia, since obviously they can never be feigned 
by patients. We are often called upon in our Clinic to treat patients who 
appear to be suffering from sciatica. The affection is traced sometimes to 
a fall, sometimes to a blow, sometimes to the lifting of a heavy weight while 
at work, and accident insurance is demanded as compensation for the osten- 
sible injury. Often the patients go so far as to bring a suit for damages, 
and demand a medical opinion. If, besides the pain, there are nervous de- 
rangements, in which case sciatica would soon produce muscular atrophy, our 
course is clear and we need not hestitate to testify to the existence of neuralgia. 
Under some circumstances, however, grave diagnostic perplexities arise. The 
alleged patients may simulate with wonderful accuracy all the subjective 
pain of which actual neuralgic patients are wont to complain. I have fre- 
quently convicted presumable sciatic patients of malingering by having them 
watched as they got out of bed, when they appeared to move in a perfectly 
normal manner, whereas, in my presence, they had limped markedly with the 
affected leg, and asserted that they could not bear their weight upon it. 



836 NEURALGIA 

DIFFERENTIAL DIAGNOSIS 

In a differential diagnosis of neuralgia, painful muscular lesions often 
come under consideration. We must bear in mind that an affected muscle 
is likely to be sensitive to pressure, that it occasionally shows swelling and 
thickening, and that the pain never extends beyond the limits of the muscle 
implicated. 

Inflammation of the hones or of the periosteum will often cause diagnostic 
error. Here we should especially seek to find sensitive swelling and local 
lesions in the tissues indicated. 

At times an inflammation of the joints is mistaken for neuralgia. We 
all know that it is sometimes impossible at the first glance to state with 
accuracy whether we are dealing with sciatica or hip-joint disease. In the 
latter there is a typical position of the leg with severe pain when the patella 
is moved suddenly against the other bones of the knee, and especially on 
rotating the thigh. It is hardly necessary to say that we must try to locate 
exactly the lesions of the joints. 

ETIOLOGY 

Up to this point we have been devoting our attention to neuralgia only as 
a fully developed disease. If we now consider the development of neuralgia, 
we will find that it seldom appears in the form which it ultimately assumes. 
Often the onset of an indubitable neuralgic attack is preceded for several 
hours or days by paresthesia; the patients complain of sensations of stiffness, 
numbness, cold, itching and the like in those areas of the skin which are sub- 
sequently affected by neuralgia. Sometimes neuralgia announces itself by 
lightning-like pain which passes away rapidly and only gradually becomes 
of longer duration, perhaps also more severe, and develops into unmistakable 
neuralgic paroxysms. 

It is sometimes impossible to determine the cause of neuralgic attacks. 
They may appear during periods of profound tranquillity, both physical and 
mental. Patients may be aroused from deep sleep by their onset without 
having previously been troubled by bad dreams. At other times the paroxysms 
may follow excitement or irritation of the most trivial kind. Lightly blow- 
ing on the skin, touching it, or dropping a scrap of paper upon it, may be 
sufficient to evoke a spasm. Many patients suffer intense pain during masti- 
cation or drinking, especially if partaking of anything very cold, very hot, 
or very hard; or if they happen to bring the teeth together with unusual 
force. In some cases the stimulus for an attack originates in the organs of 
special sense ; a glaring light or sharp, shrill tone may produce them. Mental 
excitement also is frequently a cause, as, for instance, laughter, weeping, the 
arrival of the physician, fear of an impending paroxysm, or something 
similar. 

Since the appearance of neuralgic attacks depends partly upon extraneous 
circumstances, in many cases they naturally follow no rule. In others it 
frequently happens that the paroxysms appear at regular and definite inter- 
vals, and continue for approximately the same length of time. One patient 
will be attacked by a spasm at exactly the same hour day after day, while for 



ETIOLOGY 837 

another there may be intervals of two, three, or even five days. This form 
of the disease is called intermittent neuralgia, and is usually the result of 
malarial infection. But it is by no means correct to assume this cause in all 
such cases, for neuralgia may show a distinct tendency to intermittence even 
when it has no dependence upon any form of malaria. 

The onset of a paroxysm is often revealed by the general behavior of the 
patients without vasomotor or secretory manifestations. Often the pain forces 
them to stop whatever they are engaged in doing. They moan and groan, 
and grasp the affected region with the hands. Sometimes they make the 
strongest possible pressure upon it, even pressing against some stationary ob- 
ject in order to deaden the pain. They may apply to themselves objects so 
hot that they receive large burns and scars, especially conspicuous and dis- 
figuring in the face, and these constitute indelible evidence of the disease 
even after recovery. In some cases the paroxysms are accompanied by clonic 
muscular contractions — seldom by tonic — of the facial and masticatory mus- 
cles on the affected side of the face. While the facial muscles present clonic 
spasms the masticatory muscles, especially the masseter, show tonic muscular 
contraction. Trigeminal neuralgia is known to be frequently accompanied by 
muscular contractions. Trousseau, 1 in his C Unique Medicate, applied to this 
form of neuralgia the distinctive name of epileptiform neuralgia, because 
the sudden appearance of muscular spasms reminded him of a similar con- 
dition in epilepsy. The name can hardly be considered well chosen since this 
condition bears no actual relation to epilepsy, and this probably accounts for 
its having so little vogue. Besides, Trousseau considered the disease to be 
incurable. It must be admitted that it often resists all therapeutic endeav- 
ors; nevertheless, complete recovery has been reported. 

Obviously the clonic muscular contractions which accompany neuralgia 
must be of reflex origin, since they often appear in the region of motor nerve 
tracts. At times distant motor paths are also reflexly affected, so that, for 
instance, in trigeminal neuralgia, there may be muscular contractions not only 
on the well side of the face as well as on the diseased, but also clonic spasms 
in one or both of the upper extremities; occasionally even general clonic 
muscular contractions. 

In a few cases slowing of the heart's action is observed during an attack 
of pain, which shows that the pneumogastric nerve is reflexly affected. 

The constitutional condition, as a rule, is but slightly disturbed. The 
temperature remains unaffected; a few reports of its rise after an attack 
still need confirmation. If the attacks preponderate during the night, sleep 
is obviously more or less disturbed, and besides suffering from physical and 
mental weariness the patient is pale and looks ill. Many persons afflicted with 
stubborn neuralgia find it necessary to lead a solitary life since the violence 
of the pain makes association with others impossible; moreover, excitement 
which cannot well be avoided in general contact is likely to increase the num- 
ber and heighten the intensity of the attacks. Thus the unfortunate patients 
are often regarded as unsocial and misanthropic. The pain, often unbearable, 
does not conduce to a calm and equable demeanor; indeed, signs of moodi- 
ness and irritation are prone to appear. In a few cases stupor and delirium 

i A. Trousseau, " Clinique medicale," Paris, 1862, T. II, p. 44. 



838 NEURALGIA 

have been observed during excessively violent attacks. It is known to medical 
experience that people with incurable neuralgia have finally been driven to 
suicide, but this, fortunately, is a much rarer occurrence than statements in 
certain text-books might lead us to suppose. 

In trigeminal neuralgia there is often decided loss of strength which 
progresses with astonishing rapidity because, in addition to want of sleep, 
reluctance to eat or drink for fear of precipitating a neuralgic attack tends 
in large measure to impair the nutrition. 

We must bear in mind that disturbances of metabolism are said to be fre- 
quently associated with neuralgia. In some cases of sciatica the excretion of 
sugar in the urine, glycosuria, has been observed. Here we must guard 
against error, and must remember that diabetes mellitus is often a cause of 
neuralgia, especially of sciatica. 

In many cases of neuralgia the pain is limited throughout to a single 
nerve branch, in others it gradually involves other branches, even far distant 
ones. It is not unusual, for example, for trigeminal neuralgia to begin in 
one trigeminal branch, and gradually to extend to other branches of the 
same trigeminal trunk. There are two reasons for this. It might be possible 
for a morbid process, e. g., neuritis of a nerve branch, to involve another 
nerve, but in many cases there are also so-Called radiation phenomena. We 
assume these to be produced by a condition of irritability within the central 
nervous system which gradually implicates adjacent sensory areas. Neuralgia 
appears first in a given nerve, and after a time involves the corresponding 
nerve on the other side of the body. This is often followed by a distinct 
amelioration or even entire cessation of the pain in the originally affected 
nerve. In some cases there is a constant alternation of the neuralgia between 
the two sides of the body. Occasionally we find it distributed to distant nerve 
paths. Thus, trigeminal neuralgia is often accompanied by neuralgia of the 
occipital nerves or the nerves of the arm. 

No fixed rules can be established for the duration of neuralgia. Some- 
times the disease ends after a few paroxysms, perhaps even after one, while in 
other cases it may last for weeks, months, years, or even a whole lifetime. 
If the causes are such that they cannot be removed, it is impossible to count 
on the cure of the disease. The termination may either come so suddenly 
that the patient is abruptly relieved at the end of a violent attack, or else 
it is gradual, the attacks becoming weaker and shorter, and the intervals 
between them longer. For some time after the attacks have ceased there is 
likely to be disturbance of sensibility in the affected region of the skin. 

Neuralgia shows a distinct tendency to relapse. A new lesion will often 
affect the same nerve, but in some cases other nerves are affected by the re- 
lapse. A nerve that has once been attacked by neuralgia often remains so 
sensitive that the slightest deleterious influence is sufficient to call forth an- 
other outbreak of the disease. 

Upon closer consideration of the causes of neuralgia, an inherited ten- 
dency to the affection is of the utmost significance. In families in which 
neuralgia is frequent, the different members are affected by the malady. We 
often find in these families persons who suffer, or have previously suffered, 
from central neuroses, such as psychopathy, hysteria, neurasthenia, or epilepsy. 
Under such curcumstances there may be an inherited neuralgic predisposition. 



ETIOLOGY 839 

In other cases this neuralgic predisposition is acquired. Persons whose 
nervous systems have become deranged through physical or mental excesses, 
such as overwork, excessive use of alcohol, tobacco, tea, sexual irregularities, 
or the like are especially prone to neuralgia. The same causes often lead to 
hysteria and neurasthenia. Therefore, we do not wonder that neuralgia is 
often associated with these two last named conditions. 

Great loss of fluids and blood often cause neuralgia, especially in women 
after a difficult labor or too long continued suckling. 

Chlorosis undoubtedly favors the development of neuralgia. 

As predisposing causes, arteriosclerosis and advancing age as well as preg- 
nancy must be mentioned. 

The causes above enumerated are sufficient in themselves to produce neu- 
ralgia, but obviously the disease develops still more readily if, besides these 
general conditions, the nerves are affected by special lesions. In other cases 
these lesions alone suffice to produce neuralgia, for the neuralgic predisposi- 
tion is by no means essential for the appearance of neuralgia. 

Neuralgia is no doubt often caused by cold — refrigeratory or rheumatic 
neuralgia. We are less likely to doubt this because neuralgia frequently 
appears shortly after taking cold. In all probability the cold produces 
changes in the caliber of the blood-vessels, and these irritate the nerves. It 
would not be consistent with impartial observation to deny absolutely the 
possibility of neuralgia being caused by cold. On the other hand, it must 
be confessed that here, as in similar fields, many manifestations of disease 
have been attributed to cold whereas the cause was something very different — 
often infection. 

Indeed recent literature forces us to the conclusion that direct infection 
of the nerves plays an important part in the etiology of neuralgia. This 
point is brought out prominently by the fact that, like other infectious dis- 
eases, cases of neuralgia appear in great numbers within a short period of 
time and in a limited region. Nine years ago Benedikt x pointed out that 
during the transition from winter to spring he had seen numerous cases of 
supraorbital neuralgia in Vienna, and he could explain these only as the 
result of infection; and only a year ago Wille 2 described the epidemic appear- 
ance of neuralgia, chiefly intercostal neuralgia, in Oberdorf in the Bavarian 
Allgau where, among 1,000 inhabitants, he had seen in three months 108 
cases. Eeilly 3 also a short time ago described an epidemic of intercostal 
neuralgia which was occasionally accompanied by herpes zoster. Absolutely 
nothing was known of the source of infection. 

Traumatic neuralgia is of frequent occurrence and is important. The 
modes in which a nerve may be injured are so exceedingly numerous that it 
is impossible here to enumerate even the majority of them. Among the most 
common are falls, blows, contusions, wounds either incised or punctured, 
foreign bodies, inflammation of the bones and the periosteum, fractures 

OI. Benedikt, " Eine endemisehe Xeuralgie in Wien." Wiener mod. Wochensclirift, 
1891, Xr. 11. 

2 V. Wille, " Xeuralgia epidernica (localis)." Munchener med. Wochenschrift, 1900, 
Nr. 33-35.) 

3 Thomas F. Eeilly, " An Epidemic of Intercostal Xeuralgia." Med. Record, Nov. 
25, 1899. 



840 NEURALGIA 

of bones, dislocations, and the formation of callus and tumors. Weir 
Mitchell correctly states that bullet wounds of the nerves often cause the 
most violent form of neuralgia, for which he has introduced a special name, 
causalgia. At times very stubborn and excruciating neuralgia appears after 
the amputation of limbs, when amputation neuroma, so called, develops at 
the ends of the severed nerves. This form of neuralgia was thoroughly de- 
scribed a few years ago by Witzel. 1 Witzel emphasized the fact that all cases 
of amputation neuroma are not followed by neuralgia, but only those in which 
there are adhesions to the adjacent parts so that movements of the limb cause 
stretching or tension of the nerve in question. Hence it is not the amputa- 
tion neuroma, per se, but its adhesion to adjacent parts which is the causative 
agent of neuralgia. Witzel maintains, not without justification, that like cir- 
cumstances may often exist in other cases in which it has been the custom 
to consider pressure upon the nerves to be the cause of neuralgia ; for instance, 
in neuralgia produced by inflammation of the bones, the periosteum, etc. 

Secondary infectious diseases, toxic and autotoxic forms of neuralgia, 
may all be grouped together, for in the final analysis of these cases it is the 
toxic material which injuriously affects a sensory nerve. 

Under secondary infectious neuralgia we may class those forms which de- 
velop in the course of any infectious disease. It is well known that neuralgia 
not infrequently appears in the course of enteric fever and syphilis, also in ma- 
laria and in acute articular rheumatism. In gonorrhea I have often seen vio- 
lent and stubborn sciatica, and, in a few cases, crural neuralgia. Herpes zoster, 
which must be counted among the infectious diseases, is a not infrequent cause 
of neuralgia. The epidemics of influenza of the last decade have demonstrated 
that this infectious disease also forms a very fertile source of neuralgia. 

In many cases such forms of neuralgia assume the characteristics of a 
substantive disease which we call masked neuralgia. The best known of these 
is neuralgia intermittens larvata, which occurs in regions where malarial fever 
is prevalent, obviously as the result and, indeed, as the only result of an in- 
fection by the malarial plasmodium. The regular recurrence o£ neuralgic 
attacks, occasional slight enlargement of the spleen, and the quick disap- 
pearance of the paroxysms under treatment by quinin are important aids in 
the recognition of this form of neuralgia. In the future when making ex- 
aminations of the blood, special stress should be laid on the plasmodium 
malariae, the more so since there can be no doubt that neuralgia has a strong 
tendency to be intermittent even without the influence of malaria, and read- 
ily yields to the action of quinin. Seeligmuller, 2 for instance, has shown that 
intermittent supraorbital neuralgia not infrequently appears in connection 
with catarrh of the frontal sinus when there is a temporary retention of 
secretion which causes pressure on the nerves. I would say parenthetically 
that the use of the nasal douche often effects a rapid cure. The late clinician, 
Immermann 3 of Basel, deserves credit for having been the first to emphasize 

i O. Witzel, " Ueber die Entstehung und Verhiitung der Neuralgie in operativen 
Theilen, besonders an Amputationsstttmpfen." Centralbl. f. Chirurgie, 1894, Nr. 23. 

2 Seeligmuller, " Neuralgia supraorbitalis intermittens." Centralbl. f. Nervenheil- 
hunde u. gerichtl. Psychopathologie, 1880, Nr. 11. 

3 Immermann, " Ueber larvirten Gelenkrheumatismus." Verhandl. d. Congresses f. 
innere Medicin, 1885, p. 109. 






ETIOLOGY 841 

that numerous cases of neuralgia appear at periods in which acute articular 
rheumatism also prevails either endemically or epidemically, yet the patients 
do not suffer from swelling of or pain in the joints. The astonishingly rapid 
results which often follow the use of salicylic acid or sodium salicylate suggest 
that neuralgia of this kind owes its origin to the same noxious influences as 
acute articular rheumatism, therefore we must differentiate a second form of 
masked neuralgia. 

In all probability this field of masked neuralgia will be expanded in the 
future. As regards influenza, I maintain to-day that it occasionally mani- 
fests itself, just as malaria and articular rheumatism, in attacks of stubborn 
and very severe neuralgia. 

In all of the above cases the cause was probably bacterial toxins which 
affected the sensory nerves and produced neuralgia. Since it is a fact, how- 
ever, that neuralgia appears with varying frequency in many infectious dis- 
eases, we must conclude that all bacterial toxins have not an equally dele- 
terious effect upon the nerves. Moreover certain definite nerves, especially 
the supraorbital, are uncommonly often subjected to infectious influences, 
which indicates that bacterial poisons have a special predilection for certain 
nerves, and perhaps also that certain nerves possess a very slight power of 
resistance to some bacterial poisons. It is, of course, superfluous to say that 
all this is purely hypothetical. 

In toxic neuralgia we find exactly the same conditions. Alcoholism is 
not seldom the cause of very severe and protracted neuralgia, especially 
sciatica. But lead, mercury, copper, phosphorus, and arsenic may have the 
same effect. 

By autotoxic neuralgia we mean such forms as develop from impaired 
metabolism; for example, in diabetes, in gout, and in uremic conditions. I 
have frequently been consulted in cases in which neuralgia appeared during 
acute or chronic gastrointestinal catarrh which soon disappeared under 
proper treatment of the digestive disturbance. Even though we have reason 
to believe that abnormal products of metabolism have affected the diseased 
nerves, nothing is at present known of their chemical nature. From this 
point of view we may explain the fact that neuralgia is alleviated when we 
relieve constipation ; this Gussenbauer * noticed in trigeminal neuralgia. 

Neuralgia is frequently caused by disease of the nerves themselves. I 
mentioned above that it often appears as a sort of central neurosis, particu- 
larly in hysteria and neurasthenia. Anatomically demonstrable diseases of 
the brain, especially tumors and suppuration, are frequent causes of neural- 
gia. Often it is the consequence of disease of the spinal cord. It is well 
known that sciatica appears as one of the earliest symptoms in tabes dor- 
salis; and it is an important point in diagnosis to infer either locomotor 
ataxia or diabetes in an obstinate case of bilateral sciatica. Under certain 
circumstances other diseases of the spinal cord, for instance, multiple 
sclerosis, may also produce neuralgia. 

Besides the diseases of the peripheral nerves already referred to, we must 
mention neuritis which is often followed by neuralgia. 

i C. Gussenbauer, " Ueber die Behandlung der Trigeminusneuralgie." Wiener 
medic. Blatter, 1886, Nr. 32. 



842 NEURALGIA 

Notwithstanding its manifold causes the origin of neuralgia may now 
and then be obscure. Some cases have been interpreted as reflex neural- 
gia, and their occurrence has been attributed to the fact that lesions in other 
organs transmitted by way of the central nervous system produce neuralgic 
changes in nerve tracts that are sometimes quite remote. Diseases of the 
female sexual organs are considered to be especially common causes of neu- 
ralgia. 

Considering the frequency of the disease, little is known concerning the 
anatomical changes in neuralgia, but this is explained by the fact that 
neuralgia is rarely the immediate cause of death. Many investigations and 
reports must be regarded as inadequate because they are limited to the study 
of the affected nerves, without considering the central nervous system. It 
is not improbable that when the peripheral nerve course is unaffected neu- 
ralgia may sometimes be explained by anatomical changes in the spinal cord. 
But this could be verified only by the use of the most delicate methods at 
present known. 

I possess a series of microscopic preparations an account of which may 
be interesting. Under three different microscopes may be seen transverse 
sections of both sciatic nerves and the spinal cord of a man who suffered for 
more than a year from a very severe left sided sciatica brought on by trench 
digging, by working in the damp earth. He died of an intercurrent and, 
therefore, very extensive pulmonary inflammation, which afforded us an op- 
portunity to examine the nerves microscopically. Even a very careful scrutiny 
of the specimens showed nothing abnormal in the nerves or in the spinal 
cord, much less an actual pathologic lesion; this was especially evident on 
transverse section, for the neuralgic and normal sciatic nerves appeared to 
be no wise different. Other specimens from the same patient revealed no 
lesions. This led to the conclusion that nerves may be the seat of neuralgia 
for a long time without revealing the slightest anatomical change by the most 
minute methods of examination. Neuralgia of this kind, according to clin- 
ical usage, should be classed with functional nervous diseases or neuroses, 
and it may aptly be designated neurotic neuralgia. 

An entirely different state of things is present in the second series of 
microscopic specimens which I wish to describe. These were taken from an 
alcoholic who suffered for many months from bilateral sciatica, the left side 
being somewhat the more markedly affected. He succumbed from exhaus- 
tion. I placed transverse sections of the spinal cord and both sciatic nerves 
under the microscopes. In the case of the spinal cord I was unable to detect 
any change. But even an untrained investigator could hardly fail to discover 
in the peripheral nerves the signs of extensive disease. One of the sections 
of the sciatic nerve was stained and hardened according to Marchi's method 
in a mixture of hyperosmic acid and potassium bichromate, whereas the 
other has the characteristic appearance produced by Weigert's process — hema- 
toxylin, copper sulphate, and ferrocyanid of potassium. The preparations 
resemble each other in the fact that many of the nerve fibers have been de- 
stroyed, that others are beginning to disintegrate, that advanced changes are 
lacking in the interstitial connective tissue; in short, they reveal an extensive 
degenerative atrophy of the nerves which some might call neuritis, but in 
my opinion it is impossible to assume in these changes an inflammatory proc- 



ETIOLOGY 843 

ess. In any case it is apparent from our own observations that, anatomically, 
there may be a degenerative as well as a neurotic form of neuralgia. 

Undoubtedly there is still another kind of neuralgia, the neuritic form 
proper. There is an example of this also in my collection. It is a transverse 
section of a sciatic nerve which was for a long time the seat of severe neu- 
ralgia in a patient affected with diabetes. In this preparation the atrophied 
condition of the nerve fibers may be easily recognized, but more decided still 
is the distribution of the connective tissue between the individual nerve 
fibers — therefore, the endoneurium — above all, its unusual richness in cells. 
Under such circumstances we do not hesitate to make a diagnosis of inflam- 
mation of the nerves; in fact, of interstitial neuritis. Moreover, the spinal 
cord, as may be perceived through the microscope, presents no demonstrable 
change. Hence, from a purely anatomical standpoint, we may speak of a 
neuritic neuralgia as well as of neurotic and degenerative forms. 

When the experiences of other physicians are recounted, we find that 
relatively often parts of nerves affected by neuralgia and excised from the 
course of the nerve trunk as a curative measure have formed the material 
for investigation. Lasalle * reports a case of stubborn supramaxillary neu- 
ralgia in which Eanvier, a universally recognized authority in the field of 
histology, had undertaken the examination of the extirpated nerve. He 
found thickening of the connective tissue and degenerative changes in the 
nerve fibers ; in other words, inflammatory lesions. Putnam 2 had occasion 
to examine ten nerves from which portions had been excised on account 
of neuralgia. He noticed accumulations of round cells about the vessels, 
thickening of the vascular intima, here and there plugging of the vessels, 
proliferation of the connective tissue, necrosis and atrophy of the nerve fibers, 
and very slender nerve fibers which he was inclined to consider as newly 
formed ones. At all events, inflammatory changes appeared by far most 
frequently in the nerves affected by neuralgia. In a case of trigeminal neu- 
ralgia which Gerhardt 3 has described there was extravasation of blood 
into the Gasserian ganglion. Besides this, active pigmentation of the gan- 
glionic cells was noticed, although this was hardly considered to indicate 
disease. These anatomical reports make it apparent to every unprejudiced 
mind that neuritic, and we may also add, degenerative neuralgia is by no 
means so rare as it is to-day considered to be. The relative frequency with 
which neurotic neuralgia occurs in comparison with the two other forms 
cannot be determined with any accuracy because these different forms are 
not always sharply characterized by typical symptoms during the life of the 
patient. Many physicians consider rapid muscular atrophy to be a note- 
worthy sign of neuritic and degenerative neuralgia; but this is not diagnos- 
tically accurate, and no one can deny that in neuralgia the sensory tract as 
well as the trophic nerve fibers may be altered in a purely functional manner. 

1 Lasalle, " Etude sur le traitement de la nevralgie rebelle du nerf maxillaire sup6- 
rieur par la resection du nerf sous-orbitaire dans la cavite de l'orbite." These de 
Paris, 1877. 

2 J. J. Putnam, " Personal Observations on the Pathology and Treatment of Neu- 
ralgias of the Fifth Pair." Boston Med. Journal, 1891, Apr. 13. 

3 Gerhardt, " Zur Therapie der Erkrankung des fiinften Hirnnerven." Deutsches 
Arch. f. klin. Med., 1880, XXVI. 



844 NEURALGIA 

Certain local lesions might form a basis for the supposition of neuritic neu- 
ralgia. In affected nerves which are easily accessible to the fingers, excessive 
pain in the nerve trunk, or palpable hardening, would indicate inflammatory 
changes in the nerves. Naturally, neuralgia points to inflammatory processes 
as a cause; that is, they are usually present when a nerve is injured. To be 
sure, a certain amount of caution is necessary in diagnosticating the anatom- 
ical nature of neuralgia. This is especially true of those cases of neuralgia 
which develop from disorders of metabolism, and may equally well be either 
neuritic or degenerative in character. When neuralgia appears in mixed 
nerves, an inflammatory or degenerative condition would lead us to expect 
a change in the electric irritability of the affected nerves and of the muscles 
which they supply, as well as muscular atrophy. Undoubtedly this happens 
very rarely, and from my own observations I must emphasize the fact that 
a normal condition of electric contractility by no means implies the absence 
of neuritic or degenerative neuralgia. A few years ago, when Eulenberg 
pointed out the occurrence of changes in electric contractility in certain cases 
of sciatica, his assertions were met with general disbelief. But Nonne x has 
shown that this is by no means erroneous, and in a few cases I have observed 
the same changes. 

All of our present knowledge of the anatomical changes in neuralgia 
points to the conclusion that these, if found at all, will appear in the affected 
peripheral nerve paths themselves. May we deduce from this that the seat 
of neuralgia is always to be sought in the peripheral nerves, or is the affec- 
tion of the peripheral nerve path merely the occasion for the development 
of a neuralgic condition in the central nervous system which may continue 
to exist after the peripheral cause has been removed? That neuralgia of this 
form does occur I am firmly convinced, for experience proves that neuralgia 
may remain after the peripheral cause has been removed, no matter by what 
means. On the other hand, it seems to me to be assuming too much to attrib- 
ute to this origin all cases of neuralgia. Abundant observations have demon- 
strated that, after removal of a peripheral center of disease, for instance, by 
operation, neuralgia of the one nerve ceased instantaneously and permanently. 
This scarcely seems possible in a neuralgic condition of the spinal cord, and 
the opinion of Anstie, who regards neuralgia as invariably caused by abnor- 
mal processes in the posterior roots of the spinal cord, can hardly be consid- 
ered more than a conjecture without real foundation. 

We are still absolutely ignorant of the reason why neuralgia appears par- 
oxysmally; neither do we quite understand why certain nerves are so much 
more frequently affected by neuralgia than others. Of course no one will deny 
that nerves which are superficially situated, which have an extended course, 
and which run through long, bony canals, are especially liable to be damaged, 
and we therefore understand why neuralgia is most common in the region 
of the sciatic and the trigeminal nerves. But this circumstance does not ex- 
plain why the supraorbital nerve is so often affected in malaria. We know 
only, for instance, when we recall a case of lead paralysis, that certain poi- 
sons possess a special, although so far unexplained, predilection for certain 

i Nonne, " Entartungsreaction bei primarer Ischias." Berl. klin. Wochenschr., 
1887, Nr. 45. 



TREATMENT 845 

nerves; lead for the radial nerve, and the malarial poison for the supra- 
orbital. 

Some clinicians have suggested that we apply the name neuralgia only 
to those affections in which anatomical changes in the nerves can be demon- 
strated. The form which we call neurotic neuralgia has also been known as 
true or idiopathic neuralgia. In my opinion this nomenclature is incorrect. 
Neuralgia under any conditions is merely a symptom, and therefore the diag- 
nosis of neuralgia is incomplete. On the contrary, we should ascertain by 
minute investigation from what causes and what anatomic changes it springs. 
Rather than lay stress on distinctions between true and false neuralgia, physi- 
cians should be careful to apply the term neuralgia to only such pain as cor- 
responds exactly with our above description; namely, pain which is limited 
to the area of distribution of a nerve and which appears paroxysmally. 
We must therefore search for the origin in each individual case because 
this will determine the treatment; moreover, it sometimes happens that the 
appearance of neuralgia reveals the existence of certain definite diseases. 
Stubborn intercostal neuralgia is often the first tangible sign of aneurysms 
which have developed insidiously. An obstinate case of bilateral sciatica may 
be the first symptom of diabetes or tabes dorsalis. 

Prognosis. — The prognosis of neuralgia is so far favorable that the 
paroxysms, per se, are not fatal ; but it is unfortunately true that many cases 
respond but slightly or not at all to treatment, and in these the patients 
suffer unbearable agony. The neurotic forms of neuralgia show the greatest 
tenacity, especially when they appear in one in whom the predisposition 
already exists. Even when the cause is perfectly obvious, it is sometimes 
impossible to effect a cure. 

TREATMENT 

In conclusion we shall discuss the treatment of neuralgia; I must state 
in advance that I shall make no attempt to give a complete summary of all 
the methods recommended, but shall consider my task accomplished when 
I have suggested certain fundamental measures. 

The first question to be asked in treating neuralgia concerns the cause, 
for upon this our therapy will largely depend. And here we must assign due 
weight to what has been called the etiological indication. Malarial neuralgia, 
for instance, is, as a rule, quickly and permanently cured by quinin which 
has been known for centuries as a trustworthy specific for malaria. The 
dose should not be too small, but about 2 grams should be given four hours 
before the expected paroxysm; in the next three days 1.5 — 1.0 — 0.5 grams 
should be administered in order to guard against a relapse. It can be most 
conveniently taken in capsule, and should be given every quarter of an hour 
in doses of 0.5 until the desired amount has been taken. Of late there has 
been an attempt to substitute for quinin euchinin, an ethylcarbonic ester of 
quinin, as its less bitter taste makes it less objectionable. When patients have 
a tendency to vomit quinin, it can be given in the form of an enema. For 
this purpose it should be mixed with 50 of tepid water, 5.0 starch flour, and 
10 drops of tincture of opium, and slowly injected into the rectum. It often 
happens, however, that after giving quinin enemata for several successive 



846 NEURALGIA 

days, there are signs of irritation of the rectal mucous membrane, such as 
pain, tenesmus, and inability to retain an enema. One can still inject the 
quinin hypodermically, using a warmed solution in glycerin; for instance: 

IJ Chinini hydrochlorici, \ 

Glycerini, I aa 5.0 

Aq. destill. 
M. D. S. : Heat the solution in warm water, and inject daily %-l Pravaz 
syringeful in the vicinity of the affected nerve. 

If syphilis is the cause of neuralgia, mercury and iodin preparations are 
indicated. To be sure, Ehrmann's x experience warns us to be cautious in the 
use of iodin, since this may sometimes cause an attack of neuralgia. In neu- 
ralgia polyarthritica larvata Immermann saw excellent results from salicylic 
acid and sodium salicylate (1.0, one powder every two hours until tinnitus 
aurium appears). Neuralgia in diabetes is greatly relieved or even entirely 
cured when we diminish, by a carefully regulated diet, the amount of sugar 
in the urine. I have treated diabetic patients who could tell exactly from 
the violence of their neuralgia whether their urine at that time contained 
much or little sugar, and who regulated their dietary accordingly. At times 
surgical interference is necessary; that is, the removal of tumors causing 
pressure and the breaking up of perineuritic adhesions. With anemic, weak, 
and nervous patients we must necessarily prescribe preparations of iron, 
arsenic or nervines (for example, the bromids), strengthening diet, and a stay 
in the country or in the mountains. Altitudes of over 1,500 meters should, 
however, be avoided since many people are attacked by neuralgia when at 
great heights. The same may be said of the seashore, where sudden changes 
of temperature and of the weather readily produce neuralgia. 

Under all circumstances people with neuralgia should partake of a nutri- 
tious and easily digested diet, and should avoid mental excitement. The use 
of alcohol cannot be recommended, as it is usually followed by more frequent 
and more severe attacks. The same is true of strong coffee, strong tea, pun- 
gent spices and acids, and the excessive use of tobacco. I have previously 
pointed out how important it is that the bowels should act regularly; if neces- 
sary, mild aperients may be used. 

In the case of neuralgia of the trunk or of the nerves of the extremities, 
I lay the greatest stress on prolonged rest in bed. One advantage of this 
is that the body is kept continuously at a comparatively equable temperature, 
and it affords the best means of resting the affected nerve. For instance, 
I consider the treatment of sciatica or of intercostal neuralgia with rest in 
bed far more valuable than the use of medicines, and I am convinced that 
many of my patients owe their recovery to this rather than to incidental 
remedies. In neuralgia of the brachial nerve it is advisable to carry the arm 
in a sling. Great relief can also be obtained by the use of a suitable invalid's 
chair. 

Besides the few examples above cited (malaria, syphilis, articular rheu- 
matism) but few specifics for neuralgia are known, although there is no lack 

i S. Ehrmann, " Ueber Trigeminus neuralgien bei acutem Jodismus." Wiener med. 
Bl. y 1890, Nr. 44. 



TREATMENT 847 

of suggested remedies. I have seen no results from methylene blue (0.3, one 
powder 3 times a day) as advocated by Ehrlich, 1 from turpentine (20 drops 
three times daily in milk), from potassium iodid (5.0 in 200, 15 c.c. three 
times daily) or from, other supposed specifics. 

Therefore, the treatment of neuralgia must be purely symptomatic. 
According to my observations the most beneficial effect must undoubtedly 
be ascribed to the narcotics, and among these to morphin. If neuralgia 
resists continued rest in bed and a suitable morphin treatment hope of a 
quick recovery can be no longer entertained. A dread of morphin, which 
is frequently found both among laymen and physicians, is quite ground- 
less, provided, of course, that it be used with discretion. Above all the 
patient must never for a moment have morphin or the morphin syringe in 
his possession, for he may easily acquire the morphin habit which is exceed- 
ingly difficult to conquer, and continue the use of morphin long after his 
neuralgia has disappeared. I wish to say most emphatically that even physi- 
cians who suffer from neuralgia should not give themselves morphin injec- 
tions. Unfortunately morphinism is not uncommon among physicians. I 
have come in contact with no small number of colleagues in whom morphinism 
developed by self-treatment with morphin for neuralgia. 

In treating neuralgia by morphin we must make a distinction between 
the attempt to relieve the patient from one specific attack, and the radical 
cure of the disease. Xot infrequently the pain is so unbearable that we are 
forced to relieve it as speedily as possible by a hypodermatic injection of 
morphin. This can, however, be accomplished only if one injects a sufficiently 
large dose, at least 0.01 gram. 

^ Morphini hydrochlorici 0.3 

Glycerini, ) ._ g Q 

Aq. destill. j ' 
M. D. S. : J— J a Pravaz syringeful hypodermatically. 

In neuralgia of recent origin a single injection of morphin will sometimes 
effect a lasting cure. After a time, as a rule, there are subsequent attacks, 
but the intervals between these'becomes decidedly longer, and the attacks them- 
selves lighter and shorter. Daily injections of morphin should produce a 
more or less rapid cure. I recommend strongly the method of choosing each 
day a more distally located point of the nerve course for the seat of injection. 
In this way I have in many cases affected a complete cure, especially of pro- 
tracted sciatica, in a relatively short time. 

Far be it from me to recommend morphin as a remedy to which all cases 
of neuralgia will yield. But, on the contrary, when morphin has failed I 
have never known a result to be produced by other narcotics, such as chloral 
hydrate, cocain, or butylchloral as in the following formula: 

^ Butylchlorali hydrate 5.0 

Aq/ destill 130.0 

Glycerini 30.0 

M. D. S. : 15 c.c. every 5 or 10 minutes until the pain is relieved. 

i P. Ehrlich and A. Leppmann, " Ueber schmerzstillende Wirkung des Methylen- 
blau." Deutsche med. Wochenschrift, 1890, Nr. 23. 
55 



848 NEURALGIA 

I wish to add particularly that the use of sulphate of atropin (atropini 
sulfurici 0.01, glycerin and aq. destill. aa 5.0, J-J of a syringeful hypoder- 
matically injected) which has been so greatly praised, has failed in all of my 
patients. Although one often hears the statement made that neuralgic pa- 
tients bear unusually large doses of narcotics astonishingly well, we must 
not place too much confidence in this; for, as a rule, the patients are able 
to take large doses of remedies to relieve pain without immediate injury 
simply because they have become accustomed to them during their illness. 

Occasionally good results are obtained by antipyretics and antirheumatics. 
Quinin, for example, often relieves neuralgia even when a malarial cause 
is out of the question. However, in such cases it is necessary to prescribe 
large doses, at least 2.0 grams or even 5 grams or more. Some patients are 
greatly relieved or even cured by the use of salicylic acid (1.0 every two 
hours), sodium salicylate (1.0 every two hours), aspirin (1.0 three times 
daily), phenacetin (1.0 three times daily), kryofin (0.5 three times daily), 
lactophenin (0.5 three times daily), antipyrin (1.0 three times daily), analgin 
(1.0 three times daily), exalgin (0.3 three times daily), tincture of gelsemium 
(15 drops three times daily), tincture of aconite or tincture of colchicum (10 
drops three times daily). 

Among the nerve tonics from which we may most readily expect good 
results, I must mention the bromids, and, after these, arsenic. Other nerve 
remedies have also been tried and praised; for instance, nitrate of strychnin, 
extract of ergot, phosphorus, zinc oxid, zinc valerianate, nitrite of strychnin, 
Among the bromids I prefer sodium bromid, potassium bromid and ammonium 
bromid. For instance: 

5 Sol. natrii bromati 10.0 :200 

Add kalii bromati et ammonii bromati aa 5.0 

M. D. S. : 1.0 c.c. three times daily. 

In cases of anemia, arsenic should be combined with iron. For example: 

ly, Tincturse ferri chlorati aethereae 40.0 

Liquor kalii arsenicosi 10.0 

M. D. S. : 25 drops three times daily. 

Or, ferri lactici, natrii bromati aa 10.0; acidi arsenicosi 0.1, pulv. rad. 
altheae q.s. ut f. pil. No. 100. D. S. : 2 pills three times daily, a half-hour 
after meals. 

Among external remedies the derivants are much used. To these belong 
mustard paste, mustard leaf, cantharidal plaster, friction with spirits of 
camphor, mustard oil, formic ether, chloroform (linimenti ammoniati, 40, 
or chloroformi 10.0, olei terebinthinae, 40.0) or friction with pure turpen- 
tine and painting with tincture of iodin. Stimulating ointments are not 
infrequently used, for example, veratrini, 0.5, adipis lanse or adipis suilli 
aa 5.0, rubbed into the painful parts morning and evening. In neuralgia 
of the nerves of the extremities, dry and wet cupping over the affected nerve 
trunk are often resorted to. Cauterization is at times effective. Paquelin's 
cautery is now preferred to the old actual cautery; moxas and heated points 
are also used. In neuralgia of recent origin we should be very cautious in 
the use of derivants, for these are occasionally succeeded by more prolonged 



TREATMENT 849 

and more violent attacks. Fontanels and setons also belong to the category 
of derivants, but are now seldom employed. 

The electric current is also liable to increase the pain, especially in a 
fresh case. As a rule, I prefer the galvanic current', but at first it should 
not be too strong nor used for too long a time. Treatment with the anode 
is best adapted to affected nerves, and if there are painful points these should 
be especially subjected to its influence. Stubborn neuralgia with painful 
points along the spinal column has frequently been known to disappear under 
galvanic treatment. Cases have been reported in which very weak but long 
continued galvanic currents produced admirable results; these were brought 
about by connecting copper and zinc plates with a metallic conductor and plac- 
ing them over the affected nerves. 

The faradic current is often used in the form of the faradic brush or the 
faradic moxa. The faradic brush is usually applied to the skin over the 
entire course of the nerve, while the faradic moxa is used only over definite 
areas, especially the painful points. 

Franklinization is a method of electrical treatment not yet generally 
adopted into practice, but is used by a few specialists. Its superiority to the 
galvanic or faradic current has not yet been demonstrated. 

Electric oaths are less used for neuralgia than for general nervousness, but, 
as a rule, they fail to give the results originally hoped for. 

Electric light oaths have recently been tried, but in this the electricity 
is probably of less benefit than the warmth. 

Many patients are relieved by the application of extreme cold or of con- 
siderable heat to the part. Pieces of ice, or an ice-bag, are applied to the 
skin over the affected nerve, or it is sprayed with ether, methyl chlorid, or 
ethyl chlorid. Vapor baths, Irish-Roman baths, sweat boxes, sand and mud 
(Fango) baths have also been employed. For sciatica I often lay the affected 
leg on bags filled with hot sand or cooking salt. Astonishingly good results 
are sometimes produced by the Scotch douche, which consists of alternating 
streams of hot and warm water. 

In chronic neuralgia bath cures are sometimes beneficial, especially the 
indifferent springs (Pfaffers, Ragatz, Teplitz, Wildbad, Wildbad-Gastein), 
sulphur, salt and mud baths. I have known the malady to be relieved by 
treatment in a cold water sanatorium after everything else had been tried in 
vain, even Kneipp's treatment. In most of these cases, ■ however, the pain 
subsequently increased considerably. 

Mechano-therapy, cautiously employed, is often effective, especially in 
chronic neuralgia. 

Massage is considered by its enthusiastic devotees as an infallible remedy, 
but I happen to know of not a few cases in which massage, although per- 
formed by experienced persons, had to be stopped because it aggravated the 
neuralgic pain. Recently the field of mechano-therapy has been greatly ex- 
tended by the introduction of vibratory instruments, and Naegeli has pro- 
posed certain manipulations for combating neuralgia. 

Notwithstanding the fact that even the great Konigsberg philosopher, 
Immanuel Kant, became interested in the cure of painful sensations, hypnosis 
and suggestion, in other words so-called psychotherapeutics, yield only insig- 
nificant results. Anatomical changes cannot be removed by suggestion. It 



850 NEURALGIA 

is always wise to encourage the patient to bear the attacks as quietly and 
patiently as possible, for anxiety, restlessness and excitement tend to increase 
their violence and frequency. 

Surgical treatment of neuralgia is generally a last resource, provided 
anatomical changes in the affected nerve and its surroundings cannot be dem- 
onstrated. For one not a specialist in surgery the simplest procedure is to 
employ subcutaneous injections near the affected nerve with substances which 
will destroy the nerve fibers. Water, ether, carbolic acid, and hyperosmic 
acid have been used for this purpose. But the method does not insure last- 
ing recovery, for neuralgia frequently returns as soon as the destroyed nerve 
fibers have reformed. If the seat is in a mixed nerve, permanent paralysis 
may be the result. Bloodless nerve stretching and compression of the large 
arteries are therefore preferable. In stubborn cases of sciatica I have seen 
admirable results from bloodless nerve stretching. By this method the leg 
is stretched at the knee-joint and then pressed as firmly as possible against 
the abdomen. Gerhardt describes a case of trigeminal neuralgia in which 
compression of the carotids relieved the pain. It is a much more serious opera- 
tion if we lay bare the affected nerve with the knife, and then stretch it by 
means of a forceps (a procedure known as bloody nerve stretching) or if we 
ligate the large arteries. Often the affected nerve is severed (neurotomy) 
or, in order to prevent as long as possible a new formation, larger portions 
of the nerve are excised (resection, neurectomy) or, as Thiersch suggested, 
pieces of the nerves are extracted from the bony canals by means of forceps 
(nerve extraction). Operations are not always crowned with permanent 
success. If the point of exit in the course of the peripheral nerve is not 
removed there is no relief, and if nerve conduction is reestablished by the 
new growth of nerves the cure will be but temporary. Very extensive opera- 
tions have been undertaken in the hope of removing the cause of neuralgia; 
for example, the trigeminal nerve itself has been traced into the cranial cavity 
in order to cut through the nerve toward the center from the Gasserian 
ganglion and to remove it. 

Abbe, 1 Chipault 2 and others cut with the knife as far as the sac of the 
spinal dura mater in order to sever the posterior spinal roots within it. As 
regards the formation of new nerves, it should be borne in mind that this 
may take place even when a portion of the nerve trunk several centimeters 
in length has been excised. Operation is unsuccessful if the neuralgic con- 
ditions have become so deeply seated in the central nervous system that 
section of the peripheral nerve tract has no effect. It is wise, therefore, to 
be very conservative and not to prognosticate complete cure after an opera- 
tion. In mixed nerves, motor paralysis will be the inevitable result of direct 
section of the nerve trunk; therefore one should consider seriously whether 
the ensuing motor paralysis will not be a more serious disability than the 
neuralgia. In any case, we could to-day scarcely find a surgeon who would 
amputate a limb because of neuralgia as the earlier physicians have occa- 
sionally advised. 

i R. Abbe, " Intradural Section of the Spinal Nerve for Neuralgia." Boston Medical 
and Surgical Journal, 1896, No. 14. 

2 A. Chipault, " Du traitement de certaines neuralgies rebelles." Gaz. des hop., 
1898, No. 8. 



HEADACHE AND MIGRAINE 

By L. EDINGER, Frankfort-on-the-Main 

When the young physician who has finished his clinical studies begins 
to practise medicine he is confronted by a number of conditions for which his 
previous instruction has not sufficiently prepared him. Among well-to-do 
patients many cases differ entirely in type from those which he had previously 
seen as a student of medicine, and which had been impressed upon his mind 
at the bedside — patients who had been carefully nursed, and prepared by 
much preliminar} r examination, etc., to be utilized in clinical instruction. 
Although he has regularly attended the Out-Patient Department, a number 
of mild disturbances are almost unknown to him, because patients who resort 
to the Clinic do not seek professional aid for these minor ailments. What, 
however, collegiate instruction has not given him, and cannot confer, is a 
correct estimate of the relative frequency of individual affections. Only in 
practice or from the nature of his work in the district in which he practises 
does he learn their range. Every experienced physician utilizes this knowl- 
edge consciously or unconsciously, and this involuntary conclusion as to what 
is likely to occur often gives him a great practical advantage over the less 
experienced beginner. Many a physician will, like myself, recall with mor- 
tification some of the experiences of his first years of practice, when he did 
not recognize what was most obvious. The obvious conditions, however, are 
the most frequent ones. Even the most intelligent of our young physicians 
are apt to take entirely too serious a view of the condition of their patients 
during the first years of practice. They have seen so few trivial cases that 
it is not at all remarkable that they are inclined to make serious diagnoses 
or prognoses. 

FIRST EXAMINATION. DIFFERENTIATION OF TRUE HEADACHE 
FROM ALLIED AFFECTIONS 

These considerations occur to me whenever, in consultation with a younger 
colleague, I see a case of long continued headache which does not conform 
to the ordinary type of migraine. The more he has studied, the more likely 
is he to behold in each case a number of grave affections. True, we should 
never forget how serious a symptom headache may be under some circum- 
stances. The knowledge, however, is more important, that most cases of head- 
ache are not caused by severe general affections. If they are actually partial 
phenomena of such, with some care this can almost always be recognized; 
it is certainly true of the pain in the initial stage of febrile infectious dis- 

851 



852 HEADACHE AND MIGRAINE 

eases, true, also, of the pain of nephritis; in most cases headache which is 
accompanied by delirium represents a symptom of disease in limited areas 
of the skull; above all, that form which accompanies the varieties of lepto- 
meningitis, and which may readily be recognized; but to these I shall revert 
later. First, I wish to describe some affections which, causing pain in the 
head, are often regarded as true headache. 

The various forms of neuralgia in the head form a special obstacle to 
diagnosis which, in my experience, is rarely passed with safety. Only recently 
I saw a young man who was treated for months with numerous remedies be- 
cause of an exceedingly severe " headache," yet found not the slightest relief. 
An examination, which was easily made, and was based upon his report that 
the pains occurred only on the left side, particularly in the frontal bone, 
revealed that the condition was one of supraorbital neuralgia. The point 
of exit of the supraorbital nerve upon the left side was much more sensitive 
to pressure than that upon the corresponding right side ; in the entire region of 
the painful nerve the touch of a cold key was felt to be much colder than in 
other parts of the head. " A single application of ethyl chlorid upon the point 
of exit of the nerve caused absolute cessation of pain, and this permanently 
ceased after the same region had been repeatedly subjected to this treat- 
ment within the next few days. During the last decade which was so rich 
in cases of influenza, I more or less rapidly cured quite a number of such 
patients. Nearly all of them came to me with the diagnosis of " headache." 
Not always is supraorbital neuralgia so rapidly cured, else a diagnosis ex 
juvantibus might be made from this fact alone. There are affections of the 
frontal sinuses which present the same typical picture and cause the physi- 
cian great diagnostic and therapeutic perplexity. 

Besides true neuralgias, there are radiating pains in the head due to dis- 
ease of isolated portions of this structure. When a unilateral pain in the 
temporal region is complained of, it is always wise to have the teeth exam- 
ined. In caries, particularly of the upper and lower molars, there may 
be excessive pain in the temples which increases during the night, even 
though the tooth itself does not show that it is diseased. Some time ago I 
saw the extraction of a tooth relieve pain which, localized for eight weeks 
in the temples, had robbed the patient of sleep, and had made her extremely 
uncomfortable. Several physicians had in this case diagnosticated " atypical 
headache." Diseases of the pharynx and of the middle ear may also 
cause unilateral localized pain which at first resembles headache. When a 
patient is brought to us with the report that he suffers almost constantly 
from increasing pain upon the side of the head, in front of the ear, or imme- 
diately behind it, the examination of the ear and, in the latter case, also of 
the pharynx, should never be neglected. If we find in these areas a patho- 
logic process which can be removed, the pain in the head will soon cease; all 
will agree with me that, especially in the treatment of headache, rational 
therapy should be based upon a careful examination. 

We should never fail to secure an accurate description of the symptoms 
from the patient himself. Here, too, I have often seen young colleagues err, 
for, having in mind the precise histories which they have heard in the Clinic, 
they often fail to give the patient sufficient time to gain the composure 
necessary for most persons when they relate their observations of themselves. 



FIRST EXAMINATION 853 

Pains are subjective sensations, and their extent depends entirely upon the 
person who experiences them. He alone is able to estimate their severity; 
objective examination will reveal to us the cause, but never the intensity of 
the symptoms. Therefore, it is always well to permit the patient to complete 
his description. When we shall have learned to what causes the various 
forms of head pain are due, we will have learned more from the descriptions 
of the patients than from their examination. 

I repeatedly encounter the statement that patients exaggerate their pain. 
Now we possess no standard by which to estimate the suffering which a cer- 
tain affection causes some one else. I therefore believe it right to assume that 
a person suffers just as intensely as he says he does. At all events, we must 
be humane provided nothing directly indicates that the patient is a malingerer 
or exaggerates. I have frequently seen hysterical patients who suffered se- 
verely from lack of attention to their symptoms, and whose cases were never 
sufficiently studied. "We will help ourselves and our patients much more if 
we devote to these cases the same consideration that we do to others. 

Since the extent of the pain, or the intensity of its expression, depends 
entirely upon the sensibility of the patient, in my professional opinion the 
entire nervous condition of the patient must be taken into consideration. 
By a neglect of this truly self-evident fact, stupendous errors have occasion- 
ally been made. For example, here and there a mere " hysterical head- 
ache " is described as being of peculiar type, while the consideration of cases 
belonging to this category reveals only that hysterical persons, and also other 
hypersensitive people, by their constant and loud complaining when attacked 
by severe headache, often cause the physician to fail to recognize or to errone- 
ously explain a picture otherwise familiar to him. Xo doubt all of the cases 
of " hysterical headache " which have been brought to me could be explained 
as due to a cause which was felt in an exaggerated way or so described. Only 
such cases may be designated as true hysterical headache as arise without a 
local lesion, and purely from the outward projection of central disturbances. 
That such cases exist has not yet been proven and it is even unlikely, for up 
to the present time no area in the brain is known to be merely the sensory 
sphere for the cranium. 

A particularly instructive case of "hysterical" headache is the following: A young 
woman, aged 25, was attacked during pregnancy by headache which so increased in a 
few days as to become excruciating. The seat of pain was first said to be the left 
temple and the vertex of the parietal bone; but, gradually, the pains felt during the 
day and especially increasing during the night until they became almost unbearable 
radiated over the entire left half of the head; they extended to the left side of the 
neck which felt as if it were forcibly compressed, and thence the pain shot to the 
shoulder and the left arm, attacks which might almost be regarded as epileptic recur- 
ring repeatedly. At first there was vomiting. The attacks were more rare after a 
few weeks, but the intensity remained the same, and this increased until labor set in, 
about three months after the onset of the headache. During this time everything was 
done to relieve the attacks. Nothing was of use, nothing relieved the distressing pain. 
The physicians in attendance considered every diagnostic possibility from simple func- 
tional headache to intracranial disease. Finally, after the labor it was decided to 
examine the teeth. Two were found to be carious, and after their extraction the 
symptoms suddenly and completely disappeared. The patient had passed through a 
long period of suffering from which she might have been exempt if, on account of the 
temporal headache, the teeth had been at once examined as I have just advised. When 
I saw the patient a year later, in consequence of the reappearance of severe headaches — 



854 HEADACHE AND MIGRAINE 

this time after a psychical irritation — I found the anemic, nervous woman to have 
a typical, hysterical weakness of the arm with anesthesia in this area. 

Our knowledge of the past history of the patient must be supplemented 
by a report as to the frequency, the type, and the duration of the first attacks, 
also as to the period of life when the first attacks appeared. Naturally we 
must ascertain also what diseases have preceded and — a very important point 
— to what extent neuroses or psychoses, particularly epilepsy and headache, 
have occurred in the family. While the patient is giving us this information, 
we have opportunity to acquaint ourselves with their general constitutional 
condition, the circulation of the blood in the head, particularly in the con- 
junctiva, their psychical habitus, sensitiveness, etc. 

Then the head should be examined; first the exit of the nerves, and then 
the muscles of the neck and their insertion into the occipital bone are to be 
palpated. Simultaneously the lateral cervical vertebral column should be 
examined. An examination of the pupils as to their size, reaction to light, 
and power of accommodation must be included, and the local examination 
is completed by a mild percussion of the head which may be done with the 
finger. In most cases the history and this simple examination , will indicate 
our subsequent procedure. 

The first question which we ask all patients who consult us for headache 
refers to the nature of the pain. I usually say to them, " We physicians dis- 
criminate between headache and pressure in the head. To which would you 
refer your symptoms ? " In many cases I learn at once that no actual pain 
is felt, but that my patient complains only of pressure in the head. This 
disagreeable symptom is an accompaniment of neurasthenia. Tired persons, 
those who are overworked, persons who perform mental work in close rooms, 
or even those who work too steadily, also those who are hereditarily weak, 
who have over-exerted themselves either bodily or mentally, those who have 
had severe diseases — influenza here jAslys an important role — and exerted 
themselves too soon afterward — all may suffer from head pressure, a sensa- 
tion which is felt in the entire top of the skull, which may be so extreme as 
to prevent all work, and is the most prominent symptom among persons who 
are otherwise debilitated. There is no positive finding in the head to ac- 
count for the sensations of these patients. Provided there are no other 
symptoms which directly point to neurasthenia the patient's description of 
his case is the only aid to the diagnosis. This description is invariably uni- 
form; there is a sensation of severe pressure especially early in the morning 
upon awakening, and this continues during the entire day, sometimes amelio- 
rating toward night. The general treatment of neurasthenia will be discussed 
in another article. Here I shall only remark that a few measures have proven 
very serviceable, especially for the pressure in the head. These patients 
should never rise from bed upon an empty stomach; they should take a little 
bread early in the morning, or even a piece of chocolate will suffice; the in- 
tervals between eating should never be longer than two hours, and late at 
night directly before going to sleep they should take food, perhaps gruel or 
zwieback, so that the interval between the night and morning meal is lessened. 
For these intervals between meals English biscuit are very serviceable because 
they can be so readily taken during the day. Early in the morning, at mid- 



FIRST EXAMINATION 855 

day, and in the evening I prescribe a lukewarm douche of the forehead given 
for a few minutes with a Weber's eye douche from a low standing vessel to 
which a rubber tube and sprinkler are attached. Some patients also use with 
decided advantage a 10 per cent, menthol pomade once or twice during the 
day. The numerous water fanatics are, primarily, to be restrained from fre- 
quently douching the head, and cold douches must be absolutely prohibited. 
For those patients who are able to give up their occupation for a time, a 
stay in a sanatorium or a residence in the mountains or at the seashore will 
benefit the neurasthenia. On resuming their occupations, the treatment must 
be continued as long as symptoms exist or when they reappear. 

By far the majority of patients with pressure in the head require psychical 
treatment. They are almost all depressed and morose; occasionally they 
cherish a secret fear that they are beyond aid, that they are in the first 
stages of softening of the brain. The fear of this has probably driven many 
of these patients to commit suicide, and thus escape a dreadful future. These 
we may greatly help by serious advice; not by pronouncing the affection as 
very slight; yet at the same time, we may conscientiously maintain that 
pressure in tlic head is not one of the pi'ecursors of paralysis, that, so far as 
I am aware, it never precedes this disease. The symptoms of the paralytic 
which resemble neurasthenia must be referred to other regions. 

The prognosis of the affection is not grave provided the patients can 
decrease their hours of labor and interrupt it entirely several times during 
the year. I have observed that it is better to take frequently a short time 
for rest, than, as is the custom, to take from four to six weeks once a year. 
Of course, patients in whom this affection has existed for a long time will do 
well to stop work for a greater period. 

If we learn from our patients that they do not suffer from pressure but 
from actual pain in the head, we must ascertain whether this is due to a 
chronic affection, whether it is relatively persistent, or whether it appears 
in paroxysms between which there are intervals of perfect freedom. In most 
cases the affections running tlieir course with headache may easily be differ- 
entiated from migraine. The attack of migraine with its aura, its sudden 
onset, its relative increase, its prostration, and the vomiting which is almost 
invariably present, is a typical, circumscribed, clinical picture. Much con- 
fusion has arisen from the fact that many physicians do not discriminate 
between this and other forms of headache. The pain in the head in migraine 
plays a predominant, but not the only, part. Before concluding this article 
we shall discuss this affection, and shall first consider those diseases which, 
although of diverse nature, are included in a common group based upon the 
symptom of headache. 

To make a diagnosis we should not for a moment forget that headache 
is not a disease, sui generis, but merely a symptom. I believe it a serious 
omission in the development of our scientific knowledge of headache that this 
point has been so rarely considered. We could scarcely have attained our 
present accuracy in the diagnosis of diseases of the lungs if the symptom 
(cough) had been persistently studied as an affection in itself. Perhaps it 
is due to the incorrect methods which have previously obtained that we only 
to-day differentiate a number of maladies in which headache at first consti- 
tutes the sole recognizable symptom. 
56 



856 HEADACHE AND MIGRAINE 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 

It has previously been impossible accurately and theoretically to describe 
headache. We are familiar with some of its main causes, and simultaneously 
with a knowledge of the causes we have obtained an insight into the methods 
of cure. Therefore, at present it appears advisable to regard the entire con- 
dition from an etiologic standpoint. 

A. FORMS WITHOUT MARKED ANATOMICAL DISTURBANCE 

Many perfectly healthy persons suffer occasionally from headaches of short 
duration but of marked, intensity if they undergo unusual mental or bodily 
strain, or when they do not rest after such exertion. In some they occasion- 
ally occur after remaining up all night, in others after excesses of any kind, 
particularly after indulgence in Baccho. Others surfer from violent head- 
ache after any unusual bodily exertion, such as mountain climbing. Others 
cannot endure being suddenly awakened, and after such awakening they have 
headache. In others this follows the omission of their accustomed midday 
nap. I can endure an intense mental strain, but am certain to suffer from 
headache if, after such strain, I must for any reason curtail my hours of 
sleep. 

We know also that some persons are especially sensitive to intense irrita- 
tion of the organs of special sense, in many headache follows a loud noise, 
exposure to an intense light, or, in others, an intense odor which they find 
especially unpleasant. 

Under some circumstances mild headache may occur without the person 
having been subjected to any strain, as, frequently, after mental shock and 
often after gastric disturbances. In some persons an empty stomach predis- 
poses to an attack. A physician, a friend of mine, is always attacked by 
headache if he is obliged to see patients before breakfast. 

In these slight ailments the individual learns how to protect himself 
from attacks, and for this cause seldom consults a physician. 

Occasionally, however, the causes enumerated may produce chronic head- 
ache, which varies according to the nature of the individual attacked. We 
shall first consider minutely some of the common and important forms of 
headache. 

HEADACHE OF CHILDKEN 

Children are often brought to us complaining of persistent presure pains 
in the forehead, behind the eyes, and in the parietal region. When we in- 
quire into their mode of life we frequently get reports of badly ventilated 
sleeping rooms and schoolrooms, that the child has a seat near the stove, 
that he is daily awakened at an early morning hour, or ' that he goes to 
school with an empty stomach or after a hasty or very light breakfast. The 
little baker boys who are obliged to deliver bread very early in the morning 
suffer from severe headache. Once I saw this form of headache pass directly 
into an epileptic attack, and observed the disappearance of both symptoms 
when the child was sent to another school to which it could go without taking 
an early morning train, as with the first one. 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 857 

Sometimes we find the cause of headache in childhood to be a too constant 
use of the eyes. 

On entering school, and as they progress in their studies, more continuous 
use of the eyes is required, and this occasionally produces pain in the frontal 
region and in the back of the head which may be directly attributed to exer- 
tions at accommodation. Xot always, but in many cases, an error in refrac- 
tion, occasionally also a weakness of the muscles of the eyes, especially of 
the internal recti, may be found. The pain not only appears when the eyes 
are exerted, but once present is persistent, increasing during the day and 
disappearing at night. During the holidays this almost invariably ceases. 
These little sufferers cannot always be helped by the oculist, for what they 
require is a lessened use of the eyes; but the physician may help them greatly 
by prescribing a roborant diet, by ordering walks, and by the use of the 
lukewarm frontal douche which I previously advised for another purpose. 
Their posture in reading and writing should be observed, and if this be faulty 
the parents should be urged to correct it. In the most severe cases the chil- 
dren are generally nervous, sometimes they vomit, are restless during sleep, 
and occasionally impress us as suffering from grave disease. I have observed 
some cases in which meningitis or chronic hydrocephalus was suspected. Care- 
ful examination, particularly a record of the temperature and, in doubtful 
cases, the result of rest in bed for several days, always led to a correct diag- 
nosis. Especially in little children who cannot accurately describe what they 
feel, it is very necessary to determine the type of the headache. If we ascer- 
tain from our investigations that there are acute attacks with free intervals, 
migraine should always be suspected, and this suspicion will be confirmed 
if we find a similar affection in one of the parents or in a brother or sister. 
Migraine is a distressing affection because it may persist throughout life, 
and it calls for the greatest care in treatment. The regulation of the diet, the 
absolute prohibition of alcohol, the strengthening of the body, and the conscien- 
tious treatment of the individual attack are primarily important. It is not 
unlikely that we can do something to abort the attacks if we begin the treat- 
ment in earliest youth. I shall later discuss these conditions more in detail. 

When a child complains of headache we must never neglect to inquire 
about its sleep. We will then learn not only of serious disturbances, of noc- 
turnal crying, of restlessness and even of night terrors, but therapeutically, 
by measures calculated to deepen sleep much good may result. The bed- 
room and the bed must be considered, and only after practicable changes 
have led to no good result should other remedies be tried. A warm bath 
before going to bed will often suffice, but no stimulating measures should be 
combined with this as is so frequently the case; no ablution should be per- 
formed, nor should they be washed or rubbed off. They should remain per- 
fectly quiet in the bath for from ten to fifteen minutes. Sometimes drugs are 
necessary. In this case I usually give a tablespoonf ul of the following mixture : 

5 Kalii bromati 10.0 

Choral hydrat 2.5 

Antipyrin 2.5 

Syrupus cortic, aur 20.0 

Aqua ad 150.0 



858 HEADACHE AND MIGRAINE 

This dose is calculated for a child aged about ten; younger children should 
receive only one-half to three-fourths of a tablespoonful. This dose is never 
to be repeated the same night, but should be given only upon successive 
evenings. 

If in the adult persistent and severe headache which does not conform to 
any of the well known types leads us to suspect a constitutional or an intra- 
cranial affection, it is even more important to bear this in mind in the case 
of children. The examination of the urine is especially important as well 
as that of the eye-ground. Nephritis after infectious diseases, which may 
develop very insidiously, may first be considered, then chronic hydrocephalus 
and tubercular meningitis. 

THE HEADACHE OF ADOLESCENTS 

Headaches due to pathologic conditions of vision occur up to the age of 
puberty, and they cannot be sharply differentiated from what is ordinarily 
called cephalsea adolescentium. Boys and girls up to eighteen years of age 
often suffer from excruciating and almost continuous headache. The pain 
is felt in all parts of the head, but is especially severe in the forehead and 
behind the eyes. It usually lessens during the night and increases during 
the day. Actual paroxysms do not occur, but the persistency is characteristic. 
Those who are attending school are tired and morose, they try to study but 
are constantly seeking the physician to get relief from their sufferings which 
are a decided drawback to the best years of their lives. As far as my experi- 
ence enables me to judge, students of the advanced grades of school are more 
often attacked than those in the lower grades. 

In later life these headaches produced by strain on the part of the eyes 
are rare, but some occupations which require great power of accommodation 
may lead to this condition. For instance, I saw very severe symptoms in a 
prominent wood carver who did very fine work such as the carving of small 
parts of machinery and the like; I saw the same in embroiderers, and once 
very tenaciously in a diamond merchant. Diamonds are assorted according 
to their value, and in deciding this the size, the cut, the luster, and many 
other conditions come into consideration which, even with excellent judg- 
ment and long practice, make constant demands on the power of accommo- 
dation. In the last case, after every possible measure had been fruitless, 
complete rest of the eyes and the strengthening of the constitution by a sea 
trip produced such marked amelioration that it was practically almost a cure. 
To-day the patient is again able to follow his very trying occupation. 

These affections are not incurable, but, unfortunately, the most effective 
remedy, the removal of the cause, is not always possible. 

Residence in the open air, if possible in a mountainous region, and bodily 
exercise which requires but little strain upon the sight, are very efficacious. 
But, of course, we cannot expect that a treatment of this kind for four weeks 
will produce any noteworthy results. To attain this several months are nec- 
essary, and these, unfortunately, the patient cannot always give. Many such 
a martyr to school and examinations must rely upon other remedies until 
the goal of school life has been attained, when, before he settles down to an 
occupation, a prolonged residence in the country or, even better, a winter in 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 859 

a high altitude or a sea voyage inay be possible. We should insist, however, 
that if during the school life or the apprenticeship such a long vacation can- 
not be taken it must be as soon afterward as is possible. The patient is then 
saved much suffering in later life. To ameliorate the difficulty exemption 
from study or, whenever possible, sports, and lukewarm baths with cold 
affusions should be advised. Cold ablutions which are so often used, and the 
douche which is also often advised, I have frequently known to do more harm 
than good. The lukewarm frontal douche is sometimes excellent for these 
cases. It should, however, fall from no greater height than three feet. The 
effect often lasts for hours, and this is worth considering. Before resorting 
to antineuralgics I must say a word of warning. They relieve only for a 
very short time, and must be given too frequently. 

Sometimes the use of the constant current has proven very effective; how- 
ever, not for any length of time. A broad electrode is placed upon the nape 
of the neck and a similar one upon the forehead (the latter being the posi- 
tive electrode) and the current is gradually increased to one milliampere, 
which is applied for a few minutes. The question of suggestion, lately so 
frequently discussed whenever electrotherapeutic successes are mentioned, I 
do not care to enter upon; I will merely state that in my severest cases no 
other method gave me such good results. 

Ccplialcua adolescentium is often coincident with severe pressure in the 
head, from which occasionally it cannot be differentiated. Unfortunately 
attacks of true migraine sometimes occur in the same persons. This circum- 
stance alone constrains the physician to do all in his power to relieve the 
condition before it assumes a chronic form which may persist throughout life. 

The factors which produce headache are especially intensified when they 
affect anemic or chlorotic persons. 



HEADACHE OF THE ANEMIC 

In this form of headache the intensity of the pain corresponds with 
the debilitated condition of the patient, and the intervals between the attacks 
of pain are very brief. The most serious forms I have seen were in girls who 
had made great efforts to pass a teacher's examination, and soon afterward 
began their arduous work, which, if conscientiously performed, gives them 
little time for recreation. This severe form also occurs in poor, under-nour- 
ished women who by anxiety and frequent pregnancies, by overworking for 
their children, by insufficient food and unhygienic mode of life, have reached 
an unfortunate state of general debility. The majority of these patients, 
before being brought to me, had gradually fallen into the habit of taking 
enormous quantities of the greatly abused analgesics, with only temporary 
effect, but usually enough to relieve the pain so that they might do their daily 
work. 

In these cases only rest and forced feeding are of any avail. Since this 
treatment cannot well be carried out at home, especially when there are small 
children in the household, the patient should go to a hospital or similar in- 
stitution. The mere rest from their ordinary duties, which they have until 
then performed, often has a favorable effect. The patients should be put to 



860 HEADACHE AND MIGRAINE 

bed. After a week of absolute rest, when they have regained the habit of 
sleep and the appetite begins to improve, a partial rest cure may be instituted 
— the patient being kept in the room alone several hours during the day with 
a window open. During this time they should be placed upon a strengthening 
diet, and preparations of iron, etc., should be given. I believe that the min- 
eral waters containing arsenic and iron — Levico or Roncegno with which I 
have had long experience — are usually very beneficial. A tablespoonful in 
half a wine glass of water should be given after each meal. This should be 
stopped during the menstrual period or if digestive disturbances appear. 
From the second to the third week the rest treatment may be relaxed. The 
patient may have cool ablutions early in the morning — in the first week this 
is still too severe — and then may be allowed to walk for a gradually increas- 
ing time or to be in the open air. All hand work and reading are to be 
strictly prohibited. The diet should be plentiful, and should contain easily 
digested fats and carbohydrates. 

It is often difficult to adhere to this plan of treatment, but I strenuously 
advise its continuance, for it is the only one which will bring about permanent 
recovery. A humane physician who realizes the exigencies of life, and who 
knows the importance of such recuperative measures, can often by obtaining 
the assistance and cooperation of his well-to-do patients make it possible for 
the poor ones who are struggling for the means of existence to carry out this 
plan of treatment. Mere advice, of course, will never suffice. 

The headache of the anemic has caused much theoretic consideration. 
Here it seemed possible to recognize the cause of the pain, and two explana- 
tions have been offered, one of which was a poisoning of the nerves by toxins 
which circulate in the patient in consequence of abnormal metabolism. 
From this absolutely hypothetical conception, from which even to-day there 
is no shadow of proof, the idea has arisen that there are actually poisons 
which produce headache, and that, for many persons, merely their presence 
in badly ventilated rooms or wherever a large number of persons have con- 
gregated is enough to produce an attack. The opinion is constantly advanced 
that anomalies of metabolism play a great role in the development of head- 
ache; for instance, that in persons of sedentary habits — however, quite rarely 
— headache is due to deficient excretion or to insufficient combustion, particu- 
larly of nitrogenous foods. So far as I am able to judge, this view needs 
further confirmation. True, experience shows that in persons who eat too 
much and who take insufficient exercise habitual headache is occasionally 
relieved by the correction of these habits. The hypothesis has also been 
suggested that anemia of the nerves will produce headache. This I believe 
to be possible, but another factor must be added, since, in the majority 
of anemics, the headaches are not persistent. We shall revert to this dis- 
cussion later. 

That abnormal distention of the blood-vessels of the cranium may pro- 
duce headache is quite certain, for there is a form of headache which depends 
upon vasomotor paralysis in the region of the head. When I inhale nitro- 
benzol, and even if I merely pass a chemical laboratory where this agent is 
manufactured, my face begins to redden, my carotids to pulsate, and I feel 
a temporary but excessively severe sense of pressure within my head. The 
same effect may be produced by, and be persistent for a long time after, the 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 861 

inhalation of a single drop of amyl nitrite. Personally I am so sensitive to 
these agents that, by a sudden headache, my attention is often attracted to 
" well corked " bottles which contain amyl nitrite. 

VASOPARALYTIC HEADACHE 

Vasomotor headache which may be artificially produced is a constitutional 
affection in some persons, and is among the severest forms of headache of 
which we have any knowledge. The faces of the patients are very red, and 
here and there are suffused by waves of deeper redness, the lids appear some- 
what swollen, the expression denotes suffering and exhaustion. They tell us 
that headache is really constant, with the exception perhaps of a few weeks 
in the year when they are in the mountains and free from all care and excite- 
ment. Pains are felt in all parts of the head, but are especially severe on the 
top and in the temples, where it seems as though the head would burst. There 
is a painful sense of pressure behind the eyes, which seem to bulge forward. 
The intensity varies, the patients rarely admit that there are days absolutely 
free from headache, but all of these unfortunates report that at times, per- 
haps before the menses or when the atmosphere is heavy, the pains become 
intolerable. The sufferers then, in spite of all their efforts, are obliged to 
suspend their work and go to bed where they lie moaning. Night does not 
always bring relief, and often in addition there are a number of other symp- 
toms which might be designated as neurasthenic. Great debility, disinclina- 
tion for any kind of work, anorexia, distressing dreams with a fright in 
awakening; occasionally actual hallucinations are present. Often during the 
comparatively easy periods there is intense pain at the top of the head as if 
it were bearing a 100 lb. weight, or as if the top of the skull were being 
crushed. This pain at the top of the head is also experienced as the only 
symptom of headache in otherwise healthy persons, or it may be associated 
with other forms of headache. As it is most severe and most frequent in 
the vasomotor form, it is likely that in other cases it depends upon a distinct 
vasomotor disturbance. Occasionally it is accompanied by edema of the vertex 
of the skull. 

Examination of patients with vasomotor headache reveals that, besides 
the special sensitiveness of the scalp and the vertebrae of the neck, the entire 
skin of the body is covered with red blotches, also striae due to the pressure 
of the clothes, or that such blotches and striae may be produced by gentle strok- 
ing of the skin, and that this persists much longer than in other patients. 
The thought is at once suggested that we are dealing with a weakness in the 
innervation of the blood-vessels. Eulenberg has termed these cases paralyses 
of the sympathetic. They all persist for a long time. Sometimes they occur 
in persons who also show other nervous disturbances, particularly in members 
of families with a hereditary taint. We know, too, that the same symptom- 
complex may be produced at any period of life by trauma of the head; not 
rarely it is a partial phenomenon of traumatic neuroses. The visible, vaso- 
motor weakness of the skin does not in itself produce headache, and the 
process may occasionally attack the skin alone and not implicate the vessels 
of the head, for I know quite a number of persons who present this symptom 
without suffering from headache. Inversely, with a feeble action of amyl 



862 > HEADACHE AND MIGRAINE 

nitrite, vasomotor paralysis occurs only in the head, not in the skin or body, 
and we are familiar with the fact that the marked rushes of blood to the 
head from which women frequently suffer during the menopause are usually 
unaccompanied by pain. We must, therefore, assume that an increased flow 
of blood to the head causes headache only when it affects the interior of the 
cranium. 

As a rule, only the traumatic cases are hopeless. In these vertigo, loss 
of memory, dizziness and psychical depression are added to the headache, and 
gradually — certainly in some cases — a transition to degenerative processes 
of the cerebral vessels takes place. The other cases are probably all curable. 
If nothing is done to relieve them, they gradually disappear in the course of 
years. I have repeatedly been led to believe that the gradual change in the 
mode of life common to every person here plays a role. I have not seen a 
single case after fifty years of age. In the prognosis, naturally, the differen- 
tiation from diseases which run a similar course is important, and, so far as 
my experience goes, only brain tumor comes into question. Perhaps further 
observation will permit a differential diagnosis; but when we see an espe- 
cially severe case it is sometimes impossible to make this immediately. I 
remember seeing once a robust young man who died from brain tumor, and 
who, at the first examination, twenty-four hours before death, complained 
only of severe vasomotor headache without any other symptom. Therefore, 
the examination of the eye-ground should never be neglected. 

Most cases run a favorable course, and may be checked or relieved by 
proper treatment. A good laxative at the onset is always advisable. This 
treatment probably causes the vessels of the abdomen to take up relatively 
larger quantities of blood. Hydrotherapeutic measures, such as cold foot- 
baths, walking in the water, and douches to the legs act in the same way. I 
have rarely seen good results from exercise, which has been recommended on 
account of its influence upon the distribution of the blood; in fact, one of 
my worst cases occurred in an enthusiastic bicycle rider. This patient, how- 
ever, always felt better while riding, but most patients are unable to exercise 
on account of their pain. Whenever possible such patients should be sent 
to the mountains, and those regions should be selected which have a cool 
atmosphere without great variations in the temperature ; for example, Rigi, 
Beatenberg, Miirren, Wengen and Obersdorf in Thuringia ; the high altitudes 
in the Southern Schwarzwald also fulfil these indications, although to a less 
extent than the Swiss regions. 

These patients are all peculiarly susceptible to thunderstorms. They in- 
stinctively feel their approach, even long before, and suffer greatly during 
the storm. Eegions in which thunderstorms are numerous are always badly 
borne by them. For this reason valleys, even valleys of high altitude, should 
be avoided. Nor is a residence at the seaside advisable. Whether the lower 
air pressure in both cases plays a role we do not know. Even at home much 
may be done for the patient. Among drugs ergot has been particularly recom- 
mended for obvious reasons. Only once have I seen benefit from its use, 
and perhaps for this reason I do not permit my patients to take it for any 
length of time. On the contrary, I recommend tincture of nux vomica, and 
also advise giving to these patients in addition for a long time a milligram of 
arsenious acid three times daily. The electric current is often found to be 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 863 

beneficial. My results, however, have not been so positive that I can join in 
unqualified praise, of it. Nevertheless, I have seen some good effects from 
increasing currents applied to the side of the neck, so that I think its occa- 
sional use may be advisable. The analgesics always relieve for a few hours, 
but never actually cure the affection. 

B. HEADACHE DUE TO ORGANIC DISEASE 

Up to the present time, the varieties of headache that have been de- 
scribed form a somewhat uncertain foundation so far as the actual etiology 
is concerned, and we gain more accurate knowledge if we consider those 
forms which depend upon organic changes and not merely upon disturbances 
in function. 

(1) The hairy scalp is exceedingly rich in receptive nerves. We may con- 
vince ourselves of this by touching a single hair, which will suffice to produce 
a sensation. Any one who knows the infinite number of fine, nervous plexuses 
surrounding the bulb of a hair, any one who has seen the fine processes like 
the points of a crown forming rings around the hair will easily understand 
this sensibility. The crown surrounds the hair, and no matter which way 
this curves it always touches one or more points of other crowns. Under 
any ordinary circumstances which increase sensibility, and also after severe 
headache of any origin, hyperesthesia of the hairy scalp may be produced 
and may also attack deeper tissues (hair-pain, clavus). The terminal branches 
of the trigeminal nerve and of the upper cervical nerves extend through 
the inner layers of the skin. Various painful disturbances may occur in 
their course, either because the nerves themselves are damaged, or because 
disease and irritative conditions in other regions of the skull are disseminated 
into these nerves. True neuralgias, as a rule, are easily distinguished from 
diffuse or localized headache, but it is different with the painful sensations 
produced by disorders of the eyes, diseases of the nose, or of the antrum of 
Highmore. Catarrh of the frontal sinuses may be felt as diffuse, severe, 
frontal pressure, and many diseases of the eyes, from insufficiency of the 
external muscles to glaucoma, usually first betray themselves by headache. 

(2) Below the skin lies the galea. This, and probably the periosteum 
which is directly adjacent, may be the seat of extraordinarily severe head- 
ache which is designated as indurative headache. 

INDURATIVE HEADACHE 

This, probably the most frequent form of headache, seems to be almost 
unknown to the majority of physicians although it has been described in 
text-books for decades, and although we possess some excellent, especially 
therapeutic, investigations concerning it. Among the cases that I have seen, 
which far exceed one hundred, few had been properly diagnosticated and 
treated. Almost invariably the affection had been taken for migraine or an 
allied condition. And, indeed, those who have written monographs upon the 
subject have not attempted to sharply differentiate it from migraine. This 
distinction is, however, very easily made, and the differential diagnosis in- 
fluences the treatment which differs greatly in the two affections. In the 
form under consideration it consists chiefly of massage, hence, as masseurs 



864 HEADACHE AND MIGRAINE 

did not differentiate sharply between the two affections, it was repeatedly 
observed that the method which gave excellent results in one case was of 
absolutely no use in the other, and some physicians who made a study 
of migraine declared the reports of other physicians untrustworthy because 
they maintained that they had cured many cases of this severe affection by 
massage. 

The majority of patients with indurative headache are women. It is 
probable that frequent local chilling in consequence of washing the hair here 
plays a role, for it is certain that refrigeration may produce the disease. 

The onset is sometimes characterized by symptoms which resemble men- 
ingitis. 

Some time ago I was called in consultation to see a robust young man ; he lay 
moaning, Avith all the signs of agonizing pain in the head and the nape of the neck, 
and had been three days and nights without sleep, with repeated attacks of nausea. 
Every movement of the head caused such pain that the patient cried aloud. He lay 
in bed, his neck rigid, and did not dare to move. The experienced family physician 
had thought of meningitis, but had begun to doubt his diagnosis on account of the 
absence of fever. Palpation of the head revealed that all the muscles at their inser- 
tions and the neighboring galea were extremely sensitive, while the forehead and the 
vertex of the skull were only slightly hypersensitive. The painful region was perhaps 
slightly swollen ; at all events, hard, extremely sensitive areas could be detected in 
it. The history revealed that this man, a few days prior to the appearance of what 
was for him a very severe illness, had been overtaken by a hail storm while riding a 
bicycle. During the evening he was uncomfortable, felt chilly, and two days later the 
headache appeared. Here the view that refrigeration had led to a localized muscular 
affection of the head was quite justifiable. A warm bath, hot poultices to the nape of 
the neck, and repeated large doses of sodium salicylate brought about such rapid 
amelioration that upon the next night the patient slept, and after a few days he 
presented himself during my office hours completely cured. 

This was a sudden and unusually severe case of the disease. In most 
instances the acute onset is overlooked. The patient consults the physician 
because he is tormented persistently by very severe headache. Of course 
there are free intervals, but these are usually in the summer; these patients 
suffer more or less during most days of the year. Sometimes the headache 
is felt merely as pressure which occasionally increases, but attacks of very 
severe headache are not rare, they recur at long or short intervals, and closely 
resemble attacks of migraine. They differ from this affection in that there 
is no sensitive aura, in the great rarity of nausea and in the absence of vomit- 
ing, as well as in the circumstance that most cases arise in later life, while 
hereditary migraine almost invariably comes on in early youth. But there 
are exceptions. Of course we cannot say that persons who suffer from mi- 
graine may not subsequently develop an indurative affection of the scalp. I 
have seen two such cases. The most important differential factor is the 
objective condition. In migraine painful areas of the head are rarely found 
except during the attack, but here the opposite is the case. One who under- 
stands palpation of the head and the muscles of the nape of the neck, and 
especially one who can palpate them without making too firm pressure, will 
find in all of these cases that the points upon the skull where the trapezius, 
the splenii, sometimes also the sternocleidomastoid and scaleni are in- 
serted are sensitive, uneven, and nodular. This unevenness often continues 
far into the galea. It is hard, as though something were deposited in the 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 



865 



muscular substance, or as if an induration were present. Sometimes there 
are spindle-shaped sensitive nodules in the points of insertion or in the 
belly of the previously mentioned muscles, and the periosteum of the upper 
vertebrae, especially of the transverse processes, is invariably sensitive to 
pressure. 

Proceeding to palpate from the head downward we will find other areas 
of the trapezius very painful, and will invariably hear from the patient that 
when the pain is most severe 
it radiates to the shoulder-blade 
and the region of the deltoid, 
which is another symptom that 
does not occur in migraine. 
Whether these conditions are 
directly due to muscle indura- 
tions — these hard points are 
usually regarded as such — or 
whether, as I suspect, there are 
only radiating sensations from 
the suprascapular nerves pass- 
ing through the deep muscles 
of the neck, is uncertain. 
Neither do we know whether 
true swelling of the lymph- 
glands and, above all, swelling 
of the sympathetic of the neck, 
occurs in this affection as has 
been maintained by some au- 
thors. I have never been able 
definitely to demonstrate this. 
As a rule the pains are re- 
stricted to the region of the 
affected muscles and to the 
fascia, but not infrequently 
there is very acute pain in the 
forehead during the attack, al- 
though nodules are but rarely noted in the frontalis. Possibly we may here 
be dealing with irradiation of sensation, radiating pains. 

The examination of the insertions of the muscles should never he neg- 
lected in any case of hcadaclic. 

Since we know that thermic influences play an important role in the 
etiology of the disease we can almost always detect the causative factors. 




Fig. 194.- 



-The Points Upon which " Indurations 
are most Frequently Found. 



Thus, an elderly lady who had been suffering for years from severe headache became 
in the course of time completely debilitated, for no treatment gave her any relief. 
She was compelled to give up her flourishing business, and to be content with more 
moderate means. Upon examining the head and neck, the previously mentioned indu- 
rations were found, and the history showed that she had been suffering in the winter 
for years from innumerable " attacks of cold " caused by the opening of the door which 
led into her store. Here suitable therapeutic measures brought only amelioration ; a 
cure was impossible. 



866 HEADACHE AND MIGRAINE 

If the physician does not bear in mind this frequent form of headache, 
he may fall into grave error. Some years ago a lady who usually lived in 
a sub-tropical region consulted me for an unbearable " neuralgia " of the 
head which distressed her day and night. She had long been treated for 
malaria but without result. Examination revealed at once that the patient 
had all over her head and the upper part of her body sensitive, subcutaneous, 
and intramuscular nodules. Here also the cause was easy to recognize. The 
patient, who was born in Germany, had exposed herself to a strong draught 
while wearing very thin clothes, as she believed that only in this way could 
she endure the humid atmosphere. 

This last case, as many previous ones, I treated carefully in various ways, 
also with the electric current, and brought about decided improvement, but 
I was unable to cure any one until I learned that in the more chronic cases 
only one remedy was effective, namely, massage. If I had earlier known 
the publications of the Swedes Henschen, Helleda and Norstrom, I would 
the sooner have been able to help my patients. These investigations are still 
not sufficiently appreciated in medical literature. 

There is no doubt that the most frequent and most distressing forms of 
headache may be etiologically understood by a careful investigation, and with 
this knowledge a rational method of treatment may at once be conjoined. 

The treatment of indurative headache is quite simple. It requires pa- 
tience on the part of the physician and of the sufferer, but it is effective. 
In the acute cases, before going to bed we give at intervals of an hour three 
doses of one gram of sodium salicylate, or one-half a gram of salophen may 
be given four or five times. The disagreeable secondary effects as well as 
the sweating then occur during the night. Besides this treatment, I make 
the patients wear nightcaps in the winter. It is also well for the patient to 
apply for several hours at night hot cataplasms, peat compresses or ther- 
mophores, etc., to the back of the neck. No time should be wasted by using 
analgesics to ameliorate the pain. In such cases these drugs are often abused. 
None of them has as permanent an effect as the preparations of salicylic 
acid. After a few days I employ massage. At first the muscular insertions 
are stroked toward the body with the thumb ; this is quite tiresome, and the 
first attempts are also painful to the patient. Especially hard areas must 
be massaged, and then should follow firmer stroking with the hand, not only 
of the induration upon the head, but also of all the muscles of the neck, 
in particular the trapezius and the splenii. The nerves should at first be 
only gently stroked. Later they will bear firmer pressure, and even a vibra- 
tory pressure with the tips of the fingers. Each manipulation should con- 
sume from ten to fifteen minutes, including the brief pauses which are 
necessary. Frequently a surprisingly rapid recovery is brought about; in 
more protracted cases, however, "the treatment must be longer continued and 
the most experienced masseurs estimate from six to eight weeks as necessary. 
As the galvanic current is unquestionably beneficial, it may be combined with 
massage. I have never seen any good results from spa treatment. As, how- 
ever, it is often necessary to send the patients who have been debilitated by 
pain or by the treatment away from home, regions that are protected from the 
wind should be chosen. I have repeatedly known a sojourn at the seashore 
to do actual harm. 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 867 

Instead of massage, I have used vibration with suitable apparatus and se- 
cured good results. I have been satisfied with this treatment, but it must 
be cautiously used in persons with arteriosclerosis, and the vibrating manipu- 
lation should not be attempted upon their heads. Besides the treatment 
which I have proposed, in older cases I have frequently seen good results 
from potassium iodid. 

The work of the physician is not completed when he has relieved a head- 
ache of this kind. He must acquaint the patient with the fact that the pain 
is caused by getting chilled and must insist that the mode of life be changed 
so as to avoid the effects of cold. Many a headache in a bald head has been 
permanently cured by wearing a wig. After the conclusion of this treat- 
ment I always give the patient a few doses of sodium salicylate, about a 
gram, and tell him that if the pain returns he is to take immediately three 
or four powders, is to go to bed, to apply hot compresses to the nape of the 
neck, and to cover himself warmly while in bed. By these measures we fre- 
quently succeed in aborting a fresh attack. 

Some of these patients have severe pains in other parts of the body which 
they believe to be neuralgia, etc., yet a careful examination shows them to 
be "rheumatic nodules," similar to those found upon the posterior part of 
the head and neck. These patients are regarded as hysterical, hypersensitive 
persons who are constantly complaining of pain. The skin should always be 
palpated when any one complains of vague pains now in one, now in another 
part of the body. 

(3) Headache due to disease of the galea and the insertion of the mus- 
cles is most common, but is rarely due to disease of the bones of the skull. 
This has always an extremely local character, so that confusion with the 
diffuse forms of headache will seldom occur. Palpation and, if necessary, 
careful percussion will rapidly facilitate our recognition of thickened or sof- 
tened areas. Periostitis traumatica and syphilis produce these conditions. 
Such organic affections need not immediately be thought of if a person 
complains of pressure pain or even of pains arising spontaneously; for in- 
stance upon the vertex of the skull. The vertex of the skull, particularly 
in the hysteric, is frequently the seat of severe pain which may even be in- 
creased by pressure. The patients sometimes liken the sensation to that of 
a nail being driven through the head, and the affection has therefore been 
called clavus hystericus. In rare cases there is also pain which is localized in 
the sutures of the skull. It is steady and is increased by pressure. One of 
my patients, a robust, military man, has suffered so greatly for years from 
this insignificant affection that he has repeatedly requested surgeons to 
trephine his skull, and once had the periosteum loosened from the vertex of 
the skull without any benefit. So far as I know, improvement can be brought 
about only by general strengthening measures and by the employment of cold 
locally, also of ice, ether spray, chlorid of ethyl, etc. No sufficiently pro- 
longed trial of the galvanic current has as yet been made. 

(4) The cerebral dura mater is so rich in nerves that disease of this 
structure, or merely its subjection to pressure, will often cause very acute 
pain. Intracranial periostitis chronica and pachymeningitis interna are 
relatively the most frequent forms. We think of the latter with its relaps- 
ing hemorrhages when severe headache occurs in old persons, sometimes 



868 .'■■ HEADACHE AND MIGRAINE 

introduced with vomiting, sometimes occurring in paroxysms with free 
intervals, occasionally combined with a disturbance of consciousness or 
paralysis of some cranial nerves. Disorders of speech may also transi- 
torily appear. That analogous processes, occur under some circumstances 
is well known; perhaps it is here worth our while to especially consider 
influenza, in which pachymeningitis is occasionally added to other intracranial 
affections. 

At the autopsy of persons who have suffered for years from agonizing 
headache, we not infrequently find upon the vertex of the skull or laterally 
to this area firm localized adhesions of the dura to the top of the skull; these 
have perhaps been caused by trauma, or may occasionally be attributed to 
syphilis. The abnormal venous circulation caused by the formation of new 
Pacchionian granulations within limited dural areas, particularly in old per- 
sons, appears to cause headache. 

Of course, these are the more frequent occurrences. If in cases of very 
severe headache pain is felt within the head, it is always well to consider the 
possibility of the dural nerves being affected; since, except for the branches 
of the fifth nerve at the base of the brain inside of the skull, sensory nerves 
are probably contained only within the dura mater. 

There is a true hyperesthesia of the dural nerves. In these cases, there 
is no, or perhaps only very slight, headache during bodily rest. As soon as 
the patient begins to walk, to ascend a staircase, to jump, or to perform any 
other bodily exercise, he has a distinct sensation of pain within the skull. 
He feels that " his brain reels," is distinctly aware of palpation over the sen- 
sitive dura, that is, he feels the variations in pressure of the liquor cerebro- 
spinalis. At other times there is actually continuous pain, and we hear that 
cough, pressure, etc., always produce aggravations. We often hear him say, 
" It appears to me that with each attack of cough my head will burst." 
This hyperesthesia is noted under varying circumstances; sometimes in very 
debilitated persons, particularly during convalescence from severe disease. 
Rest combined with good nutrition, etc., has an excellent curative effect. 
The same pains are experienced by persons who suffer from chronic constipa- 
tion. These patients with headache are cured by proper diet and by treatment 
at Marienbad, Kissingen, etc. 

The pia mater probably contains only the nerves intended for the vessels 
but as it is closely adjacent to the dura, and since nearly all inflammations 
run their course with exudates and increase the intracranial pressure, it is 
evident that all forms of leptomeningitis may directly produce headache by 
pressure upon the dura which is so richly supplied with nerves. 

(5) The brain. It is probably only by the transmission of pressure that 
the headache occurs which accompanies all diseases of the brain which limit 
the extent of this organ. For the brain itself, at least upon its surface, is 
insensitive. I saw a little boy who had been trephined cry loudly when his 
bandage was changed, but I noted with astonishment that he was perfectly 
quiet and engaged with toys which had been given him while a knife was 
several times plunged through the normal cortex to discharge an abscess. 
Certainly there are portions of the brain the irritation of which produces 
disturbances of sensation and pain which are then conveyed to the periphery, 
such as the opposite arm or opposite leg. The area from which painful sen- 



CAUSES, COURSE, AND TREATMENT OF HEADACHE 869 

sations are transmitted to the top of the skull may be theoretically assumed 
but has not yet been found. 

An organic intracerebral disease or a severe uremic cerebral irritation 
must always be thought of when attacks run their course with fever, when 
the pupils are very widely distended or unequal, or when headache is almost 
continuous, and vomiting constantly recurs. Accurate investigation of all 
the aspects of the case then naturally becomes our duty. 

In brain tumor headache may for a time cause the greatest perplexity in 
diagnosis. It is extremely severe, occasionally persists for a long time as the 
only symptom, and is usually confined either to the forehead or to the occipital 
region. Often other severe symptoms appear, such as rigidity of the pupils, 
disturbances in gait, and vomiting, and these leave little doubt as to the origin 
of the severe pain. Nevertheless, I have seen a few cases of excruciating 
headache which continued for many months before it was possible to diag- 
nosticate tumor. I remember a patient who, after years of suffering, suc- 
cumbed to a tumor in the region of the island of Reil, and who only a short 
time before death was seized with severe attacks of headache between which 
there were intervals absolutely free from pain. 

In cases of persistent headache it is always wise to examine the eye- 
ground, and several examinations of the urine should be made. Contracted 
kidney, especially, is often accompanied by headache which we may fail to 
recognize as a partial symptom of this affection because the amount of 
albumin in the urine is sometimes almost infinitesimal. In addition to pos- 
sible albuminuric changes, under examination with the ophthalmoscope we 
should particularly note the condition of the papilla (neuroretinitis, stasis 
papillae). 

The treatment of these severe intracranial headaches is often exceedingly 
difficult. The best results are obtained by continuous treatment with cold, 
by means of an ice-bag and the like, by rest in bed, and in some cases, par- 
ticularly when changes have been produced in the dura, by the administration 
of potassium iodid and purgatives. Of course, wherever possible, we should 
treat the underlying affection itself. Symptomatically, the various antineu- 
ralgics which are to be described under the treatment of migraine are for 
a time of use. 

SYPHILITIC HEADACHE 

Headache in the various parts of the capsule of the skull or of the brain 
may be caused by syphilis. Upon the whole, however, relatively few of the 
numerous cases of headache are due to this cause. Nevertheless, if the physi- 
cian is consulted by one who was previously free from severe headache, and 
who without a known cause is attacked by severe, localized pains here and 
there at the top of the skull, pains which are particularly worse at night 
or after excitement and rarely cease, it is well to remember that in the ter- 
tiary stage as well as in the initial stages of general syphilis this symptom- 
complex is frequent. Syphilitic headache is extremely severe. The excessive 
pain, which scarcely ever remits but occasionally even increases, rapidly de- 
bilitates the patient and renders him incapable of exertion. We sometimes 
find these unfortunates crying, kneeling in bed, and even fearing to touch 
the pillow. Sometimes a definite part of the cranium is particularly sensitive 



870 HEADACHE AND MIGRAINE 

to pressure, sometimes even one-half of the head. Autopsies and the careful 
analysis of their findings have demonstrated that all of the parts surround- 
ing the brain may be attacked by syphilis. We recognize a leptomeningitis 
syphilitica which especially implicates the pia mater, and a diffuse and miliary 
form of pachymeningitis gummosa which lead to many adhesions between the 
dura, pia, and top of the skull. We also know that there is a syphilitic 
osteitis which being specially developed between the dura and the top of the 
skull produces in the course of time many osseous deposits upon the latter 
structure. Not infrequently gummata form in the bones of the skull and in- 
crease its size. Many of these rapidly disintegrate, and thus produce large 
openings in the top of the skull. The physician who can make a correct 
diagnosis in this case, which is not at all difficult, performs a beneficent act. 
In a differentio-diagnostic respect, only lead poisoning and the various forms 
of infectious leptomeningitis can be confounded with it. The former very 
rarely leads to severe headache, and may be differentiated by the history 
(colic, the occupation, etc.) as well as by the usual lead line on the gums. 
Meningitis almost always attacks the pia of the cervical cord, and causes 
rigidity of the neck. On account of the usually increased pressure it is 
often accompanied by vomiting and slowing of the pulse, which is not apt 
to be the case in cerebral syphilis. The temperature record will often suffice to 
differentiate meningitis from the more chronic syphilitic affection. If we are 
certain that infection has preceded, and other causes of headache are excluded, 
we should not temporize with symptomatic remedies in these severe cases, but 
give upon the first night a hypodermic injection of morphin in order to secure 
rest for the patient, and then as soon as possible institute antisyphilitic treat- 
ment. These are the cases in which we should not delay; more than one life 
has been lost by too long waiting or by a too conservative treatment. Iodin 
as well as mercury should be given at once in large doses. 

This review of the causes of headache and its different varieties may 
awaken the thought that it is difficult to decide in the individual case with 
which variety we are dealing. This is not quite true. For, if we exclude 
the usually recognizable intracranial affections and the exceedingly common 
form of headache due to induration, only the headaches of children and ado- 
lescents remain as relatively frequent forms. In my opinion at least two- 
fifths of patients with headaches suffer from the indurative form, one-fifth 
from other forms. The remaining two-fifths are afflicted with migraine, an 
affection now to be described. 

MIGRAINE 

Primarily we must regard it as certain that headache of the migraine 
type occurs in several forms of disease which are perhaps related. It may 
appear in epilepsy, at the onset and during the course of relapsing paralysis 
of the oculomotor nerve, in tobacco poisoning, perhaps also in severe forms 
of hysteria. Most frequently, however, these pains assume the form of a 
definite, circumscribed disease, typical migraine. 

The patients who suffer from migraine report that they do. not have 
headache constantly; that, on the contrary, there are sometimes intervals of 
months, often shorter periods of relief, then quite suddenly, usually early in 



CAUSES, COURSE, AND TREATMENT OF MIGRAINE 871 

the morning, an attack of headache sets in with general prostration and 
malaise which rapidly increases to such an extraordinary degree that they 
are incapable of attending to their work; this attack usually lasts the whole 
day, but becomes milder toward evening, often after preceding vomiting. 
When the paroxysm is over there is usually complete euphoria. Occasionally 
the patient who, even late in the afternoon, was suffering severely, is met in 
the evening in company or at the theatre. 

During an attack of migraine many interesting details come to light. 
First, we almost invariably learn that the disease is well known in the family 
of the patient. We find that one or another member of the immediate family 
has similar attacks, also that the parents, sometimes even the grandparents, 
have suffered from it. Where the type is unknown among the other members, 
we often ascertain that other neuroses have appeared in the family, especially 
epilepsy, congenital degenerations, and neuralgias. Of course, there are also 
cases in which such investigations reveal nothing. 

The affection frequently appears in extreme youth; usually, however, it 
develops only after the responsibilities of life have been assumed. We can 
scarcely doubt that predisposition, mental, and even bodily exertion may 
produce the disease. It is also known that it may be caused by prolonged, 
debilitating diseases and privation. More than one of these patients accurately 
dates the onset of the affection from the time of his convalescence from some 
infectious disease. They all know what circumstances produce the individual 
attack, but they cannot determine the general cause. There are times when 
the slightest cause or change of condition will produce headache; for in- 
stance, just before the menses, a long railroad journey, being present for 
some time in a close, badly ventilated room, or during great heat ; in some 
cases migraine follows slight constipation; and anger, excitement, the use 
of the smallest quantity of alcohol or tobacco, an unusually early awakening 
in the morning, and the omission of a meal will produce a severe attack of 
migraine. 

Whether true migraine ever arises from disease of the nasal cavity and 
may be relieved by treatment for this is not yet certain; the same is true of 
the frequently maintained relation of migraine to disorders of accommoda- 
tion and of the ocular muscles. But there are some patients in whom an 
attack is produced by a strong light such as they must encounter at concerts 
and in theatres, and in others an attack follows any work requiring close 
sight. 

It is obvious, therefore, that in migraine we may combat the predisposition 
as well as the occasional causes of the individual attack. Many children, 
headache in whom has been previously described, certainly have a predisposi- 
tion to migraine. Strengthening of the body, the avoidance of over-exertion, 
careful ventilation of their rooms, particularly their bedrooms, is very im- 
portant. It is self-evident that children should not be allowed alcohol in 
any form, but I particularly desire to mention it here. Persons with a ten- 
dency to migraine should consider this when choosing an occupation. I have 
seen many a one prevented by this disease from carrying on his work who 
might have prospered in some other calling. Migraine is most frequently 
found among professional people, especially among the brain workers, and 
we possess excellent descriptions by prominent authorities who have themselves 



872 HEADACHE AND MIGRAINE 

suffered from the disease. Most patients soon learn what will produce an 
attack; nevertheless, there are a few rules of general application. Among 
the most important is this, that persons who frequently surfer from migraine 
should never exert themselves on an empty stomach. I advise, just as in 
pressure headache, that food be partaken of every two hours even if only a 
little zwieback or a swallow of milk. The interval between the evening and 
morning meals should also be lessened by taking food before going to bed. 
If the patients upon awakening, or even during the night, feel that an attack 
is threatening, without raising their heads and while still in the recumbent 
posture they should take some food, milk, for example. I believe that an 
attack may frequently be prevented or aborted by this method. 

In some persons an attack of migraine announces itself the evening before 
its appearance by lassitude, irritability, an incapacity for arduous work. In 
most patients there are distinct prodromes early in the morning, so distinct 
that the patients almost invariably know what will happen during the day. 
They awaken with numbness in the head, and feel extremely tired. They 
have a distinct consciousness that sleep was heavy and deep; others again 
have disquieting dreams, and waken feeling completely worn out. Headache 
appears immediately on raising the head or during dressing, but does not 
reach its acme until a few hours later, although the pain constantly increases 
in severity, and is almost exclusively unilateral. It is felt deep in the head, 
" as if the head were splitting." There is pressure and a sense of burning 
behind the eyes and in the eyes. In the frontal sinuses, even in the antrum 
of Highmore, there is pain. To this is added a feeling of great prostration. 
Energetic persons and those in whom the attack comes on slowly still attempt 
to carry on their daily work, but in many cases are finally compelled to aban- 
don it. Then a hypersensitiveness of all the senses develops; light, sounds, 
odors, etc., are distressing; all attempts at accommodation, reading, and any 
hand work become impossible, and the symptoms frequently increase to such 
an extent that the patient must be left alone in a quiet and darkened room. 
The appetite is lost, many patients suffer from retching, and almost all from 
vomiting, either early or, more frequently, toward the end of the attack. 
Watery discharges suddenly appear. In the majority of cases these severe 
attacks last from eight to twelve hours, therefore for an entire day. Of 
course all are not of the same intensity; some attacks are mild, especially 
if proper therapeutic measures are instituted. But, unfortunately, there are 
also extremely severe cases in which an actual status hemicranicus develops 
in consequence of the attack lasting for several days and nights. Directly 
after its cessation, which may be quite sudden, many patients feel perfectly 
well. Weaker persons, naturally, still feel debilitated by the sufferings which 
they have undergone. 

Examination during an attack always reveals anomalies of the circulation : 
The face is usually pale; in some cases, however, extremely red, and the 
conjunctivae are injected. Many patients complain of cold feet throughout 
the attack and, in fact, are inclined to chilliness during the entire period. 
The pupils are usually somewhat contracted which is an important symptom, 
since, as a rule, under other circumstances of great pain they are dilated. In 
some cases there is a difference in the size of the pupils. The palpebral fis- 
sure is generally narrowed. 



CAUSES, COURSE, AND TREATMENT OF MIGRAINE 873 

I believe that we may occasionally prevent such an attack of migraine, 
and have previously mentioned some of the measures to be employed for this 
purpose. Some drugs appear to be useful, and first among these is potassium 
bromid, probably because it induces sleep and has a generally quieting action. 
After a particularly exhausting day, or on a day when the patient notes any 
of the prodromes of migraine, also before a period during which, according 
to his own observations, he is particularly predisposed to attacks, two or 
three grams are given in solution before going to bed, and this treatment 
should be continued for a few days. A combination with 0.5 chloral hydrate 
appears to me justifiable. If early in the morning there are signs indicative 
of an attack — the patient is usually familiar with these signs — after some 
food has been taken in the recumbent posture one of the analgesics should 
be administered. Their number is exceedingly large, but I have gotten along 
so well with the earlier ones, antipyrin and phenacetin, that I have but little 
experience with the others. Antipyrin seems to have the most certain action 
in the majority of persons, more so than phenacetin. The dose of either is 
from 10 to 15 grains. I have often noted that the best results were not 
secured from these excellent remedies because the patient had not been in- 
formed that he must remain absolutely at rest for a full hour after their 
administration. If it is possible for him to sleep during this time, the attack 
is aborted or is very mild. If this be impossible, if the attack of headache 
has set in, we have remedies with which to relieve it but we cannot abort 
it. Some patients recover the sooner if they resist the attack as long as 
possible, carry on their work, and try to occupy their minds. If we consider 
the subjective aspect of the sensation of pain, it appears perfectly rational 
to divert the attention as long as possible. Absolute rest with self-observa- 
tion is calculated to add to the difficulties. Often, however, the general pros- 
tration is so great as well as the pain that nothing but rest is possible. In 
this state everything has a disturbing effect, even the ingestion of food, 
which must be forced, for voluntarily the patients eat nothing. A number 
of remedies calculated to influence the circulation in the head are useful, but 
we are not yet so sure of their action that we can order now one, now an- 
other remedy. We must still experiment. Heat is acceptable to most pa- 
tients. Even a bandage applied tightly around the head affords some relief; 
possibly the artificial pressure is active here by producing anemia of the skin. 
Rubber bags filled with hot water or thermophores are excellent, others are 
benefited by hot foot baths. Nitrite of amyl was formerly much employed. 
It is, however, of such doubtful utility, and may occasionally increase the 
pains so extraordinarily, that it is no longer used; neither is nitroglycerin 
which has a similar effect. Only caffein and the analgesics, out of the num- 
berless drugs that have been advised for migraine, have maintained their 
position. Some years ago an elderly gentleman consulted me for migraine. 
For many years he had gone for advice to celebrated physicians of America, 
England, Germany and France. His collection of prescriptions formed a 
consensus of the opinions of the medical faculty of the world as to the treat- 
ment of migraine. Almost the entire materia medica was represented, but 
only one remedy continually recurred, and this was caffein and pasta guarance 
containing caffein. There can be no doubt that this remedy, particularly in 
combination with an analgesic, will afford decided relief. I order 



874 HEADACHE AND MIGRAINE 

IJ Antipyrin 0.5 

Pasta guaranae 0.3 

Caffeini citr 0.02 

F. pulv. d. t. dos. Nr. S. : One or two powders at intervals of an 
hour. 

Large doses of potassium bromid during the attack are also advised by 
some authors. I do not sanction their employment for they induce vomiting, 
perhaps on account of the quantity of fluid necessary to dilute them and 
to prevent the potassium bromid from having a disagreeable and corrosive 
effect upon the gastric wall. They also increase somnolence after the cessa- 
tion of the attack; sometimes, but very rarely, caffein produces palpitation of 
the heart. 

A word in regard to morphin. I admit freely that I cannot get along 
without this remedy in the treatment of the severest forms. The attack has 
so extremely depressing an effect upon the psychical condition of the patient, 
the pains are so great, the general disturbance from vomiting and, in fact, 
also from the limited ingestion of food is so extreme that an injection of 
morphin is often quite justifiable. Morphinism need never be feared so much 
as to make us, for this reason, withhold from the patient a remedy of the 
highest value. Indeed, I am aware of no single case that has developed from 
the employment of this remedy in migraine. The pain and nausea are greatly 
lessened by the injection of 0.015-0.02. 

The treatment of the intractable forms will severely tax the skill of the 
physician. Besides the treatment of the attack, it is occasionally necessary 
to remove the patient from his usual surroundings and place him under more 
favorable hygienic conditions. 

I know a lady who suffered so much that she was obliged to leave her husband 
and child, and spend a whole year at the seashore and in the mountains to regain her 
health. In the main, she attained her object. The attacks, which at first occurred 
several times a week, have become very rare and have almost ceased. 

In addition to ordinary and general strengthening remedies, and the 
previously described precautionary measures, food, etc., arsenic and iron may 
be employed with advantage; I know of but one other remedy which will 
lessen the number of attacks, and this is the galvanic current. I have fre- 
quently employed it, and have notes of many cases in which it proved of 
value. 

A very active woman, the superintendent of a large business, had almost daily 
attacks during her busy season. Here I succeeded, not once but repeatedly, in pro- 
longing for several days the periods free from pain. There was no doubt of the success. 
I resorted to galvanization, applying weak currents to the neck. 

As it has been repeatedly asserted that true migraine disappears after the 
cure of nasal disease, I always examine the nose in severe cases, but I must 
admit that I have seen no case permanently cured by operation upon the 
nose. 

A number of migraine patients are not attacked primarily by headache, 
but by derangements of vision. In these patients the condition is usually 



THE THEORY OF HEADACHE 875 

designated as "flittering scotoma" and to this the attack of true migraine 
is added, although it may in rare cases be absent or very slight, for instance, 
there may be merely numbness in the head or frontal pressure. We must, 
therefore, assume the cause of the disordered vision and of the headache to 
be the same, but they do not always produce these symptoms in combination. 
Attacks of headache without disturbances of sight are more common than 
flittering scotoma without headache. The disordered vision is always an early 
symptom, a sort of aura of the attach of migraine. It appears upon the side 
of the head opposite to that which is attacked by headache. 

The subjective ocular symptoms are usually described by the patients, 
especially by those who study their symptoms, as floating dark spots in the 
visual field, the borders being serrated and illuminated. Others see only the 
illuminated edges, or they may complain of dimness of vision. The latter 
may be the precursor of manifold disturbances of sight, concentric limita- 
tion, hemiopia, even actual amaurosis fugax. The affection is at first lim- 
ited to one eye only, but it is quite certain that in most or in all cases the 
other eye also becomes implicated. Such an attack of scotoma may last for 
a few minutes, or its duration may be an hour. 

The same cause which produces migraine also produces other brain symp- 
toms, but more rarely than disturbances of sight. Here it must be emphasized 
that transitory disturbances of sensation in one-half of the body may appear, 
even transitory aphasia. After prolonged attacks of migraine, especially if 
they are frequent, motor disturbances are observed, such as hemiparesis, also 
contractions in the extremities. 

The clinical picture of migraine is of course circumscribed, but the phy- 
sician who investigates the nature of the disease will find these occasional 
additions to the symptom-complex highly interesting. The fact that epilepsy 
may begin with a flittering scotoma, and even with a true attack of migraine, 
is of great significance. There are also attacks of migraine in which the 
visual disturbances, the debility which rapidly ensues, and the numbness in 
the head play so great a role that the malady may be confounded with an 
abortive epileptic attack. 

The relations of epilepsy to migraine are still somewhat vaguely under- 
stood, but from what has been reported we may conclude that these diseases 
are either occasionally due to the same cause or, what is much more likely, 
that the same predisposition of the nervous system produces them. 

It has been previously remarked that migraine is sometimes combined 
with other forms of headache, especially often with the headache of children 
and with indurative headache. I mention this again because of its diagnostic 
importance. 

THE THEORY OF HEADACHE 

Having described the important forms in which headache plays the pre- 
dominant role in the symptom-picture, in some being regarded as the cause, 
and in some having been shown to be the causative factor, the questions arise : 
What symptoms are common to all these forms ? How does headache 

ARISE ? 

All forms of headache become manifest in the region of the trigeminal 



876 HEADACHE AND MIGRAINE 

nerve. Only in isolated cases are the sensory branches of the upper cervical 
nerves affected. These nerves first run to their ganglia (Gasserian ganglion, 
intervertebral ganglion), and thence as "roots" to the central organ. 
Numerous observations have proven that the irritation of a sensory nerve, of 
its ganglion, or of its root produces pain. It is just as certain that pain is 
then felt in the area of distribution of this nerve. As to the involvement of 
the roots, so little has as yet been reported that I shall cite a few cases. A 
celebrated musician, throughout his life, suffered frequently from excruciat- 
ing headache which usually radiated to the back of the head. At the post 
mortem, I found embedded in the roots of the upper cervical nerve an old 
cicatrix which involved particularly the region of the posterior horn. 

The roots penetrate the central apparatus, and from the pons to the 
cervical cord those which supply the trigeminal nerve gradually become more 
scanty and finally enter their terminal nuclei. The terminal nuclei of the cer- 
vical nerves lie a little above their points of entrance. In spite of the fact 
that many diseases of the pons and the upper spinal cord have been studied, 
nothing has as yet been revealed which unquestionably proves that a disturb- 
ance of the intracerebral portion of the root produces pain. A priori, this 
is not impossible, but conditions which produce pain are apparently very rare 
or they never exist. 

The nerves of the central tract pass into the terminal nucleus of the sen- 
sory nerve, which runs to the corpora quadrigemina, and on to the thalamus 
opticus. It is quite conceivable that a disturbance within this tract would 
be communicated to the periphery as pain. But, in spite of the fact that many 
researches have been made, distinct pain in the path of individual nerves 
after a lesion of the central tract has never been observed. It is, however, 
known that when the sensory tracts which unite and are enclosed in the 
caudal-ventral portion of the thalamus are subjected to definite impressions, 
very severe pain is produced throughout the other half of the body. This has 
been proven by autopsies. 

From the region that has been mentioned nerve tracts pass to the thala- 
mus and the cortex of the brain, and the terminal portions in the thalamus 
unite with those of the cortex. Yet, notwithstanding the fact that cerebral 
localization has been minutely studied in the human brain and in animals 
for the last thirty years, no area of the cortex has yet been found in which 
the trigeminal nerve alone is present. Irritation of such a region must, under 
some circumstances, produce pain in the area traversed by this nerve. 

On surveying this path we conclude that isolated irritative symptoms are 
practically communicated only from the peripheral area of distribution of 
the nerve, and thence to the supposed cortical field of the same. In all other 
areas of the central apparatus, the paths intended for other nerves lie so 
close to the small central tracts that any disturbance must certainly involve 
a large area containing many fields of innervation. This may at once be ap- 
plied to the question of headache. As headache is rarely accompanied by pain 
in other parts of the body, the areas between the nerve roots and the cortex 
of the brain may be excluded as the apparatus of transmission. 

We now turn to the discussion of the nerves and the cortex. It has been 
stated that no cortical disease is known which produces pain. If we assume 
such to exist, two other assumptions must follow. First, that there is a closed 



THE THEORY OF HEADACHE 877 

trigeminal field; second, what is certainly most unlikely, that isolated por- 
tions of this field, for instance, only the definite fields intended for the fibers 
of the trigeminus at the top of the skull, are diseased, which form a brace of 
very hypothetical views. Even if the assumption of a cortical headache were 
tenable, it is at present absolutely unjustifiable to assume that its point of 
origin is the cortex. Naturally the perception of pain is quite a different 
matter. This can hardly take place anywhere except in the cortex. 

That very severe headaches may be caused by an affection of the periph- 
eral branches of individual cervical nerves is taught by the previously de- 
scribed base of " rheumatism " in the muscles of the head and neck. What 
about the fifth nerve? In so far as typical headache is concerned we may at 
once disregard a localization in the root or in the Gasserian ganglion. If 
this really occurred, headache would occasionally be associated with pain in 
all other branches of the trigeminal nerve, and this, as we know, is not the 
case. The same objection might be made to the view of a localization in the 
main branches. 

Only the view remains that somewhere in the cutaneous or dural branches 
which supply the attacked area lies the pathologic cause. No doubt rheumatic 
affections or indurations of the head often lead to direct damage of the cutane- 
ous branches of the fifth nerve, for the pain is not only felt in the posterior 
part of the head but also very acutely over the eyes and in the temporal 
region. The dural branches of the trigeminal nerve must, however, be fre- 
quently damaged. Many patients distinctly state that intense pain is felt 
within the head, especially behind the eyes ; in the most severe forms of head- 
ache, in migraine, and with an increase of intracranial pressure, other dural 
nerves, the branches of the pneumogastric and the sympathetic, are impli- 
cated, as is proven by vomiting and various changes in the pupils. This 
damage must, as a rule, simultaneously affect all intradural nerves, for the 
headache is of diffuse character, and this especially distinguishes it from 
neuralgia. We therefore arrive at our second conclusion that the dural 
branches of the fifth nerve form the point of attack in intracranial headache, 
and that all the dural nerves are uniformly attacked, at least all upon one 
side. 

So far we are upon an anatomical and sure foundation. 

Of what nature is the lesion of the dural nerve? 

It is certain that increased pressure within the skull is manifested by 
headache, but pressure alone probably does not cause the disturbance. We not 
infrequently see local pressure upon the dura without the production of pain, 
and we often note that in tumor of the brain the headache increases and de- 
creases without corresponding change in the size of the tumor. There must 
be another factor conjoined with cerebral pressure. Such, for instance, as 
the disturbance produced in the circulation of the blood and the lymph 
within the tissues surrounding the dural nerves. It is known that these dis- 
turbances may cause acute nerve pain. The severe neuralgias in the extrem- 
ities due to the contraction or occlusion of individual vessels and the neural- 
gias caused by an increase of venous blood in the sciatic and other nerves 
are excellent examples of this. If. however, anemia, and probably also venous 
hyperemia of the dura and of the pia may produce headache, many of its 
well known clinical forms are explained. I refer to vasomotor headaches in 



878 HEADACHE AND MIGRAINE 

those persons with abnormal vasomotor irritability, and to the headache 
occurring during menstruation. Headache from the inhalation of amyl 
nitrite, which unquestionably dilates the vessels of the head, should not be 
forgotten. Hyperemia in the area of the dura, therefore, may produce head- 
ache, as may also local anemia due to pressure. 

These considerations bring us, somewhat in advance of the explanation 
of headache, to the discussion of intradural and extradural organic diseases. 
When we consider the forms of headache not produced by severe organic 
changes, we are entirely in the realm of hypothesis. Obviously we must here 
consider also anemic conditions not due to pressure but to vasomotor dis- 
turbance, to constriction or dilatation of the nerve vessels. It is quite con- 
ceivable that headache may be produced renexly either because abnormal 
irritation (as from the eyes, from the nose, or the ears) is conducted to a 
normal apparatus and produces more or less intense vascular spasms, or be- 
cause otherwise normal irritation may produce the same effect in an abnor- 
mally irritable nervous system. The most severe vascular spasms would con- 
sequently be observed in hereditarily debilitated persons, in whom, as is well 
known, migraine is common, its paroxysmal character and its intimate re- 
lation to epilepsy having for some time been explained by this view. In 
migraine the pallor of the face so frequent during the attack, the pale eye- 
ground, and the general vascular spasm which causes chilliness are distinc- 
tive. Moreover, the disturbances of sight, the occasional disturbances of 
speech, and the rare epileptic attacks during the painful period find explana- 
tion in the assumption of vascular spasms which are not limited to the dura. 
Since DuBois-Reymond explicitly reported his own attacks of migraine 
which he explained by this theory, the malady has been much discussed. 
His hypothesis, however, has lately failed to be generally accepted. The 
investigators who have most closely studied the disease in the last few years, 
Liveing, Glowers, Mobius, do not believe the proofs brought forward suffi- 
cient to meet the possible objections. In the circumstance that dilatation of 
the blood-vessels also produces headache I see no contradiction. Any dis- 
turbance of the circulation, no matter of what nature, will temporarily damage 
the peripheral nerve. 

Nor do I concur in the objection that migraine, like all pathologic and 
physiologic processes, must be produced by the parenchyma cells, that the un- 
doubted vascular disturbance is only secondary; for it has been demonstrated 
to be impossible to attribute the symptom-complex, " headache," to any area 
of the brain. But the opponents of the vasomotor theory consider this alone, 
and they even go so far as to locate there absolutely hypothetical irritations 
and processes of discharge. Still less reasonable is the claim that amyl 
nitrite, which paralyzes the vessels, only rarely relieves migraine produced 
by spastic processes. We know that paralysis causes pain. Further researches 
will determine whether the reported cures do not depend upon a medium dose, 
and whether this might not, perhaps more readily, be attained by nitroglycerin. 
The innervation of the head by the sympathetic nerve is so complicated a 
process that we cannot expect headache to be especially prominent in disease 
of its entire tract. But from the circumstance that none of the relatively 
few cases of disease of the sympathetic have been associated with migraine, 
etc., objection has been made to the vasomotor theory of individual varieties 






THE THEORY OF HEADACHE 879 

of headache. Upon the hypothesis that many forms of headache depend upon 
vasomotor, especially upon vasospastic, processes it might readily be con- 
cluded that disturbances in various sensory areas might reflexly produce 
headache. This theory alone would explain toxic headache. Unfortunately 
we are too little acquainted with the relation of the finer vessels of the dura 
to those of individual nerves. 

This must incite us to renewed investigations. It is quite conceivable that severe 
pain in these vessels may lead to conduction disturbances in other nerves. We know 
this to occur in paralysis, which is known as periodic oculomotor paralysis. There is 
a complete paralysis of the third nerve always accompanied by a true attack of 
migraine. It is usually very severe; sometimes it is the first from which a person 
suffers, at other times paralysis occurs only with a later attack. This paralysis may 
exist for months and then disappear, or, long after it has passed away, even years 
'after, there may be a renewed attack with most severe pain. Mobius, who first 
described the symptom-complex, elaborated a few differential points in the picture of 
migraine which leads to paralysis of the ocular muscles in comparison with a typical 
attack. According to this author a most important point is that the attacks are very 
rare, that they are particularly severe, and that hereditary predisposition, as in most 
cases of true migraine, cannot be determined. Since, however, oculomotor paralysis 
has been observed occasionally in typical migraine, the question whether these affec- 
tions are distinct is still a matter of discussion. Two necropsy reports of such cases 
are at hand. In both a tumor was found at the root of the nerve. We know that 
intradural tumors when situated in the motor zone, or even in other areas of the 
cortex within the region of the hypocampus major, often lead to occasional attacks of 
true epilepsy. It is therefore quite possible that they may generate other spastic 
symptoms at the base of the brain, and may also be the cause of vascular spasm. We 
then assume that vascular spasms in the area of the cerebral arteries are due to an 
unknown cause, probably based upon heredity, and are also directly due to a tumor 
of the base. The similarity of the clinical pictures as well as their slight differences 
would thus be explained. 

It will be observed that, on the whole, we are upon hypothetical ground. 
If, however, renewed investigations give us any light, we must clearly dis- 
criminate between what we positively know and what is mere surmise. We 
know tliat the agent which produces headache attacks the dural nerves, per- 
haps also some of the nerves of the pia. The sensation of pain is, of course, 
conveyed through, the brain. We know that headache may be due to various 
causes. We do not understand the mode by which they damage the nerves, 
nor in what manner they produce pain. Anemia of the nerve is, at all events, 
a factor which may here be included, as well as hyperemia. Perhaps most 
forms of headache may be attributed to these two conditions. 

Naturally, the physician must not allow this theoretic explanation to fore- 
stall treatment. Empiricism has made us acquainted with a number of reme- 
dies and processes which may afford relief. The development of diagnosis 
indicates the points where our aid may with advantage be offered. We know 
this much of headache: that each individual case should interest and stimu- 
late the physician ; and we know so little of its theory that now when positivej 
fundamental laws are everywhere determined, the questions which have arisen 
should form a new stimulus to scientific endeavor. 



57 



PARALYSIS AGITANS 1 (PARKINSON'S DISEASE) 

By W. ERB, Heidelberg 

Our knowledge of paralysis agitans has almost wholly been acquired since 
the beginning of the nineteenth century. In 1817 the disease was for the 
first time clearly described by Parkinson, and subsequently by various authors 
(Todd, Komberg, Trousseau and others) with more or less exactness; in 
these descriptions, however, there was some confusion with different related 
pathological conditions, with chorea, and with other forms of tremor, espe- 
cially with the intention tremor of multiple sclerosis. 

It was about thirty or forty years later before the clinical history and 
diagnosis of the disease, as well as its nature and pathologic importance, were 
based upon a firm and positive foundation. 

We must assign to Charcot and his pupils in the Salpetriere the credit 
of having given us an almost exhaustive symptom-picture of the features of 
this peculiar disease; they had at their disposal uncommonly rich material 
which facilitated the diagnosis and, above all, enabled them to sharply dif- 
ferentiate it from multiple sclerosis. The work of Ordenstein (1867), which 
was inspired by Charcot, marks this important advance in the history of 
paralysis agitans. 

This field has since been further developed, especially by German authors; 
some characteristics of the pathologic picture are still doubtful, for others 
a correct explanation has been found. The two principal symptoms of the 
disease are the following: The tremor and muscular rigidity (to define it 
briefly), which have been clearly emphasized; and special stress has also 
been laid upon the remarkable and frequent occurrence of cases without 
tremor ; we now, at the beginning of the twentieth century, possess a definite, 
complete, and almost exhaustive symptom-picture of Parkinson's disease. 

We have been less fortunate in the recognition of the pathologico- 
anatomical foundation and the nature of the disease. With increasing minute- 
ness in histologic methods of investigation, renewed efforts have been made 
to base this severe disease, which with apparent justice was regarded as a 
" functional neurosis," upon a recognizable anatomical foundation. Many 
observers obtained more or less conclusive findings, but these have by no means 
as yet furnished a satisfactory explanation, and are still sub judice. We may 
assert to-day that no positive and undoubted anatomical foundation for the 
disease has yet been revealed, at all events none has been generally accepted. 
The solution of the problem is left, therefore, to the twentieth century. 

i Synonyms: Shaking Palsy; Spasmus Agitans; Chorea Festinans seu Procur- 
sive, etc. 

880 



ETIOLOGY 



881 



The therapy of this intractable malady has not kept pace with its exact 
recognition ; in its treatment we are almost as powerless as we were fifty years 
ago, although we have succeeded by some general procedures, which are ex- 
tremely acceptable to the patient, and by a few palliative drugs (hyoscin, 
duboisin) in ameliorating this most distressing affection. 

DEFINITION 

By paralysis agitans we mean a disease of advanced age which is almost 
exclusively confined to the motor-apparatus, and is manifested by a charac- 
teristic tremor of the limbs gradully increasing in severity and extent J sub- 
sequently affecting the trunk, and also marked by general rigidity and the 
slowing of muscular movements with increasing muscular tension; from these 
symptoms almost all of the other phenomena of the affection may be derived, 
the anatomical foundation of which has not been positively determined. The 
disease is of progressive character and is usually fatal, but only by exhaustion 
and marasmus after a prolonged duration. 

ETIOLOGY 

Up to the present time the causes of paralysis agitans are very imperfectly 
known. Gradually, however, a few points have been clearly demonstrated to 
be of undoubted significance and effect. 1 

1 In the following I shall give in part the substance of numerous original observa- 
tions, consisting of 183 cases which I saw within fifteen years. Of these 183 cases, 143 
occurred in my private practice and 40 were observed in my Clinic. ( A number of cases 
which were also seen in the Clinic have not been included because the notes of the 
cases were not sufficiently comprehensive.) 

I shall here mention these results somewhat in detail as they may be of use in the 
compilation of later and more full statistical reports. 

Among the 183 cases there were 120 men and 54 women, which gives a proportion 
of about 5 to 2. In my private cases the number of men was greater than this : 105 
men to 38 women, which gives a proportion of about 3 to 1, while among my clinical 
patients there were 24 men and 16 women, therefore an exact proportion of 3 to 2. 
The total number of these (40) is, however, relatively small. 

In regard to the age at which patients were attacked by the disease, the following 
table is conclusive : 







Men. 






Women. 






Onset at Age of 














Total. 


Private. 


Clinic. 


Total. 


Private. 


Clinic. 


Total. 


21-30 vears 


2 


1 


3 








3 


31-40 years 


10 


4 


14 


4 


1 


5 


19 


41-50 vears 


18 


10 


28 


11 


4 


15 


43 


51-60 vears . . . 


46 


6 


52 


18 


11 


29 


81 


61-70 vears . . . 


27 


3 


30 


5 




5 


35 


71-80 years 


2 




2 








2 




105 


24 


129 


38 


16 


54 


183 



From this it is evident that by far the greatest number of cases (81 of 183 cases) 
occurred in the sixth decade of life (the age from 51 to 60) ; in the next greatest 



882 PARALYSIS AGITANS 

The disease may be designated as not very common; only nerve special- 
ists often see it; but these are the patients who congregate in almshouses. 

Heredity plays a certain but by no means a prominent role; from a 
moderate number (about 16 per cent.) of the patients we hear that parents 
or grandparents suffered from the same affection; I know a family in which 
three or four sisters are afflicted with the disease. 

number the onset was in the fifth decade (43 cases) ; in the seventh decade (61-70 
years) there were 35 cases; but even prior to the 41st year of life there were 22 
cases, three of these occurring before the 31st year, the youngest being 28; after the 
70th year there were only 2 cases, both men. Therefore two-thirds of all the cases 
(124 out of 183) occurred in the pre-senile period of life, their onset being between 
the 41st and 60th years. 

Curiously enough, the earliest period for the onset of the disease, at least in men, 
was observed in the lower classes, and then in those in better circumstances ; among 
24 men seen in the Clinic 4 were attacked in the fourth decade, 10 in the fifth, 6 in 
the sixth, 3 in the seventh, and none in the eighth decade, while among men in the 
higher walks of life 46 cases occurred in the sixth, 27 in the seventh, and only 18 in 
the fifth decade. Therefore the average age at which the 105 men of the higher classes 
were attacked = 54.4 years ; that of the 24 men in the Clinic = 46.8 years. This dif- 
ference is not apparent among the women. Here the average age at which those in 
both categories (38 from the higher classes and 16 from the lower) were attacked 
is almost the same, 51.2 years. 

In regard to individual etiologic factors my investigations show that syphilis plays 
no r61e; among 101 men from the better classes only 11 had previously had chancre; 
and 8 had well defined syphilis, therefore a total of 19 per cent. ; that is, a smaller pro- 
portion than would otherwise be found among men in the better walks of life ( 22 to 24 
per cent, according to my large statistical compilation). Among the 15 men from the 
lower classes, of whose cases a record was kept, none had syphilis. Among the 54 
women there were no reports of this disease nor was any ever suspected of having 
syphilis. But in women such a history is of very little value unless the facts have 
been most minutely investigated. 

I have endeavored to ascertain whether direct heredity (occurrence of tremor, that 
is, paralysis agitans, in parents, brothers, sisters, or other relatives) was present. 
Among 62 men there were 10 who positively reported this, among 31 women there 
were 5; therefore, among 93 cases there were 15, = about 16 per cent., in whom heredity 
was suspected; hence this factor appears to play no predominant role in the develop- 
ment of the disease. 

The effect of bodily trauma (wounds, shock, fall, etc.) was cited as a cause in 
9 cases. In some instances, however, psychical trauma was probably also a factor; 
this condition in a more acute form is mentioned nine times (as a severe, "fatal" 
fright, fear, extreme excitement, etc. ) , in a more chronic form ( as prolonged anger, 
sorrow, care, excitement, etc.) in 30 cases (this being the total number of cases in some 
of which, however, accurate reports are lacking). 

In 4 cases the affection is said to have developed after an attack of influenza, and 
this does not appear unlikely, considering our experience with influenza. 

In regard to other etiologic conditions — refrigeration, abuse of alcohol and of 
tobacco — my notes reveal nothing of value. 

I shall at once add to these statistical reports a few clinical observations as 
follows : 

In 173 cases there were reports concerning the localization of the symptoms at the 
onset of the affection. These began with tremor and stiffness in the upper extremity 
(hand and arm) in not less than 137 (77 times upon the right, 60 upon the left) ; 
in the lower extremity in 25 (12 times upon the right, 13 upon the left) ; in a more 
diffuse manner upon both sides (according to the report of the patients) in 9 cases, 
and especially often with stiffness; and in the bulbar nerve tracts, in the lips, and in 
speech in 2 cases. 

Cases with and without minimal tremor occurring late were found in not less 
than 37 of 183 cases, among these 28 times in men and 9 times in women. 



ETIOLOGY .883 

Age is a predominating factor; paralysis agitans is certainly a disease of 
advancing years although not actually of the senile period; it occurs almost 
exclusively after the fortieth or the fiftieth year; I know of some cases which 
began at seventy or seventy-one, but the rule shows many exceptions. Some- 
times the disease begins prior to the fiftieth, and not rarely even before the 
fortieth year; in most exceptional cases it may appear even in the twenties; 
I saw three cases of this kind, the youngest being 28 years of age. Other 
authors have seen cases at twenty-six, at twenty-one, and even beginning at 
nineteen and seventeen years ; Lannois reports a typical case following rotheln 
at twelve years of age. It is questionable in these cases whether the diagnosis 
was correct, and this appears the more likely since we have recognized the 
hysterical imitation of paralysis agitans. 

My own experience in 183 cases shows that 118 of these began after 
the fiftieth year (of these, two beyond the seventieth year), 43 between the 
forty-first and fiftieth years, in 19 cases between the thirty-first and fortieth 
years (see note on page 881). At least two-thirds of all cases occurred be- 
tween the ages of forty-one and sixty, therefore certainly not in the senile 
period. Almost exactly the same proportion (70 per cent.) was given by 
v. Krafft-Ebing. 

The disease does not occur, as was formerly believed, with about the 
same frequency in both sexes; the number of men predominates decidedly. 
My figures show that in the higher classes (143 cases) the proportion is 
about 3 to 1; among the lower classes (40 cases) 3 to 2, and in the total 
number it is about 5 to 2. Gowers found it 5 to 3, v. Krafft-Ebing, among 
100 cases, 3 to 2. 

Whether the disease is more frequent among Jews than among other Euro- 
peans, as might appear from v. Krafft-Ebing's figures, I am unable to state. 

That syphilis plays no prominent role in the development of the affection 
is shown by my statistics; these prove that among 116 cases there were only 
19 with a previous chancre or syphilis; therefore the number of cases with 
syphilis was less than the average proportion usually found among the male 
adult population ; this is sufficient proof ! Among women suffering from 
paralysis agitans syphilis could not be determined and, indeed, was hardly 
suspected. 

Neither can a decided influence be attributed to alcoholism. This would 
naturally be more frequent and predominate most extensively in men. The 
very common alcoholic tremor differs essentially from the tremor of paralysis 
agitans. 

Other factors mentioned as causes — refrigeration, damp dwellings, insani- 
tary conditions of life, infectious diseases and the like — have by no means 
been positively proven, but perhaps influenza alone cannot be wholly ex- 
cluded. 

Numerous recent observations — in conformity with earlier reports — have 
demonstrated beyond doubt that psychical and somatic traumata may produce 
the disease. 

It has been proven that sudden and profound psychical emotion, a severe 
and acute psychical trauma, severe " deadly " fright, fear, horror, and the 
like are prone to cause the affection; among my cases there were 9 such, 
of which I have described 2 in detail. Even more frequent are chronic 



884 PARALYSIS AGITANS 

psychical conditions, such as prolonged sorrow, anger, anxiety, or excite- 
ment, which have preceded the disease; such psychical lesion was indicated 
by the patient in 30 of my cases, and numerous similar examples are found 
in literature. 

On the other hand there appears to be no doubt that bodily trauma (in- 
juries, shock, contusions, fractures, burns, etc.) are the precursors of paral- 
ysis agitans; Walz published a long article on this subject, v. Krafft-Ebing, 
v. Gorski, Leva, Bychowski and others have made similar observations, and 
cases of this kind are increasingly frequent in the literature of accidents; 
in 9 of my cases bodily trauma was named as the cause. These traumata 
were in part of mild nature; but a few weeks or months later the first symp- 
toms of the affection appeared — usually in the member attacked by the 
trauma. Since in most accidents fright and fear, therefore bodily and 
psychical trauma, act together, the latter not rarely predominating, it is diffi- 
cult to decide which of the two has had the larger share in causing the dis- 
ease. In any event it appears unquestionable that one as well as the other 
may produce paralysis agitans, and more readily when both causes act simul- 
taneously. 

In how far a certain predisposition to the affection is here operative, 
which is quite likely, cannot at present be determined. 

According to Krafft-Ebing, over- exertion, surmenage local, is said to be 
the provocative cause in some cases. 

Therefore, besides advancing years (which alone do not explain the con- 
dition since, otherwise, the disease would be much more frequent and would 
appear almost as a senile phenomenon) only psychical and physical traumata 
may be recognized as in any sense positively determined causes of paralysis 
agitans. 

SYMPTOMATOLOGY 

The symptoms of paralysis agitans have been so frequently and so accur- 
ately described in all of their individual features that we may here be con- 
tent with a brief sketch outlining the most salient points. In the well devel- 
oped cases these are so pregnant and so uniform that the disease is recognizable 
at the first glance, and it is somewhat remarkable that this was formerly 
for so long a time grouped or confounded with other diseases. 

The name of the disease indicates that in the " tremor," the " shaking," the 
" agitation " of the limbs the most important symptom of the disease is 
found. A closer study of the malady shows, however, that besides the tremor 
another symptom is just as marked and of as great significance, and in con- 
nection with the tremor impresses upon the pathologic picture its charac- 
teristic stamp; this is a peculiar muscular stiffness, a rigidity, which reveals 
itself by a rigid posture and by a certain slowness and difficulty of move- 
ment which frequently annoys the patient more than the tremor; a symp- 
tom, moreover, which sometimes exists alone, the tremor being wholly 
absent. 

These two symptoms, tremor and muscular rigidity, are the ones which 
first appear, are the most typical of the clinical picture, and clearly reveal 
the individual processes of the affection. They are always present although 
in different degree, sometimes uniformly, at other times one, and then the 



SYMPTOMATOLOGY 885 

other, being predominant or absent. From this various forms of the disease 
arise, with which we should become familiar. 

Tremor. — The tremor for the most part begins gradually, almost invari- 
ably in the hand and unilaterally, more frequently upon the right side than 
the left (77 to 60), and is accompanied by feeble, quite rapid, yet scarcely 
noticeable oscillations (4 to 7 in a second), first in the fingers and thumb. 
Either from excitement or exhaustion, or apparently without cause, it is 
temporarily increased, the movements gradually become more marked, more 
extensive but slower ; finally, actual tremor occurs, and continues in a mechan- 
ical manner for hours, sometimes without an interruption for an entire day; 
the finer and complicated finger movements imitate those made in rolling 
pills, in spinning, in counting money and the like, and depend upon alternate 
contractions of the small muscles of the hand and forearm. The movements 
toward the trunk become more feeble, they usually continue to be distinct at 
the wrist, but are absent in the elbow- and shoulder- joint or only appear 
there much later; sometimes they are less prominent in the fingers, and are 
observed particularly in the wrist. 

After the hand the leg of the same side is attacked, then the foot less 
intensely but distinctly, and this is later so increased that a continuous un- 
rest develops accompanied by a beating sound of the foot upon the floor. In 
relatively rare cases (25 in 173) the affection began in the foot, and subse- 
quently attacked the arm of the same side ; here also the toes may be especially 
implicated in the movement. 

In two cases I first noted a bulbar localization — in the mouth and neck, 
and in the speech; in nine cases the affection — tremor or stiffness — was either 
diffused upon both sides or at once involved an entire extremity; therefore, 
any mode of onset is possible. 

Usually the tremor is limited for a long time, even for years, to one side ; 
then the other hand, subsequently the other leg, are attacked; sometimes the 
affection begins simultaneously in both hands. 

Finally — but not in all cases — the neck and the head are implicated; 
not rarely the affection is distinctly visible in the muscles of the lips, chin, 
jaw, tongue, and throat. But it never reaches such an intensity as the tremor 
of the head in multiple sclerosis. In very rare cases the muscles of the 
larynx have been found implicated (Eosenberg, Fr. Miiller). 

The intensity of the tremor varies extraordinarily in different patients, 
and increases from scarcely perceptible oscillations to extreme shaking of 
the limbs; it varies with the moods and with the general condition of the 
patient, is augmented by excitement, exhaustion, and the like, and it is also 
increased and manifested by energetic movements first in one hand, and then 
appearing to a still greater extent in the other. 

This tremor — a point especially characteristic — now appears when the 
body is completely at rest; it begins soon after awakening, is more or less 
violent during the day, and ceases during sleep; only in very advanced stages 
does it prevent sleep. It is not increased by voluntary movements (or but 
very rarely), it is therefore absolutely not an intention tremor; on the con- 
trary, it may be transitorily suppressed — at least at the onset of the affec- 
tion — by voluntary movements; this enables the patients to use their hands 
for an astonishingly long period for all the finer movements, as in eating, 



886 PARALYSIS AGITANS 

writing, drawing, piano playing, dressing themselves, etc. Coarser move- 
ments are not at all impeded. Naturally, in advanced stages of the disease 
a decided disturbance of the power of motion appears. 

Muscular Rigidity and Muscle Tension. — Much more frequently this is 
due to the other principal symptoms: Muscular rigidity and muscular 
tension; the majority of the muscles of the body are apparently in a con- 
dition of increased tonus or slight tension which may be objectively deter- 
mined and is combined with a peculiar difficulty in rapidly and easily bring- 
ing the muscles into play; hence, there is a brief delay, or, rather, a prolonged 
latency of voluntary stimulation. 

As a result of this peculiar disturbance in the muscles, we have the char- 
acteristic picture which most patients with paralysis agitans present: Their 
posture, their movements, their facial expression, their voice, and their 
speech. 

The rigidity of the muscles of the face produces the serious, surly, or 
tearful facial expression; that of the muscles of the larynx and the organs 
of speech, the peculiar indistinctness of the speech and voice which often 
seems to vanish; that of the muscles of the arm or hand, the slight flexure 
of the elbow-joints, and the characteristic position of the hand " as if hold- 
ing a pen " ; that of the musculature of the trunk causes the bent posture, the 
difficulty experienced in rising suddenly from a chair, in turning rapidly, in 
turning in bed, or in quickly resuming the equilibrium of the body and alter- 
ing its gravity. The rigidity of the muscles of the leg causes the stiff, spastic 
gait, the bent knee, and the dragging of the tip of the foot upon the floor. 

The body is generally bent forward; the head droops, the back is markedly 
curved, the arms are slightly flexed at the elbows, the legs at the knee; in 
rare cases, however, a more extended, stiffer, and more erect posture is seen, 
the head and the vertebral column being flexed somewhat backward (so-called 
" extension type " in contrast with the ordinary " flexion type " ) . 

The much discussed symptoms, " propulsion " and " retro pulsion," depend 
mainly upon this muscular rigidity which was long regarded as a constrained 
movement. In walking the rigid legs of the patients seem to cling to the 
floor, the upper part of the body is propelled forward, and the patients " hang 
fire" but are alike unable to regain their equilibrium quickly, or to fall, or 
to go forward until they find an obstruction to sustain them, or until they 
finally regain the necessary command of the trunk (chorea festinans!); the 
same condition is noticed with retropulsion. If the patient who is quietly 
standing is drawn back and thus caused to walk backward, it is just as diffi- 
cult to stop him as in walking forward, and for the same reasons. 

On these two symptoms — the tremor and the muscular rigidity — the 
typical picture of paralysis agitans is based; it is recognizable at the first 
glance, for in most cases it reappears monotonously. 

Of course, other more or less constant symptoms are observed: All kinds 
of vasomotor disturbances, redness and heat of the face and head, often com- 
bined with a feeling of heat and burning of the shin, with an increased secre- 
tion of sweat over the greater part of the body, or else a disagreeable sensa- 
tion of cold; rarely there are other paresthesias of the skin, occasionally also 
more or less severe pain in various parts of the body, and, above all, a sen- 
sation of extreme fatigue in the muscles; finally there is a general restlessness 



SYMPTOMATOLOGY 887 

of the limbs, a desire for frequent changes of position which increases with 
the duration of the disease and becomes actual suffering on account of the 
helplessness of the patient; every quarter or half hour these unfortunates 
long for a change of position and are unable to effect it without help. The}' 
must be raised from chair or bed, be turned a countless number of times in 
bed, and thus become a torment to themselves and to their nurses. 

Xotwithstanding all this, the psychical functions and those of the special 
senses are absolutely undisturbed. [Mental activity and initiative are, as a 
rule, undoubtedly reduced. Parkinsonians are loath to undertake the con- 
sideration of any new subject or to actively pursue mental work formerly 
agreeable to them. — Ed.] Except for occasional pain and paresthesia, note- 
worthy sensory disturbances are absent ; the sphincters are intact ; aside from 
the tremor and stiffness gross power and motility remain for a long time and 
only gradually decrease : the vegetative functions are normal. 

Consequently objective examination in the early stages of the disease — 
again excepting the tremor and stiffness with their sequelae — reveals nothing. 
The motility, the gross power, appears quite normal except for the slowness 
on attempting movements; but for all muscular movements — for example, 
in dressing, in eating, and the like — the patients require an increasingly long 
time; the muscles do not atrophy, and the electric contractility of the 
motor apparatus remains normal; but muscular tension and stiffness may be 
objectively demonstrated, and usually are accompanied by a moderate in- 
crease of the tendon reflexes (up to a slight foot clonus) ; only very rarely 
do the tendon reflexes appear to be diminished (on account of muscle tension) 
and just as rarely are they markedly increased : as a number of recent investi- 
gations in a series of cases lias taught me. the patella reflex is quite normal, 
and shows nothing of the pathologic condition described by Babinski — per- 
haps quite an important proof that the affection is primarily of functional 
origin. 

In regard to the cutaneous sensibility, it is usually stated to be absolutely 
normal; however, some authors (Ordenstein, Eulenburg, Kornilowicz, Holm, 
v. Krafft-Ebing, Heimann, and others) have reported disturbances of sensi- 
bility in rare cases, and upon accurate examination Karplus recently found 
in a number of cases all the varieties of objective disturbances in sensation 
which he himself designates as "very varying and inconstant"; namely, 
hyperesthesia- and hypalgesias, also thermal hyperesthesia partly associated 
with hyperalgesia, usually circumscribed and not limited to definite nerve 
tracts, but existing for a long time; they are most likely to be developed in 
the extremity or on the side of the body affected by the motor disturbance, 
but are never very extensive; in one case he found also a well developed 
bilateral hyperalgesia, in another a hemi-hypalgesia ; Karplus believes that 
we may certainly assume from this a hysterical disturbance in sensibility. 
In many instances, however, in spite of preceding subjective pain and pares- 
thesia, this objective disturbance in sensibility is absent. 

It would add to the interest of our conception of the affection if these 
findings of Karplus could be confirmed. 

Frenkel has lately described a peculiar change in the shin — a thickening 
and tense adherence to the subcutaneous layers — as a very constant symptom 
in paralysis agitans which he has utilized to explain some of the symptoms. 
58 



888 PARALYSIS AGITANS 

Karplus tested these reports with absolutely negative result, and for this 
reason questions the accuracy of Frenkel's statements. 

The internal organs, as well as the sphincters, the organs of special sense, 
of ocular movements, the pupils, etc., are normal. There is no aphasia, but 
there is a certain weakness of the voice with a somewhat hasty, monotonous, 
" vanishing " utterance of sentences as though the patient were anxious to 
get them out and done with; this is especially prominent in the later stages, 
and with the gently trembling lips gives us a very peculiar impression. 

The vegetative functions are not affected; the intelligence and memory of 
the patient appear unimpaired. 

I have known such patients to carry on a large business, conducted prop- 
erly in all respects, without difficulty; even a mild disposition and good 
humor may be long retained in spite of the severe and hopeless nature of the 
affection; but, of course, to this there are many exceptions. 

This clinical picture will not be found uniformly developed in all cases; 
as has been already stated, even the two main symptoms, tremor and muscular 
rigidity, show the greatest possible variations; one or the other may be less 
prominent; this is especially true of the tremor, which chiefly impresses its 
clinical stamp upon the disease. While the rigidity shows a varying intensity, 
although it is rarely absent, the tremor may he completely lacking at the 
onset and in the further course for years, or it may be temporarily only 
slightly apparent, or may be so rudimentary that even after prolonged obser- 
vation it is exceedingly difficult to perceive it, yet, in spite of this, the clin- 
ical picture may be well developed and unmistakable. These are not the 
infrequent cases of Par-hinsons disease without tremor — of paralysis agitans 
sine tremore or sine agitatione — which are of especial importance since they 
may lead the inexperienced observer into most unpleasant diagnostic errors, 
and may direct the treatment into improper channels. Such cases are not 
rare. Among my 183 observations I find no less than 37 — therefore about 
20 per cent. In these instances the first symptom of the disease was a certain 
stiffness, awkwardness, and weakness in one or the other hand or in a leg; 
or the onset was more gradual with a sensation of fatigue, a paresthesia, dif- 
ficulty in executing certain movements, the dragging of one leg, and other 
nervous symptoms. I have seen a number of such patients in whom either 
beginning disease of the brain or spinal cord, slight apoplexy, spastic spinal 
paralysis, brain tumors, or even neuroses of writing and the like appeared 
likely, yet in which, frequently at the first glance, or only upon prolonged 
and continuous observation, the diagnosis became clear. The further course, 
the final appearance of the tremor, the increasing characteristic habitus of 
the patient, the absence of other symptoms of the diseases in question, ren- 
dered the diagnosis positive. I* must admit that before I became sufficiently 
familiar with the affection I made several such erroneous diagnoses, and 
therefore I desire to call especial attention to these " formes fruste " of paral- 
ysis agitans. They are readily recognized by the peculiar position of the 
hands and arms, the facial expression, the posture, the gait, and the voice 
of the patient. 

The question arises whether the opposite — paralysis agitans without stiff- 
ness and muscular rigidity — exists. This is quite likely; certainly in some 
cases there is very slight rigidity. It appears to me doubtful whether the 



PATHOLOGY 889 

absence of rigidity can be regarded as proof that the tremor closely resem- 
bling paralysis agitans is of hysterical origin. This is quite commonly 
assumed, but the condition should be more comprehensively studied. 

COURSE 

The disease is generally very protracted but uninterruptedly progressive; 
it usually lasts for many years, five, ten or fifteen, and sometimes even 
longer. 

Tremor and rigidity increase; the former may pass into general trembling 
of great intensity and duration, so that the patients can no longer sit up 
and can no longer lie in bed, cannot divert their minds with anything, and 
can only be fed with extreme difficulty; the rigidity becomes so marked that 
the patients are absolutely helpless, constantly require aid, and must be given 
a change of position continually during day and night, being a source of 
great discomfort to themselves and others ! In fact their condition is pitiable 
— a condition in which death alone can bring relief. Xot rarely insomnia, 
psychical disturbances, irritative conditions, delusions of persecution, im- 
becility, senile and arteriosclerotic dementia are added to complete the misery 
of this existence. 

Provided intercurrent diseases (bronchitis, pneumonia, influenza, apo- 
plexy and the like) do not early terminate the life of the patient, he finally 
succumbs miserably with bed-sores and general marasmus. 

Some cases run an exceedingly rapid course toward such an unfortunate 
termination; only recently I saw a lady who, in the course of a single year, 
was reduced to this terrible plight; on the other hand, many remain for years 
and decades with onl} r the initial symptoms of the affection, and perish before 
its more advanced stages are reached. 

There is no recovery from this unfortunate disease. Reports of individual 
cures are not sufficiently trustworthy, and probably depend upon diagnostic 
errors (confusion with hysteria!); here and there the affection may be ar- 
rested, but very rarely does a transitory improvement occur; in the most 
favorable cases the disease runs a protracted course to its unavoidable end. 

PATHOLOGY 

The anatomical foundation of this severe and incurable affection has re- 
cently been investigated countless numbers of times by our improved modern 
methods, but, unfortunately, with but little success ! We must still admit 
that we do not know the positive and constant pathologico-anatomical founda- 
tion of paralysis agitans. 

I must, therefore, decline to give a detailed description or to criticise 
the anatomical findings which have been reported by various authors, and 
shall be content with a brief sketch. A number of expert observers have 
attempted this task with all of the aids of our modern technic; in contrast 
'to the former almost negative findings, modern investigators have almost in- 
variably reported positive findings in the brain as well as in the spinal cord, 
in the peripheral nerves and even in the muscles (Borgherini, Koller, v. Sass, 
Ketscher, Hunt, Dana, Redlich, Sander, Nonne, AVollenberg, Karplus and 



890 PARALYSIS AGITANS 

others), and most of these are inclined to regard their findings as of some 
significance in the pathological anatomy of paralysis agitans. That other 
competent observers, such as Oppenheim and Fiirstner, report entirely nega- 
tive results in undoubted cases by the same methods robs these opinions of 
much of their weight. 

What has been found is about as follows: In the brain dilatation and 
thickening of the vessels, connective tissue increase, diffuse and insular 
proliferation of the giia, atrophic and degenerative changes in the nerve 
fibers and ganglion cells, vacuole formation, pigment degeneration, nuclear 
disappearance and partial destruction of the fine dendrite branchings of the 
latter; according to Nissl the same changes have been found with remarkable 
frequency in the spinal cord, especially in the large ganglion cells of the 
anterior columns, also in the peripheral nerves; occasionally proliferations 
of the neurilemma, degenerative changes in the nerve fibers, and slight fatty 
degeneration of the muscular fibers have been reported (even in the motor 
end plates, Dana) besides thickening of the perimysium and of the vessels; 
usually, however, the muscles were found to be normal. 

Most authors regard these changes as by no means specific or character- 
istic; but, on the contrary, they consider their quality to be identical with 
the senile changes invariably found in the nervous system of the aged ; quan- 
titatively, perhaps also by their special localization in paralysis agitans, they 
chiefly differ from the alterations in the aged; therefore these changes are 
the chief expression of an abnormal, extreme, and early senility of the nervous 
system. 

But the fact alone that paralysis agitans is frequently, and even as a 
rule, observed at a period of life when no other signs of senility are noted, 
which period might at most be designated as presenile, also the circumstance 
that the disease would necessarily appear much more frequently and invari- 
ably in elderly persons, that senile tremor, and in fact the usual signs of 
advancing age, are by no means identical with the clinical picture of paral- 
ysis agitans, and, finally, that the most marked senile changes in the nervous 
system may be present without paralysis agitans, absolutely undermines the 
support of this view and renders it most unlikely that the alterations found 
are essential features of paralysis agitans. 

The circumstance that the malady apparently possesses no characteristic 
localization, that it is irregularly disseminated throughout the entire central 
(and even peripheral) nervous system, that the lesions are by no means 
strictly confined to the motor apparatus (motor neurons I and II), as might 
be expected from a symptom-complex limited almost exclusively to the motor 
apparatus, is still more in opposition to this explanation of the anatomical 
finding. 

In the anatomical findings, therefore, we recognize nothing distinctive; 
they probably belong only to the terminal stages of a process going on for 
many years, the onset of which was unnoticed. It does not appear strange 
that in muscles which have been immoderately active for years (Dana cal- 
culated that a muscle examined by him had undergone not less than 840 
million contractions during the continuance of the disease for eight years!), 
" slight fatty degeneration " and some connective tissue increase should be 
found; or if, in the peripheral nervous system and the spinal ganglion cells 



PATHOLOGY 891 

of persons who have been subjected to frequent irritations, who have suffered 
for years or have finally succumbed to marasmus or other fatal acute disease, 
such degenerative changes are found as are revealed by MssPs method under 
varying conditions, or if we find in the brain, under the same circumstances, 
vascular degeneration, glia proliferation, and degeneration of the nervous 
elements. 

For the present these reasons permit us to conclude that the actual, un- 
questionable, and invariable anatomical lesions of paralysis agitans have not 
yet been found; perhaps in the pathologic changes mentioned above there are 
important conditions, but these are not positive. 

Thus, pathological anatomy leaves us in absolute ignorance of the actual 
localization of the affection, and the solution of the question must be attempted 
from a clinical standpoint. 

It has sometimes been thought that the muscles themselves might be the 
seat of the affection; in my opinion everything militates against this view — 
chiefly because of the reasons which cause us to locate the disease in the 
central nervous system, and because there are no facts which make it likely 
that such a peculiar spasm of the muscles could occur without an implication 
of the nervous system. If the report, lately promulgated, of the frequent 
occurrence of sensory disturbances in paralysis agitans is actually confirmed, 
this view will be shattered. 

The same is true of the peripheral nerves; we have no knowledge of a dis- 
ease of the peripheral nervous system in the symptom-picture of which there 
is even occasionally the slightest syndrome resembling paralysis agitans aside 
from the fact that this is not localized in individual nerve trunks but, quite 
differently (analogous to affections due to central causes), in motor as well 
as sensory regions. 

Implication of the spinal cord might much more readily be thought of; 
from the earliest times anatomical changes in the spinal cord in this disease 
have been sought and were supposed to have been found ; this is explained by 
the fact that the spinal cord can be investigated with more facility, and that 
the brain has often been neglected in the examination; the entirely negative 
findings, mentioned previously, would be opposed to such localization pro- 
vided we recognize an anatomical lesion to be the determining factor. By 
analogy, one of the symptoms of paralysis agitans — muscular tension and 
rigidity — could be clinically localized in the spinal cord and in the pyramidal 
tracts; but there is no marked increase of the tendon reflexes, and the normal 
plantar reflex controverts this theory ; a functional irritation of the pyramidal 
tracts might be conceived, but this might also originate from the brain. For 
the localization of the tremor in the spinal cord (gray anterior columns?) 
we possess no analogy; we know of no spinal cord affection, whatsoever its 
name or seat, in which primarily or secondarily the tremor of paralysis 
agitans or the entire syndrome occasionally appears. 

On the contrary, everything in the clinical picture indicates that the 
original seat of the affection is the brain; for instance, the fact that a uni- 
lateral paralysis agitans has sometimes been found associated with coarse 
local brain lesions (tumors in the thalamus and cerebral peduncles, sclerosis 
of the hippocampus major), etc.; that a* posthemiplegic paralysis agitans 
(analogous to chorea and athetosis, posthemiplegia) is occasionally observed; 



892 PARALYSIS AGITANS 

that the affection so frequently occurs in consequence of psychical trauma; 
that it almost always appears unilaterally; finally, its remarkable resemblance 
to the symptom-complex in the most common form of hemiplegia (after 
apoplexy in the region of the large central ganglia and in the internal cap- 
sule). In this condition we find paralysis, contractures, and increased tendon 
reflexes, most marked in the arm, less so in the. leg, and most feeble in the 
head and the face; in paralysis agitans, however, we note the same intensity 
of the tremor, the same rigidity and muscular tension, the same increase of 
the tendon reflexes ! The posture and gait of the side first affected so closely 
resemble those of hemiplegia that cases without tremor can sometimes with 
difficulty be distinguished, and for this reason are frequently confounded 
with cerebral affections. The terminal stages of the disease, the mental de- 
terioration, the not infrequent psychopathic complications are also in favor 
of this view. 

Naturally this does not give us an exact localization of the process within 
the brain. Whether its course runs directly in the path of the cortico-spinal 
motor neuron or in its collateral associated tracts, whether in the motor 
cortical region, in the neuron cells or the axon bundles, in the corona radiata, 
in the internal capsule or in the central ganglia of the brain-stem — who can 
decide? At all events speculation is futile; it is, however, certain that at 
the onset of the affection there is no severe or coarse lesion in the motor 
apparatus, for with such a condition the unimpaired motility would not be 
compatible; we must therefore think primarily of finer, nutritive, molecular, 
or chemical changes which may subsequently cause structural lesions and 
profound nutritive disturbances. 

If the sensory disorders which appear should prove constant, a more 
diffuse process must be considered ; the conditions here are analogous to those 
of tetany, which was also considered to be a pure motor neurosis until 
J. Hoffmann discovered changes in the sensory nerves and those of special 
sense. This furnishes a clue and guide to any one capable of investigating 
the actual nature of the malady. 

But of this we are still absolutely ignorant. We do not recognize the 
true foundation nor the nature of the disease from the anatomical changes; 
even the obvious and very plausible hypothesis that the affection is a prema- 
ture and extreme senility of the nervous system has been rejected for impor- 
tant reasons previously stated. Modern experience and opinions in regard 
to many endogenous toxic effects of glandular origin — I refer particularly 
to Graves' disease, myxedema, tetany, Addison's disease, and similar ones — 
may lead us, and in fact have done so, to regard paralysis agitans as a glan- 
dular autointoxication little as we can reconcile this view with the usual 
psychic and traumatic origin of the disease. But this very interesting 
theory is scarcely more than a possibility; no definite facts support it, and 
therapy based upon this idea (Dana) has been entirely without result. 

The hypotheses of exogenous, infectious, rheumatoid or simple toxic 
action, which, after a long time, exert a destructive power upon the nervous 
elements only (perhaps with advancing age or other deleterious condition) 
are at this time visionary. 

To develop these hypotheses further would be futile; I coincide in the 
opinion of Jolly, v, Krafft-Ebing, Wollenberg and others that at this time 



DIAGNOSIS 893 

we must accept the view of an originally functional, fine, nutritive, molecular 
disturbance principally localized in the motor apparatus, but probably also 
in the central nervous system. That during the long course of the malady 
this derangement may finally lead to visible nutritive disturbances, to atrophy 
and degeneration of the nervous elements and their surroundings is not re- 
markable, and finds its analogy in other diseases of the nervous system (com- 
motion, traumatic neuroses, some psychoses, etc.). 

How these molecular disturbances arise, how they are produced by the 
causes of the disease, is a complete mystery ; paralysis agitans is, in the main, 
a rare disease. The etiologic factors said to be its cause, advanced age, 
psychical and somatic traumata, emotional conditions, refrigeration and the 
like, are so common and so generally existent that in the persons who are 
attacked by paralysis agitans there must still be an unknown factor, an 
existing tendency or endogenous peculiarity which predisposes them to this 
disease. The disease is not particularly apt to be associated with hereditary 
conditions, nor with a special or general neuropathic predisposition; the 
processes of involution both in the male and female, vascular degeneration 
and perivascular proliferations which have been thought of, appear to have 
but little significance. How, and during what period of life this predisposi- 
tion arises is therefore still a mooted question. 

DIAGNOSIS 

In the great majority of cases, even in their first stages, the diagnosis 
of the affection is very easy. In the developed cases the picture is so typical 
and characteristic that the malady is recognizable at the first glance — even 
when no tremor is present; usually, when the patient enters the room, a cor- 
rect " instantaneous diagnosis " may be more easily and positively made than 
in any other disease; I need only refer to the preceding description. 

As a rule, the typical cases with tremor may readily be differentiated 
from other forms of tremor, particularly from the toxic forms (alcohol, 
coffee, absinthe, lead, mercury, etc.), by the quality of the tremor and the 
simultaneous muscular rigidity with its characteristic sequelae apart from 
the recognizable etiology; also from neurasthenic tremor which is of dis- 
similar nature and unlike in appearance; from the tremor in Graves' disease 
(by its different mode of appearance and accompanying symptoms) ; from 
tetany, etc. The differential diagnosis of even senile tremor, as a rule, causes 
no perplexity. It is different in nature, form, and localization, and the other 
symptoms of paralysis agitans, the vasomotor disturbances, the rigidity, the 
typical posture, the voice, the facial expression, etc., are absent; nevertheless 
a senile tremor may sometimes resemble that of paralysis agitans (transi- 
tional forms ?). Since the classic researches of Charcot's adherents, the inten- 
tion tremor of multiple sclerosis can no longer be confounded with the tremor 
of paralysis agitans, and it now appears scarcely worthy of discussion ; never- 
theless the differential factors may be briefly enumerated ! These are em- 
bodied in the nature and manifestation of the tremor. In paralysis agitans 
the tremor comes on during rest and ceases with voluntary movement — at 
least transitorily; in multiple sclerosis it is absent during rest and appears 
upon voluntary movement, increases the more the greater the effort to make 



894 PARALYSIS AGITANS 

exact movements and to near the goal; in paralysis agitans it is fine and 
rapid, appearing with the effort at complicated movements as imitative tremor 
of the hands and fingers, while in the trunk, the neck and head it is absent 
or only slightly noticeable ; in multiple sclerosis it is a coarser, slower tremor, 
with marked excursions, being especially prominent in the trunk and in the 
head. Although there are cases of advanced paralysis agitans in which the 
tremor becomes coarser, " shaking " conies on and even continues with vol- 
untary movement, and although multiple sclerosis occasionally causes a 
tremor resembling paralysis agitans, these diseases differ so markedly from 
each other, and their other symptoms are so pronounced that confusion is 
hardly possible. Paralysis agitans is a disease of advanced age; besides the 
tremor, it primarily presents only the symptoms of muscular rigidity and 
slowed movements with their typical consequences; multiple sclerosis occurs 
chiefly in youthful and middle-aged persons, and, besides the tremor, shows 
a number of other symptoms (headache, nystagmus, scanning speech, spastic 
paresis of the legs, sensory disturbances, affections of the bladder, ataxia, 
optic atrophy, psychical anomalies, etc.) which are completely foreign to paral- 
ysis agitans; these readily permit differentiation. [Exceptional cases of par- 
alysis agitans present a distinct intention tremor in early stages of the 
disease, and tremor in the limbs apparently un involved may frequently be 
demonstrated by directing the execution of somewhat difficult movements, 
such as extracting the contents of an inside breast pocket, reaching behind 
the back to the interscapular area, etc. — Ed.] 

The differentiation from certain forms of tremor in hysteria is sometimes 
difficult. It appears that hysteria may actually " imitate " paralysis agitans. 
Such cases have recently been studied and described by Ormerod, Greiden- 
berg, Eendu, Oppenheim, Dutil, and Bechet, and, finally, by v. Krafft- 
Ebing. Indeed, an astonishing resemblance to paralysis agitans is found. 
Only minute observation of such cases reveals that we are dealing with 
hysteria. Hysterical stigmata are present although not invariably (char- 
acteristic sensory disturbances, attacks, ovaralgia, anesthesia of the pharynx, 
etc.), the tremor often appears suddenly, is widely distributed after a psychi- 
cal trauma, presents great variations, may disappear entirely, and does not 
increase but remains stationary for years; it is more polymorphic, and ex- 
tends rather to the proximal part of the limbs, is not checked by the will, 
but rather increased, as also by excitement; on the other hand, typical mus- 
cular rigidity and slowing of movements are absent as well as the charac- 
teristic posture, the position of the hand as if holding a pen, the facial 
expression, the gait, etc., so that usually a differentiation is still possible. 
Nevertheless, many cases of this kind and others of hysterical origin are 
obscure and cannot be accurately classified. 

More difficult — at least at the onset — is the recognition of the cases with- 
out tremor; here great perplexity arises as to the diagnosis, and errors are 
prone to occur. Nevertheless, if we bear in mind the occurrence of paralysis 
agitans without tremor, these forms also, as a rule, will be distinguished by 
the characteristic posture, the habitus and facial expression of the patient, 
and occasionally slight tremor will be subsequently discovered. Moreover, the 
symptoms of those diseases which come into question (usually of the brain 
on account of their unilateral development!) will by no means be so marked 



TREATMENT 895 

as to remove at once all doubts. But a considerable time may occasionally 
elapse before we are sure of the diagnosis ; advanced cases, even those without 
tremor, may be correctly diagnosticated at the first glance. 

PROGNOSIS 

The prognosis, as is evident from what I have stated, of the course of 
the disease, is hopeless. According to universal experience, the affection is 
progressive and incurable. It is true this advance is often extremely slow, 
and its occasional arrest and transitory improvement not rarely afford the 
patient a certain degree of comfort. In the individual case a relatively favor- 
able prognosis can only be given after prolonged observation ; that is, we may 
perhaps assume that the patient will very gradually and with comparatively 
little suffering approach the inevitable termination of the disease ; other cases, 
however, may sometimes pursue a rapid course and terminate fatally in one 
or two years. 

The few isolated reports of cure must be regarded with extreme skepticism, 
and they probably depend upon erroneous diagnoses, especially upon confusion 
with hysterical forms of tremor; nevertheless, I think we should not entirely 
exclude the possibility of a cure, particularly in young persons. 

TREATMENT 

Considering the prognosis, we can attain no brilliant results in the treat- 
ment of this distressing affection; nevertheless it may be said that, notwith- 
standing the incurability of the disease, we may in mild cases retard the 
progress of the malady and even arrest its course, and in severe cases the 
sufferings may be alleviated and existence rendered tolerable, at least for 
years. 

In an experience of many years, a number of measures were shown to 
be efficacious; these I published in 1898, and I can add but little to these 
to-day. 

As a rule, a causal indication cannot be followed; heredity cannot be re- 
moved, psychical and physical traumata cannot be prevented, sorrow cannot 
be assuaged nor care lessened, and other causes, as a rule, are unknown. 

We must, therefore, primarily fulfil the indicatio morbi by combating the 
disease itself. 

In the first place we must strictly regulate the patients' diet and entire 
mode of life. They must be moderate in eating and drinking, must subsist 
upon simple, nourishing, but varied food, must take little alcohol, no strong 
tea or coffee, and must not smoke to excess; cold sponge baths, tepid ablu- 
tions, and lukewarm baths are beneficial; all mental exertion and excitement, 
everything which strains or fatigues the nervous system, should be avoided; 
moderate outdoor exercise, frequently repeated during the day and for as 
long a time as possible is advisable, also moderate indoor gymnastics and 
mild massage; in the summer an outdoor life and in the winter a residence 
in the South is highly desirable. 

There are three remedies from which I have frequently seen favorable 
results: First, arsenic, which is unquestionably a nervine and alterative of 



896 PARALYSIS AGITANS 

the first rank, and in some neuroses (chorea, tic, Graves' disease, etc.) has 
produced brilliant results. Its favorable influence upon hematopoiesis, metab- 
olism, the nutrition of the skin, the nutrition and function of the nervous 
system is unquestioned, and it has also been frequently employed in paralysis 
agitans; it was first advised as a remedy for tremor in general, but it is 
probably more effective as a nervine and tonic. The form in which it is to be 
administered appears to be immaterial, whether as arsenious acid, in solution, 
in pills, granules, or as Fowler's solution; whether subcutaneously with all 
necessary precautions, or in any combination, is immaterial. I usually give 
Fowler's solution combined with equal parts of aqua foenic. and tincture nux 
vomica (6 to 15 drops three times daily), and find that this mixture is well 
borne by the stomach. 

Lately I have often employed sodium cacodylate which permits the intro- 
duction of arsenious acid in organic combination and in relatively large doses 
without disagreeable consequences; it is best given subcutaneously (0.05 gram 
daily with occasional pauses) or internally either in solution or in granules; 
the preparation used must always be fresh. 

Electricity has also been employed in various ways ; I have most confidence 
in bipolar faradic baths of indifferent temperature and moderate intensity 
continued from 10 to 15 minutes, and these I order three times a week; I 
have little actual experience as to the value of galvanic baths, but they have 
been advised by different authors. Alternating with the baths I usually em- 
ploy galvanization of the head, of the back of the neck, and of the sympathetic, 
as well as of the back and of the extremities, and, it appears to me, with some 
degree of success. 

From my large experience, I advise as a third remedy mild hydrotherapy, 
best when combined with forest and mountain air ; everything that can prove 
exhausting is to be avoided, and only full lukewarm baths, gradually passing 
to cooler half baths with slight friction and affusions, partial washings, etc., 
are permissible. These measures are usually very acceptable and refreshing 
to most patients. 

The cooler, indifferent spas (Johannisbad, Schlangenbad, Eagaz, Wildbad, 
etc.) have a similar effect, but we must not exceed bath temperatures of 
33.5° C, since, as a rule, at higher temperatures the springs have an unfavor- 
able effect upon the patient. 

The combined or alternate employment of these three remedies may be 
advised; but it is evident that, besides these, occasionally other nervines and 
tonics (iron, quinin, strychnin, glycerophosphates, silver nitrate, bismuth, 
valerian, etc.) may be employed. [Persistent massage, combined with full 
passive movements and active but gentle exercises, may do much to liberate 
the contractured postures, reestablish a normal gait, and retard the disabling 
physical features of the disease. A faithful, intelligent and tactful massage 
operator is essential to success. Passive exercises by means of the Zander 
apparatus are also useful. — Ed.] 

Symptomatic treatment which has for its object the amelioration of the 
distressing symptoms of the disease — the tremor and the muscular rigidity — 
is of paramount importance. That this can only temporarily relieve, the affec- 
tion being incurable, does not diminish the value of these palliative remedies, 
and among them we possess some which are often satisfactory and sometimes 



TREATMENT 897 

brilliantly successful. Chief among these is hyoscin, which was first employed 
by me; this is best given in the form of hyoscin hydrobromate, but only in 
a fresh and reliable preparation. It should be given subcutaneously, and is 
exceedingly effective in small doses; it decreases the tremor, the restlessness 
of the limbs, and the rigidity for a number of hours, and makes life bearable 
to the suffering patient. I have seen most astonishing results from this, 
and have continued the use of the remedy for years without its losing its 
effect; an increase in the dose was sometimes necessary, but only very 
rarely were there any disagreeable secondary effects; the remedy was seldom 
ineffective, but this is sometimes the case, and many patients cannot take 
it at all. 

The dose is calculated according to decimilligrams — hence we must be 
cautious with our prescription ! — and two to four decimilligrams injected once 
or twice daily are sufficient; sometimes I give the full dose in the morning 
and one-half as much in the evening which has an excellent effect upon the 
tremor and induces sleep. 

The remedy may also be given internally in pill form, but larger doses are 
then necessitated, and the action is not quite so certain and prompt. 

Duboisin, recommended by Mendel as less dangerous and quite as effective, 
acts similarly to hyoscin. But in my experience this is the case only when it 
is given in large doses (6 to 12 decimilligrams daily divided into three doses) ; 
it occasionally produces unpleasant secondary effects. Under any circum- 
stances we possess in these two remedies excellent palliatives in paralysis 
agitans, and Oppenheim's opposition to their use (Text-Book of Nervous 
Diseases) appears to me as incomprehensible as unwarranted. 

Many other palliatives have been advised and extolled ; the salts of bromin, 
atropin, hyoscyamin, ergotin, strychnin, veratrin, valerian, cannabis indica, 
etc., deserve little confidence, and this applies also to the narcotics and hyp- 
notics which, of course, cannot be entirely dispensed with in the distressing 
terminal stages of the affection. 

Organotherapy, which according to the hypotheses previously mentioned 
might perhaps be useful, has been variously tried, most thoroughly it appears 
by Dana; he used the thyreoid, the trrymus, the hypophysis, the brain, the 
testicle and adrenal extracts in tablet form. The results were absolutely 
negative except with thyreoiodin — which aggravated the disease ! Never- 
theless, I do not regard these results as conclusive. 

" Vibration therapy" which was first employed by Charcot after patients 
had reported to him the favorable and palliative effects of a railroad jour- 
ney, has been carried out partly by means of a so-called " tremor chair/' 
partly with a head mask which was caused to oscillate by means of an 
electro-motor; I mention this merely to add that it is of no real therapeutic 
importance; the same is true of nerve stretching and suspension which have 
occasionally been resorted to here and there; the results at best are very 
questionable. 

In conclusion I believe I have shown that notwithstanding our advancing 
clinical knowledge of this peculiar and severe affection, our recognition of its 
nature and pathogenesis is still incomplete, and our attempts at treatment 
and cure are still exceedingly unsatisfactory. It is to be hoped that in the 
present century more fortunate results may be achieved. 



898 PARALYSIS AGITANS 



Literature 

Parkinson, "Essay on Shaking Palsy." London, 1817. 

Romberg, "Lehrbuch der Nervenkrankheiten," 1851, 2. Aufl., II. 

Trousseau, " Medicinische Klinik des Hotel Dieu." Deutsch von Culmann, 
1868, 2. Aufl., II. 

Charcot et Vulpian, Gaz. hebdom. 1861 et 1862. 

Ordenstein, "Sur la paralysie agitante et la sclerose en plaques." Paris, 1867. 

Charcot, "Klinische Vortrage iiber die Krankheiten des Nervensystems." 
Deutsch von Fetzer, 1874. 

Charcot, "Lecons du Mardi," I. 1892, 2d edition, p. 331. 

A. Eulenburg, " Ziemssen's Handbuch der speciellen Pathologie." 1877, 2. Aufl., 
XII. 

A. Eulenburg, "Real-Encyclopadie." 1888, 2. Aufl. 

Gowers, "Handbuch der Nervenkrankheiten." Deutsch von Grube. Bonn, 
1892, III. 

Walz, "Die traumatische Paralysis agitans. Vierteljahrsch. f. gerichtl. Med. 
1896, 3. Folge, XII. 

Erb, "Paralysis agitans und ihre Behandlung." Zeitschr. /. prakt. Aertzte. 
1898, Nr. 5. 

Erb, "Ueber Hyoscin." Therap. Monatsh. Juli, 1887. 

v. Krafft-Ebing, "Zur Aetiologie der Paralysis agitans." Arbeiten aus den Ge- 
sammtgeb. der Psychiatrie und Neuropath. Leipzig, 1898, III. Heft. 

v. Krafft-Ebing, "Ueber eine typische, an Paralysis agitans erinnernde Form 
von hysterischem Schutteltremor." Wiener klin. Wochenschr., 1898, Nr. 49. 

Wollenberg, "Paralysis Agitans" in "Nothnagel's Handbuch der speciellen 
Pathologic" 1899, XII, II, p. 3. 

Jolly, "Paralysis Agitans" in "Ebstein u. Schwalbe's Handbuch der praktischen 
Medicin." 1900, IV, p.^863. 

Frenkel, "Die Veranderungen der Haut bei der Paralysis agitans." Deutsche 
Zeitschr. /. Nervenhk. 1899, XIV. 

Karplus, "Ueber Storungen der cutanen Sensibilitat bei Morb. Parkinson." 
Jahrb. d. Psych, u. Neurol. Wien, 1900, XIX, p. 171. 



ATHETOSIS 
By L. v. FRANKL-HOCHWART, Vienna 

This disease, or — more correctly — the symptom athetosis, was described in 
1871 by Hammond. 

We understand by the term an abnormal and peculiar movement, inde- 
pendent of the will, and usually appearing in the extremities, the upper being 
chiefly implicated, the lower to a less extent ; the phenomena are always most 
conspicuous in the distal ends. These conditions are also noted in the muscles 
of the face, in rare cases the palate and tongue being involved; the contrac- 
tions are usually unilateral, occasionally bilateral. 

The condition belongs to the group of true cerebral diseases which occur 
chiefly during youth. In a few cases, like symptoms have been observed in 
patients with disease of the spinal cord; and in a few obscure cases the 
necropsy was negative. The following is the history of a typical case : 

In a girl, aged 6, this remarkable symptom-complex revealed the most common 
type of the disease. The mother related a history, usual in such cases, of hereditary 
predisposition and of injuries during birth; there was no report of hereditary syphilis; 
she stated that the child was normal at birth and was well and strong. At the age 
of twelve months, without assignable cause, it was attacked with fever, preceded by 
chills ; the physician was unable to make a positive diagnosis. After a few days the 
child entirely recovered ; in the course of time, however, the mother noted that the 
right extremity was but little and imperfectly used; some weeks later the move- 
ments slightly improved. But a certain stiffness of the right extremity was noticed, 
and, gradually, peculiar movements in the right hand. When the child began to walk 
it dragged the leg; now and then the mother observed that the toes contracted. Upon 
close questioning it was learned that in the child's second year there were now and 
then epileptic attacks, she did not develop well mentally, and now distinctly showed 
psychical defects. The little patient was said to have no pain. It was late before 
she learned to control micturition. When examined, however, there were neither 
bladder nor rectal disturbances. Examination revealed a slight weakness of the right 
facial nerve supplying the mouth, and a little deviation of the tongue to the right, 
the other cranial nerves being normal. The child's psychical development appeared 
to be greatly retarded, but speech was normal. The right side showed distinct paresis, 
most marked in the right upper extremity. In walking the right leg was dragged. 
In this member as well as in the upper extremity there was slight atrophy and mod- 
erate contracture with distinct increase of the reflexes; sensation was normal. 

We have here the familiar picture of spastic hemiplegia which often fol- 
lows the febrile diseases of infancy, and the cause of which, according to 
Benedikt, Vizioli, Strumpell and others, is usually encephalitis. 

Following this description of the preceding condition, we now turn to the main 
symptom, the peculiar movements which were conspicuous in the child at the onset 

899 



900 ATHETOSIS 

of the disease. We noted that the upper arm was adducted, the elbow-joint was pecu- 
liarly and slowly flexed, and extensor movements alternated. At the shoulder- joint 
the upper arm sometimes changed its position from adduction to abduction; now and 
then this was quite marked. In the intervals there were slight movements of pronation 
and supination in the elbow- joint. 

The wrist- joint was usually hyperflexed, frequently with a tendency to a radial 
position. It was markedly resistant to passive movement; occasionally the position 
changed, but not to a great extent. The disturbance, however, had its principal seat 
in the hand, the fingers of which frequently were more or less flexed; the thumb 
was often tucked slightly under the fingers. In these abnormal movements the small 
muscles of the hand were almost invariably implicated, but the long finger muscles 
were rarely at rest. Sometimes the fingers were distally flexed, the thumb opposed, 
the patient unsuccessfully attempting to extend the phalanges. When I tried to 
bring about extension passively, I succeeded only by exerting considerable force. 
After the lapse of scarcely a minute, the rigidity ceased, the fingers were slowly 
extended, but such extreme hyperextension of the finger appeared as a normal person 
could scarcely bring about voluntarily. 

During passive motion, a surprising condition was discovered in the fingers. The 
phalangeal joints were hypermotile, and resembled those of articulated skeletons in 
which the joints are substituted by rubber bands. It was also noted that the finger 
movements were not always uniform, the unrest being sometimes more marked in one, 
sometimes in two or three fingers, while at times all moved in one direction, sometimes 
in the opposite. Occasionally there was complete extension, at other times hyper- 
extension of the basal phalanges with flexion of the terminal phalanges, sometimes the 
inverse. Now and then the fingers were separated, then adducted — all this with a 
sluggishness which sometimes simulated voluntary movement. These movements have 
been somewhat aptly compared to the motions of the tentacles of a polyp. From time 
to time there were intermissions of varying length, and during these pauses the little 
patient could perform voluntary movements; it was stated that no abnormal move- 
ments occurred at night. 

If the foot was examined while in a position of equino varus, it appeared at first 
to be motionless ; but on prolonged observation, and occurring independently of the will, 
there was slow dorsal and plantar flexion of the toes, occasionally of other joints; these 
movements were less energetic than those in the hand. 

This is the type of movements the symptom-complex which we designate 
"athetosis," and we now ask, How do these abnormal movements due to cere- 
bral disease differ from other similar conditions? 

Among motor irritative phenomena we recognize the following: Hemi- 
tremor, hemi- paralysis, agitans, hemi-chorea, and tic. It is needless to enter 
into a differentio-diagnostic discussion of tic and tremor, for these conditions 
bear no actual resemblance to each other. On the other hand, we must give 
some consideration to the symptom of " choreic contraction" This explana- 
tion is necessary since choreiform movements are not rarely an accompaniment 
of the spastic hemiplegia of infancy. 

A glance at the following table, constructed by v. Monakow after that of 
Greidenberg, will show us the various points in the differential diagnosis of 
hemichorea and hemiathetosis : 

Hemichorea. Hemiathetosis. 

1. Face, trunk and all extremities are at- 1. The forearm and hand (fingers), as 
tacked ; in any case, all extremities. well as the lower leg and foot ( toes ) 

are chiefly or solely attacked ; the 
other parts of the body show slight 
paresis with increased muscular ten- 
sion. 



ATHETOSIS 901 

2. The movements are active and mani- 2. The movements are slow, rhythmical, 

fold, they resemble tremor, are more and hyperextensile ; there is elastic 

marked with intended movements, resistance; the contortions appear to 

and are purposeless. After short be voluntary; the joints are fixed, 

stimulation there is a brief transi- although with varying intensity, 

tory relaxation of the muscles. during the movements. 

3. Contracture is often absent ; frequent- 3. Contracture, i. e., involuntary fixation 

ly the limbs are flaccid. No deform- of the joint, is common, but is sub- 

ities are noted in the joints. ject to continuous change; there are 

deformities in the joints. 

4. Hemianesthesia common. 4. Hemianesthesia rare. 

5. Rest during sleep. 5. Rest during sleep not invariable. 

6. Intended movements and attempts to 6. The movements are limited by the will 

suppress the unrest increase the to only a slight extent. 

tremor. 

If these points are borne in mind the typical case just described must be 
considered as athetosis. But we do not maintain that such a differentiation 
is always possible. Sometimes there are similar conditions which one observer 
regards as athetosis, and another, perhaps, as chorea. Literature furnishes 
examples of cases in which there were choreic movements as well as athetosis, 
also of cases in which athetosis passed into chorea, or vice versa. The transi- 
tions are so gradual that, in the localization theory which will later be de- 
scribed, almost all authors who have written upon this subject fail to regard 
the necropsy findings of these conditions distinctive. 

In the question of localization, another condition has repeatedly been 
studied in disease of the brain in adults; this is manifested by the peculiar 
movements usually seen in one extremity alone, but which by their slowness, 
their awkwardness, and their intermissions, closely resemble athetosis; they 
differ, however, by giving us the impression of an intended movement, by 
being sometimes just as marked (or even more so) in the shoulder- or elbow- 
joint as in the wrist-joint, as well as because there is no hyper-flaccidity of 
the finger-joints. Here we are dealing with a condition midway between 
athetosis and a sort of constrained movement, a variety of constrained move- 
ment which has nothing in common with that occasionally observed in severe 
neuropathic conditions in consequence of constrained ideas. 

This description exhausts the main symptoms of the affection; but we 
must consider some peculiarities not observed in our typical case. An im- 
portant point is the distribution. In some cases other muscle groups are 
affected, most commonly the muscles of the face (although this is rare). 

In well marked cases we observe a continuous, peculiar, sluggish, and 
grotesque muscular contraction of the face, sometimes a tonic rigidity appears 
for a short time and then gradually disappears. In these cases the branches 
of the facial nerve which supply the mouth are chiefly affected; more rarely 
the other branches. Eare as these symptoms are in the well developed forms, 
there is some indication of them in most cases. Then we note a slight con- 
traction, a slight drawing of the muscles to the affected side. The slow 
contraction of the tongue, of the hard palate, or of the platysma is exceed- 
ingly rare. 

So far we have spoken only of unilateral symptoms. We must not, how- 
ever, fail to mention that there are also bilateral cases. In some the extremi- 
ties only are affected, in others also the muscles of the face. I accidentally 



902 ATHETOSIS 

saw such a case in a Greek street beggar in Patras. The symptoms were very 
peculiar. The upper extremities moved continuously, assumed the strangest 
positions, and marked contortions of the face occurred now on one side, now 
on the other, sometimes on both. Occasionally there were brief tonic spasms 
which gave to the face an almost frightful expression. 

PATHOLOGICAL ANATOMY 

In discussing the pathologic anatomy, we find that in many of these cases 
there is a bilateral cerebral affection; some of these belong to the group of 
obscure " idiopathic " athetoses without anatomical finding. 

Among the other symptorns of athetosis, we must mention pain, from 
which some of the patients suffer more or less severely. As a rule, any symp- 
toms may appear which correspond to the underlying condition. For in- 
stance, in infantile athetosis there may be hypertrophy of the muscles, 
paralysis of the various cranial nerves, psychical anomalies, epileptic spasms, 
disturbance of the reflexes, contractures, etc. Naturally these disorders vary 
according to the underlying affection. They differ greatly in adults when 
they are the result of an apoplectic disease of the brain (particularly hemor- 
rhage, softening, embolism) ; they differ also in tumor, in progressive pa- 
ralysis, in meningitis, and in abscess. 

This consideration leads us to the more minute specialization of the con- 
ditions which cause athetosis; and we have repeatedly mentioned that they 
are chiefly associated with the spastic hemiplegia of infancy. Athetosis with 
congenital paralysis may appear immediately after birth; when paralysis de- 
velops subsequently it rarely appears at once, but usually after weeks or 
months, and when the paralysis has partially or almost wholly disappeared, 
when the contracture has become more or less distinct, and when the reflexes 
are increased. In very rare cases athetosis alone, not actual paralysis, can be 
determined. 

PATHOLOGY 

The pathologico-anatomical findings in infantile hemiplegia are only re- 
vealed to us by cases which originated in a distant antecedent period; recent 
cases rarely terminate fatally. In the former we find foci of softening, cysts, 
defects in structure, porencephalia (Kundrat), microgyria, or anomalies in 
the cerebral membranes; in other cases there may be diffuse sclerosis. The 
initial affection may be due to embolism, and sometimes, especially in children 
with fatty degeneration of the arteries, to hemorrhage and thrombosis. We 
have already spoken of encephalitis which is in many cases supposed to be 
the cause. It is probable that pre-natal defects of development play a role 
in the forms acquired in intra-uterine life. 

Athetosis after apoplectic hemiplegia is extremely rare in adults. Here 
the causes are the same as in the sudden hemiplegias of adults: Thrombosis, 
embolism, and, more rarely, a rupture of the vessels due to various etiologic 
causes. 

According to Gowers, hemianesthesia is an accompanying symptom in the 
majority of cases. After the twentieth year, however, this symptom is rarely 
associated with the disease. I have seen it but twice in adult hemiplegia, 



PATHOLOGY 903 

once in the case of a merchant, aged 24, who as a young man used alcohol 
to excess and was syphilitic ; suddenly he became unconscious, and on awaking 
there was a right-sided paralysis which subsequently somewhat improved; 
gradually typical athetosis developed in the right hand. The second case 
was that of a laborer from Jerusalem, aged 24, who suffered from mitral 
insufficiency, and presented a clinical picture similar to that of the preceding 
case; this was apparently due to embolism. 

In the scale of frequency, tumors follow the affections which have been 
described. I possess microscopic preparations of a brain tumor which pro- 
duced similar symptoms. It is true the case was not one of typical athetosis, 
but it had exhibited the previously described peculiar, constrained movements. 
It was the case of a woman, aged 62, who was under my observation in the 
Clinic of Professor Nothnagel from the 3d to the 24th of January, 1903. 

The patient had measles when a child, and in her twenty-fifth year articular rheu- 
matism. In the beginning of November, 1902, she lost appetite, was occasionally 
dyspneic, and obviously became weaker and more debilitated. About the middle of 
December there was a peculiar paresthesia in the right hand, gradually a certain degree 
of unrest became noticeable, and objects fell from her hand; this unrest soon became 
so great that the patient was unable to hold anything still. The right upper and lower 
extremities steadily became weaker. Later the patient suffered from vertigo, which 
confined her to bed. There was no vomiting, no loss of speech nor of consciousness, 
no bladder or rectal disturbances, and no symptoms of alcohol or syphilis. 

When examined upon the 5th of January, the patient seemed to be well informed 
but very talkative, complaining much, and constantly making grimaces. She drew 
up her forehead, blinked her eyes, contorted her mouth, and periodically produced a 
laughing expression, chiefly by overaction of the muscles in the right side of the face. 

The tongue was incessantly moved about. The branch of the right facial supplying 
the mouth was less active than the left. The pupils were of equal size and reacted 
sluggishly; the eye-ground was normal. The speech was somewhat indistinct, and had 
a nasal tone. 

The extremities were freely movable, both actively and passively (particularly upon 
the right side), but were weak. While the left side was absolutely quiet, the right 
upper extremity was in constant motion. All of the large joints were involved in this 
motion. The hand was raised, extended, flexed, or rotated, the movements showing 
no purpose and being rapid, after which the extremity would again become quiet and 
return to its original position. On any attempt to suppress the movements, they were 
increased. Upon intended movement a slight degree of ataxia was noticeable. Sensa- 
tion was normal. Knee-jerk and Achilles tendon reflex were also normal. The gait 
was uncertain, and with the eyes closed she tottered. There was a moderate goiter. 
In the left breast was a tumor the size of a pigeon's egg and painful) on pressure. 
The patient also suffered from arteriosclerosis and bronchitis. There was enlargement 
of the glands in the inguinal region. She rapidly grew weaker, became demented, and 
developed incontinence. The speech w T as low and indistinct. Catarrhal pneumonia set 
in with a gradual rise in temperature, the patient succumbing on January 24th. The 
necropsy, performed by Dr. Stork upon the 25th of January, 1903, revealed the follow- 
ing: Scirrhus carcinoma of the left breast, chronic tuberculosis of the lungs and of 
the glands, diffuse colitis. Many segments of tapeworm. A conglomerated tubercle the 
size of a hazelnut in the left thalamus opticus. The histologic examination, made in 
Professor Obersteiner's Institute, showed that death was actually due to a tubercle 
which largely occupied the lateral portion of the optic thalamus, and compressed an 
area of 8 to 10 mm. in the middle of the peduncular portion of the internal capsule. 
The spinal cord was intact. 

Some years ago I saw a similar case. It was published by Dr. Pineles in his article, 
" The Functions of the Cerebellum." It was the case of a man, aged 60, who suffered 
from headache and moderate vertigo: later his locomotion began to be uncertain; there 
w T ere also peculiar involuntary movements of the left upper extremity. Examination 



904 ATHETOSIS 

revealed slight nystagmus and mild disturbances of coordination in the left extremities, 
but otherwise the motility was intact. " When the patient was asked to extend the 
left arm or to perform any movement which necessitated the spreading of the hand, 
he made movements as if grasping at something, and the fingers were flexed and ex- 
tended in a slow and quite rhythmical way. Occasionally there were movements in the 
elbow- joint as though the patient were reaching for some object. This slowly resulting 
spreading of the fingers closely resembled the play of the muscles in athetosis, and 
could only be controlled by a great effort on the part of the patient. Indications of 
this disturbance of movement were also evident in the left toes." 

After a few weeks' illness the patient died of tuberculosis. The autopsy revealed 
a tubercle the size of a walnut in the left cerebellar hemisphere. On section it was 
evident that the neoplasm exclusively occupied the left half of the cerebellum and the 
middle of the quadrangular lobe. 

While athetosis is relatively frequent in brain tumor, it is extremely rare 
in abscess. A. Berger, whose excellent compilation we shall frequently refer 
to, mentions only a single case of this kind. We note that the symptoms 
may appear in paralysis as well as in exceptional cases of compression of the 
brain. 

In this discussion we have several times casually referred to localization ; 
it is now time to consider this theoretically and practically. The number of 
published necropsies of athetosis and hemichorea is not very large 1 , and among 
these are some which cannot be utilized on account of the multiplicity of the 
foci. In others again the autopsy finding is inconclusive. 

As a clear illustration of a case which fulfilled all requirements we may 
consider the one histologically examined by Berger, and published in the 
Jahrbuch fur Psychiatrie, Bd. XXIII, page 214. The patient was a man, 
aged 61, who was admitted to Xothnagel's Clinic for carcinoma of the 
stomach, and there succumbed. In his third year, apparently after a fright, 
he was stricken with right-sided paralysis; this soon improved, but peculiar 
permanent contractions persisted in the face and right arm, and became 
more marked with the lapse of time. As a matter of fact, in addition to a 
certain debility of the right hand typical athetotic movements appeared in 
the right half of the face as well. The necropsy disclosed a large carcinoma 
of the stomach; in the brain was a finding which I shall briefly sketch. A 
cavity in the posterior portion of the left nucleus lentiformis was almost 
filled with calcified masses about the size of a cherry. Upon histologic exam- 
ination the internal capsule and the other parts of the pyramidal tract were 
seen to be perfectly normal. In reviewing the cases which maintain their 
position notwithstanding the sharpest criticism, we find exclusive foci in the 
thalamus opticus 11 times, also 3 cases (including my case mentioned above 
which has not been previously published) with foci in the thalamus and 
visible destruction of the internal capsule. Capsular foci were twice found, 
in 2 cases the lenticular nucleus was affected without implication of the in- 
ternal capsule, in 3 cases the lenticular nucleus was the seat of the focus and 
the internal and external capsules were implicated, and a circumscribed focus 
in the cortex of the brain was only once found. Furthermore, we have just 
learned from Pineles's case that, under some circumstances, a lesion of the 
cerebellum may produce movements resembling those of athetosis, and this 
author collected several similar cases from literature. A case described by 
Halban and Infeld is very interesting: A girl, aged 15, from her first year 
had shown ophthalmoplegia upon the left side and right-sided hemiplegia 






PATHOLOGY 905 

with spasm and hemichorea. The autopsy revealed a focus in the left teg- 
mentum which had destroyed the greater portion of the red nucleus. 

The results of autopsy still differ so greatly that it is evident the con- 
dition is far from uniform. These diverse results of necropsy have given 
rise to numerous and equally diverse theories, a few of which I shall briefly 
mention. A very peculiar view, hardly accepted in Germany, was promul- 
gated by Charcot and his adherents. They believed that besides, and without 
doubt anterior to, the posterior nerve fibers in the corona radiata which con- 
vey sensory impressions, there is a layer of fiber bundles which possess peculiar 
motor properties, disease of which gives rise to hemichorea. Gowers regards 
disease of the optic thalamus as the cause. Anton believes that certain forms 
of chorea (not all) are to be referred to disease of the lenticular nucleus 
and corpus striatum, by the destruction of which inhibition disappears ; some 
authors (Eulenburg) have suggested theories for its possible development 
from the cerebral cortex. Eisenlohr maintains, on the basis of an autopsy, 
that the spinal cord is responsible for choreic movements. In regard to the 
manner in which foci of the cerebellum produce athetosis, Pineles states the 
following: The cerebellum with its three important communication systems 
is to be included among the subcortical portions of the brain; therefore in 
the innervation of movements it gives off important constituents. Disturb- 
ances of motion occurring in man after disease of the cerebellum are also 
due to the fact that the power to regulate voluntary movements is lost; this, 
under normal circumstances, belongs to the cerebellum, and principally affects 
the hemisphere of the cerebrum upon the other side. 

Later authors, such as Anton, Halban-Infeld, and Berger declare that all 
attempts to localize the phenomena in a circumscribed area are futile. The 
first to express this view were Kahler and Pick, according to whom these 
pathologic movements may be produced in any area as well as in the compact 
pyramidal bundle, v. Monakow believes that abnormal irritative processes 
on the part of the interbrain which are transmitted centripetally to motor 
centers of the cortex are necessary for the development of the condition. 

Berger suggests a hypothesis by which these conditions may be made to 
conform to a uniform principle: If we investigate simple hemiparesis, we find 
that this may occur anywhere in the large motor tract from the cortex to 
the peripheral nerves, but it may also be produced by disease of the cerebellum. 
Is it not conceivable that, like paralysis, disturbances of movement may also 
be produced by the affection of several different cerebral regions? This ap- 
pears to be favored by the fact that foci are found in almost all cerebral 
regions, in the cerebellum, and in the spinal cord. Berger does not state 
whether there is direct irritation of the pyramidal fibers or whether the irrita- 
tion is transmitted by centripetally running fibers. 

Besides the symptoms of athetosis in severe cerebral changes, those forms 
must be discussed in which either (1) no anatomical lesion can be determined 
or (2) in which affections (a) of the spinal cord or (b) of the peripheral 
nerves are the cause. The first group again leads us to the consideration of 
bilateral athetosis; this usually attacks all of the four extremities, it rarely 
spares the face, and it is usually indicated at birth ; later onset is rare. The 
defective intelligence and the dragging, indistinct speech are usually con- 
spicuous. As a rule, rigidity is very marked, paresis is slight. In rare cases 



906 ATHETOSIS 

the musculature of the trunk and pharynx as well as respiration is implicated. 
Hypertrophy of the muscles is said sometimes to occur. The results of 
autopsy are still very scanty and inconclusive. Concerning the point which 
at present interests us it must be emphasized that anatomical findings have 
occasionally been absolutely negative ; for instance, Dejerine and Sollier found 
nothing but an anomaly of the cerebral convolutions which Oppenheim also 
observed. In some cases of unilateral athetosis no lesions were found at the 
necropsy. 

Under some circumstances movements resembling athetosis may be re- 
ferred to spinal diseases; this is shown by a case of Eisenlohr's in which, 
however, the brain was not thoroughly examined. Years ago several authors, 
Eosenbach, Audrey and others, reported similar conditions in tabes. For 
years I have had under my care an official, aged 70, who, while a young man, 
contracted syphilis; in 1899 he was attacked by tearing pains and paresis of 
the loAver extremities. While motion became normal after an energetic in- 
unction treatment, the pains persisted ; gradually moderate ataxia of the legs 
developed and the pupillary and patellar reflexes failed — conditions which did 
not improve under continuous mercurial treatment. In this man, almost 
from the onset of the affection, independent of the will, very slight move- 
ments were produced in the toes particularly upon the left side. Similar 
conditions are said sometimes to occur in myelitis and poliomyelitis. Several 
authors have described this symptom in multiple neuritis. 

The prognosis is grave ; recovery is not to be expected, and there is rarely 
improvement. Electricity, baths, massage and gymnastics are the curative 
agents, and these should be tried whenever possible. 



TETANY OF ADULTS 

By L. v. FRANKL-HOCHWART, Vienna 

HISTORY AND CLASSIFICATION 

The chief symptom of tetany is the appearance of bilateral, tonic, and 
intermittent spasms which are often accompanied by pain; they almost in- 
variably occur during full consciousness, and are first visible in the hands, 
which show the peculiar position assumed by an obstetrician. They often 
affect the lower extremity, occasionally the muscles of the larynx, of the face, 
and of the jaws ; more rarely the muscles of the chest, the abdomen, the neck, 
and the tongue. Now and then there are spasms of the diaphragm and of 
the muscles of the eye; spasms of the bladder and rectum, at least in adults, 
are almost unknown. 

In the sensory sphere we seldom note any disturbance except the above 
mentioned pain; at most it is insignificant. Still more rare is any disorder 
of the organs of special sense. It is an interesting fact that in many cases 
pressure in the region of the brachial plexus between the paroxysms will 
evoke spasms (Trousseau's phenomenon). The electric, and especially the 
galvanic, contractility of certain motor nerves is invariably increased (phe- 
nomenon of Erb and Hoffmann), sometimes also of the sensory nerves. We 
often note an excessive, mechanical excitability of certain motor nerves 
(Chvostek's phenomenon), occasionally also of the sensory nerves (Hoff- 
mann's phenomenon). The mind is usually unaffected. Mental disturbance 
is observed only in exceptional cases. Among the relatively rare phenomena, 
trophic secretory anomalies deserve mention : excessive perspiration, reddening 
of the skin, swelling of the joints, slight edema, loss of hair and of the nails, 
discoloration of the skin, the appearance of urticaria, herpes, polyuria, and 
glycosuria. The pulse is often accelerated. In some forms of spasm there 
is dyspnea ; in others an occasional rise of temperature is noticed. The tendon 
reflexes may be increased; occasionally they are absent. Epileptic attacks not 
infrequently occur between the periods of contraction. 

For the first clear description of the disease, as sketched above, we are 
indebted to Steinheim of Altona (1830) and to the French author, Dance. 
Soon after this the French (Constant, Murdoch, and others) made a study 
of tetany in children. Following this, Trousseau made a comprehensive re- 
search into tetany in adults. It was only in the early 70's that the German 
clinicians (for example, Kussmaul and Riegel) began to master the problem. 
Then came Erb's pioneer work on electrical contractility, and soon afterward 
other treatises by his pupils (Hoffmann, Fleiner, etc.). The works of 
Schultze should also here be mentioned. In 1878 Chvostek of Vienna de- 

907 



908 



TETANY OF ADULTS 



scribed the phenomenon which bears his name. From that time on Viennese 
physicians closely studied this remarkable affection. In Billroth's Clinic, 
Nathan Weiss made the memorable discovery of tetania strumipriva, a dis- 
covery which gave a renewed impetus to the far reaching experiments on the 
thyreoid gland. This was followed by my detailed report of the most im- 
portant phenomena, the appearance of psychoses in tetany and the epidemico- 
endemic foundation of the disease. Prior to my investigations, N. Weiss and 
v. Jaksch had made a notable report on tetany in working men; subsequently 
Chvostek, Jr., and H. Schlesinger investigated its symptomatology. After 
the treatises of Abercrombie and Babinski, a voluminous literature on tetany 
in children appeared. I refer to the researches of Ganghofner, Escherich, 
Hochsinger, Loos, Kassowitz, Mann, and others. At present we shall limit 
our discussion to the main theme — tetany in adults. According to its occur- 
rence we classify it as follows : 

(1) Tetany in healthy young working men. This is most apt to appear 
at certain seasons, and in certain cities (Vienna and Heidelberg) ; it chiefly 
attacks young men, and preponderates in certain trades (cobblers and tailors). 

(2) Tetany in gastrointestinal affections. 

(3) Tetany in acute infectious diseases (enteric fever, influenza, angina 
tonsillaris, etc.). 

(4) The rare cases of tetany caused by poisoning from certain substances 
(chloroform, morphin, ergo tin, and lead). 

(5) Tetany of the puerperium (during pregnancy, labor, and lactation). 

(6) Tetany following loss or absence of the thyreoid gland. 

(7) Tetany in connection with other nervous diseases (Graves' disease, 
tumor, syringomyelia, etc.). 

Group 1 contains the most common cases of tetany in working men. Even 
the early French authors had noticed the prevalence at certain times of tetany 
in children. Subsequently, like observations were made concerning adults 
(Delpech, Verdier, Aran, and Eabaud). The observations of Weiss and v. 
Jaksch gave us the first intimation that this applied also to the working men 
of Vienna. The first fairly comprehensive statistics were compiled by me 
from the admission cards of the General Hospital of Vienna, and are here- 
with presented. The 368 cases admitted were distributed throughout the year 
as follows : 



Month. No. of Cases. 

January 45 

February 56 

March..* 87 

April 75 

May 33 

June 19 



Month. No. of Cases. 

July 5 

August 5 

September 4 

October 5 

November 12 

December 22 



It is evident, and is noted every year, that there are few cases in July, 
August, September, and October. In November there are slightly more. 
The number of cases steadily increases throughout December, January, and 
February until the acme is reached in March and April ; it distinctly declines 
in May, and more markedly in June. In other countries where tetany of 
working men prevails similar observations have been made. My study of 



HISTORY AND CLASSIFICATION 909 

the admissions to the General Hospital also revealed the peculiar fact that 
certain years bring many cases, others but a few. 

Another table gives a compilation of the occupations. Among 399 men 
there were 174 cobblers, 95 tailors, 26 carpenters, 20 locksmiths, and 19 
lathe- workers ; of the remaining 65, 1 to 5 were found in each of various 
other occupations. It is interesting to compare these figures with the per- 
centage of workmen in the total population of Vienna; for instance, in 1894 
the cobblers formed 1.3 per cent, of the whole population, and furnished 
43.6 per cent of the cases of tetany in the Hospital; therefore the cases of 
tetany were out of proportion to the percentage of the total population, being 
41 times as numerous. A similar ratio was observed among the tailors. In 
comparison with this it is noteworthy how very rarely builders were affected 
by the disease ; my statistics show only two masons ; there was but one factory 
hand. These figures will at once strike us as insignificant on comparing them 
with the enormous number of persons engaged in such occupations in a 
metropolis like Vienna. It must also be remarked that tetany is not observed 
among the higher classes of Vienna. 

The persons attacked by the disease are usually only those of a certain 
age. My statistics show that 83 per cent, of the cases were between 16 and 
25, and only about one-eighth of the number had advanced beyond the 25th 
year. 

We must bear in mind an important fact which has already been men- 
tioned, that the malady is by no means equally distributed throughout the 
world; in the cities in which it was formerly rare it is now common, and in 
those in which it once prevailed it is now almost extinct. I have for some 
years devoted considerable attention to this peculiar distribution of the affec- 
tion, both by personal observation when I traveled as well as by questioning 
many foreign colleagues who came to Vienna. 

I have scarcely ever had a case from the country, notwithstanding the fact 
that I have a large number of hospital patients from lower Austria and other 
provinces, and observers in the principal cities of the various provinces have 
reported but little concerning tetany among the laboring classes. To my argu- 
ment the objection has sometimes been raised that tetany is not reported 
outside of Vienna because unrecognized, hence the opinion prevails that the 
disease is rare elsewhere — an objection which is shown to be without founda- 
tion since a number of neurologists have accepted medical chairs in cities of 
the provinces after having had for years the opportunity of studying tetany 
in Vienna, and have thus been enabled to recognize it when observed in the 
cities to which they removed. Outside of Vienna cases have been reported 
by relatively few physicians — by v. Jaksch in Prague, by Kraus, by v. Wagner 
and Anton in Graz, and Mayer in Innsbruck; Schultze, who observed many 
cases in Heidelberg, never saw the disease in Bonn. The rarity of the affec- 
tion in many other German Clinics was also demonstrated; for instance, in 
Berlin, Hanover, Leipsic, and Kiel. The same exemption from the disease 
is noted in France, Italy, Scandinavia, America, and Japan. While such 
cases are rarely observed in Russia, yet actual but mild epidemics have been 
twice reported (Minor, Wermel) ; Vaughen described a limited epidemic 
among Italian laborers in New York. 

In Paris tetany of workmen is a great rarity. In 1891 Charcot told me he 



910 TETANY OF ADULTS 

well remembered seeing a few cases in his youth, but that after about 1860 
he observed none. Is it not remarkable that the Viennese physicians should 
have been so backward in taking up the study of this disease? Skoda knew 
the affection well, one of his clinical lectures on the subject having been pre- 
served. If the cases had at that time been as frequent as they now are, the 
fact would certainly have been commented upon by so prominent a clinician as 
he. I have made many inquiries among Viennese physicians who were attached 
to hospitals during the years between 1860 and 1880: they all assured me that 
at the time mentioned such diseases were exceedingly uncommon. 

We therefore consider that not only the epidemic but also the endemic 
nature of the affection is fully proven, and we will now demonstrate similar 
conditions in the other forms of tetany. A few instances are recorded of 
severe cases of the disease being observed in one and the same family. 

Group 2 includes those cases in which the symptom-complex is noted in 
the course of severe gastrointestinal affections. Most interesting are those 
greatly discussed cases which are combined with gastrectasis — a combination 
which Kussmaul described many years ago. The progress of these cases is 
usually very rapid, and they are often fatal. As a rule the underlying affec- 
tion is severe and the dilatation marked, the cause often being pyloric stenosis, 
occasionally stenosis of the small intestine. From a theoretical standpoint 
it may be confidently asserted that my statement, made in 1890, that such 
cases are most likely to occur during the tetany months has been confirmed. 
Tetany is not invariably produced by severe gastrointestinal affections, as 
has been stated, for a few cases of moderate severity have been reported in 
perityphlitis, in helminthiasis, and in acute gastritis. 

Group 3 includes the cases which occur in the course of infectious diseases. 
Aran described tetany which appeared in enteric fever, and similar cases have 
been frequently reported. Now and then it has been noted in other infectious 
diseases, as in measles, influenza, tonsillitis, croupous pneumonia, and acute 
articular rheumatism. 

Group 4 embraces those cases in which the spasms are produced by a non- 
organic poison introduced into the system from without. The earliest cases 
belonging to this category were probably caused by ergotism, and perhaps, if 
individual observations are reliable, also by alcohol, chloroform, morphin, 
lead, phosphorus, and carbonic oxid. 

Group 5 is more readily understood: it is the tetany of fraternity. Since 
the time of Steinheim and Dance cases of this kind have been known to 
appear after childbirth, and Trousseau has described an actual epidemic in 
nursing women. 

Among 61 cases (49 from literature and 12 of my own) 23 occurred in 
pregnant women, and the majority of these in women who were in good 
health. A few were suffering from maladies not connected with the puer- 
peral state; in others intercurrent affections existed — for example, goiter, 
enteric fever, ergotism, and variola — the relations of which to tetany were 
not without interest. Tetany usually appeared between the sixth and eighth 
months, occasionally between the fourth and fifth. Eelapses in subsequent 
pregnancies are not rare. In 10 cases the disease appeared after parturition; 
generally, however, during lactation, sometimes in the early, sometimes during 
the later, course. 



ETIOLOGY 911 

In 1846 Delpeeh reported two mild epidemics, a fact which is the more 
remarkable because subsequently tetania lactantium absolutely disappeared 
in Paris. In 1856 Verdier expressed his surprise that so many cases occurred 
in winter. Of 52 cases in which I ascertained the date of onset, 39 occurred 
between January and April, and only 13 in the remaining eight months. 
These conditions, therefore, remind us of workman's tetany. Most inter- 
esting of all is the circumstance that, in the pregnant, even partial strumec- 
tomy may produce tetany, whereas under other circumstances the operation 
is rarely followed by this disease. 

We now come to the much discussed Group 6 — tetany with thyreoid in- 
sufficiency. The fact, of scientific value, that acute and fatal tetany often 
follows total extirpation of the thyreoid gland was made known in 1880 by 
Nathan Weiss, of Billroth's Clinic in Vienna. Many surgeons have reported 
similar conclusions; it is true the frequency of these occurrences rapidly de- 
creased, for surgeons learned to appreciate the danger of such an operation. 
Nevertheless isolated cases were still reported, either because total extirpation 
was unavoidable, or because even partial strumectomy was not always devoid 
of consequences. Pineles reported 16 cases of this kind. The fact was noted 
that Vienna furnished many more cases than other cities; the season did not 
appear to exert any special influence. Some cases make it seem likely that 
tetany may also occur in congenital deficiency of the thyreoid gland, but this 
is not yet quite certain. 

To complement the forms of the disease Group 7 may be mentioned ; here 
I include cases the nature of which is still very doubtful, and in which the 
typical attacks of tetany appear in the course of other diseases of the nervous 
system. In a few instances this form of tetany has been very clearly described 
as combined with Graves' disease; and here a thyrogenous development comes 
into question with both affections. The appearance of tetany in spinal disease 
(syringomyelia) as well as in brain tumor is very obscure. 

ETIOLOGY 

In the presentation of the etiology of this disease we must admit that our 
knowledge is very limited. The only form in which we are certain of the 
cause is tetania strumipriva. That the extirpation of goiter is not rarely 
followed by fatal tetany has already been stated. When this fact became 
known years ago, doubts were expressed whether the loss of the thyreoid 
gland could actually cause the disease. Suppuration of the wound, injury 
to the recurrent laryngeal nerve, and similar conditions were thought of, and 
doubts were removed only by resorting to animal experiments. Schiff's re- 
searches which had remained unnoticed were now taken up; he had demon- 
strated that strumectomy would kill a dog, but that death could be averted 
if a thyreoid gland were grafted into the abdomen. Eiselsberg removed the 
thyreoid of a cat, and sewed it into the abdominal cavity; the animal con- 
tinued to live, and only succumbed when the ingrafted organ was subse- 
quently extirpated; v. Wagner- Jauregg pointed out the analogy between ani- 
mal and human tetany, and a number of investigators confirmed the 
foregoing observations. 

Further discussion of these experimental findings is probably to-day un- 
59 



912 TETANY OF ADULTS 

necessary. By "untiring labor, and upon the basis of these studies, as well 
as by researches in myxedema, a number of prominent investigators have 
proven that the thyreoid gland furnishes a secretion which is formed from 
the blood, and in the blood it has an antitoxic effect upon the toxic products 
of metabolism (Ewald). 

The view just described has recently been considerably modified ; it is not 
the absence of the thyreoid gland which produces tetany, but the loss of its 
epithelial bodies. The chief exponent of this theory is F. Pineles, whose rea- 
soning we shall now follow: The auxiliary organs of the thyreoid gland — the 
so-called glandular parathyreoidea? — were first described by the anatomist 
Sandstrom, and Gley and Moussu first studied their functions. But only 
when the internal glandulae thyreoidese contained within the thyreoid gland 
were discovered in 1895 by A. Kohn of Prague was the condition actively 
discussed. Upon the basis of this study Vassale and Generali on the one 
hand, and Pineles on the other, came to the conclusion above expressed. 
Besides the results of the animal experiment, Pineles pointed out that in 
thyreoplasia or congenital myxedema, which forms a definite pathological pic- 
ture (extreme dwarfism, idiocy, and myxedema), the epithelial bodies are always 
intact; tetany has never been observed in such individuals. (See volume 
"Diseases of Metabolism and of the Blood," article "Myxedema/' p. 179.) 

Furthermore, Pineles attempted to prove by the study of thyreoid gland 
operations in adults that tetany shows itself only when the epithelial bodies 
are destroyed. It is certain that the ominous symptom-complex appears 
when, in partial extirpation, the isthmus or the upper portion of the lateral 
lobes has been allowed to remain. If we bear in mind the fact that in man 
the epithelial bodies are located in the lateral lobes of the thyreoid gland; 
that in strumous degeneration of the thyreoid gland the relation of the 
epithelial bodies to the thyreoid is altered ; that in the removal of both lateral 
lobes we must weigh the probable effect of extirpation or lesion of the 
epithelial bodies; and that the epithelial bodies are usually located in the 
lower half of the lateral lobes near the point of entrance of the inferior 
thyreoid artery (even considering the change of position in goiter), the 
view seems very reasonable that the upper parts of the lateral lobes which 
adjoin the point of entrance of the superior thyreoid artery contain no 
epithelial bodies. The greatest danger from removal or injury of the epi- 
thelial bodies therefore exists in those cases of partial strumectomy in which 
the lower parts of the lateral lobes have been extirpated, and the isthmus or 
upper portion of the lateral lobes has been allowed to remain. In fact a 
review of the cases indicates that those patients are most susceptible to tetany 
in whom the lesions of the epithelial bodies are most extensive, or in whom 
these bodies have been extirpated. Pineles attaches value to the fact that 
when extirpation affects the lingual portions tetany does not result. These 
patients probably have no cervical thyreoid; nevertheless they have epithelial 
bodies. 

I mention these recent views on account of their intimate relation to the 
subject under discussion; but further research will be necessary in order to 
decide this question. We notice how many explanations are still offered in 
the discussion of this disease, and in the "other groups" of tetany there is 
probably still less of accurate knowledge. Let us first consider the most 



ETIOLOGY 913 

common form, the epidemico-endemic form of occupation tetany. So many 
remarkable facts have been compiled regarding this variety of the disease 
that it seems as though at any moment the enigma of its nature might be 
solved. Eesearches have been carried on for years, yet neither other observers 
nor I have advanced beyond the theory which I developed in 1890. At that 
time, in quoting a suggestion of v. Jaksch's, I proposed the hypothesis that 
this form of the affection must be produced by some factor which appears 
only at certain seasons and in certain localities. In corroboration of this 
view I urged the following considerations: 1. Its epidemico-endemic appear- 
ance which has been fully described above; 2. The not infrequent onset with 
fever; the course, occasionally with high, occasionally with subnormal, tem- 
perature; 3. The intercurrent acute psychoses, which were first described by 
me, and were afterward repeatedly reported; 4. The occurrence of several 
cases in one family, which has been a few times observed; 5. The statistical 
proof that in some years there are many, in other years but few, cases ; 6. The 
sudden appearance of groups of these cases in places where formerly (perhaps 
also subsequently) the disease was unobserved. Here the decline of the affec- 
tion in Paris is noteworthy, as well as its rarity in Vienna until about 1870. 
The idea has been abandoned that the form of manual labor is of consequence, 
for why should the cobblers of Vienna only be attacked, and not those of 
Paris? Why do we see so few seamstresses with these spasms, and so many 
tailors? Nor can exposure to cold be considered the cause, when in much 
colder countries — for example, Scandinavia and England — occupation tetany 
is rarely seen. 

Valuable reports of thyreoid tetany (Schultze, Hoffmann, and Pineles) have 
led to the question whether the previously mentioned organs (the epithelial 
bodies) here play a role. In favor of this idea it was stated that goiter is 
not rare in Vienna ; in Steiermark and the Tyrol it is much more com- 
mon, yet in these places tetany is particularly rare. In the tetany of mater- 
nity a powerful influence has been ascribed to the thyreoid gland, and the 
increase of goiter observed in pregnancy — even its appearance during this 
state — has been urged to support the view. Here we must call to mind the 
remarkable demonstration of Lange that pregnant cats require more thyreoid 
gland substance to keep them healthy than other cats; if more than four- 
fifths of the thyreoid gland is extirpated they are seized with tetany, which 
improves under the administration of iodothyrin. I must also refer to the 
fact that if partial strumectomy is performed in women during pregnancy 
many of them develop tetany. Although we have attempted to ascribe to the 
thyreoid gland an influence in the tetany of maternity, this organ cannot be 
the actual causative factor. It must be reiterated that only in certain regions, 
and even here only at certain seasons, do these cases arise, and epidemics have 
repeatedly appeared and disappeared suddenly. Occasionally we note that a 
combination of maternity and acute infectious disease precedes the spasms of 
tetany. I believe that maternity is merely a predisposing cause. It often 
forms a basis for the same diseases of the nervous system as do the infectious 
diseases : for chorea, polyneuritis, acute transitory mania, and optic neuritis. 

Only in this way can I regard maternity as predisposing to tetany, only 
this role can I ascribe to the acute infectious diseases. There must be an 
auxiliary agent; this alone would explain why this combination should be 



914 TETANY OF ADULTS 

noted only in definite regions and at certain periods. We do not know why 
tetany appears after the introduction of poisons (morphin, chloroform, etc.) ; 
cases are so rare that no explanation has yet been offered. On the contrary, 
gastrointestinal tetany (which is not so rare) furnishes a fruitful field for 
hypothesis. The best known are those of Fleiner and Albu. Fleiner has 
lately brought forth Kussmaul's theory, in which it is assumed that the 
predisposition to spasm noted in severe gastric affections is caused not only 
by autointoxication and hyponutrition, but chiefly by a great loss of fluid 
which the weakened and parched organism sustains for a short time prior 
to the appearance of the spasmodic attack in consequence of processes gen- 
erated in the stomach. The fluid lost to the organism collects in the stomach, 
where, combined with the gastric contents already present in a state of fer- 
mentation, and the development of gas, it causes an enormous distention of 
the diseased organ. This is distressing but endurable, and in itself does not 
produce spasms, probably because it occurs gradually; if, however, it is sud- 
denly relieved by severe vomiting or the introduction of the stomach-tube, the 
over-distended gastric wall contracts violently and immediately causes spasms. 
Fleiner, therefore, ascribes to these irritations (vomiting and the introduction 
of the stomach-tube) merely a final and causative effect, but does not seem to 
regard them as the actual primary cause (as authors have previously done). 

Another hypothesis originated with Gerhardt, and was concurred in by 
Albu and many others. It is based on the idea of an autointoxication of the 
organism from stagnant, decomposing, and fermenting masses in the dilated 
stomach. 

But we should remember that those who have advanced these theories 
have proposed only hypotheses; none has furnished final proofs. This has 
been unsatisfactory, and the latest authors upon this subject (Eudinger and 
Jonas) have returned to the view which I advanced in 1890, when, in my 
first monograph, I called attention to the fact that the majority of cases of 
tetany occur in the typical " tetany months." It is true I could then compile 
but few cases from literature; but with the appearance of additional reports, 
and the increasing prominence of this factor, the more likely does it appear 
that the changes in the organism due to severe gastric affections form only 
a predisposing basis for infection from without. 

SYMPTOMS 

Having sufficiently discussed the occurrence and etiology of tetany, we 
shall now study the symptoms. Most prominent among these are the follow- 
ing: (1) Tonic, intermittent spasms of the transverse striped muscles; the 
upper extremity is first affected, the fingers being contracted, and reminding 
us of the form which the hand of the obstetrician assumes for introduction 
into the vagina ; the hand often appears as though actuated through the ulnar 
nerve ; now and then other positions are observed. Sometimes there is flexion 
at the wrist-joint, more rarely at the elbow-joint; the shoulder-joint is often 
adducted. In many cases the lower extremity is first attacked, there is plantar 
flexion of the joints of the feet and toes, and occasionally extension at the 
knee. In rare cases there are intercurrent contractures of the muscles of the 
face and those of mastication. The muscles of the chest, of the back, and of 



SYMPTOMS 915 

the abdomen, are now and then implicated, occasionally those of the larynx 
(this is different in children), the diaphragm, the pharynx, and the eyes; 
not infrequently there are spasms of the tongue, particularly on yawning. 
In the adult the transverse striped muscles of the bladder and rectum do 
not appear to be involved. Tonic rigidity is usually symmetrical — rarely uni- 
lateral. The attack may last some hours, or, in rare cases, days. As a rule, 
the causative factor is unknown, but psychical stimulation, rheumatic influ- 
ences, or bodily fatigue, can sometimes be demonstrated. In many cases 
there is true intention spasm — a fact to which Schultze first called attention: 
intended movements are increased to tonic spasm. Slight tremor and mod- 
erate motor weakness are often observed in the extremities of these patients 
as auxiliary phenomena. 

(2) Trousseau's Phenomenon. — Trousseau discovered a method by which, 
even in the period free from attack, we may produce the spasm of tetany, or, if 
already present, may increase it. According to him, we may accomplish this by 
exerting pressure in the affected members, either upon the principal nerve 
trunks or the vessels, in such a way as to arrest the arterial or venous circu- 
lation. 

Trousseau's discovery was soon everywhere confirmed. The test is more 
frequently successful in the upper than in the lower extremity; the phe- 
nomenon is seldom seen except in tetany, but it can be demonstrated in two- 
thirds of the cases of this disease. For decades the actual cause was much 
discussed. While some authors attributed it to compression of the artery 
and consequent anemia, others maintained that the spasm was the result of 
irritation of the nerve from pressure. Years ago I settled this dispute by a 
simple animal experiment: In dogs attacked by tetany after extirpation of 
the thyreoid gland I not infrequently noted Trousseau's sign as well as ex- 
tensor spasms and electric hvperirritability, for pressure in the region of the 
crural artery during the attacks free from spasm will evoke extensor spasms. 
When I laid bare the crural artery and the crural nerve, no matter how strong 
the compression of the vessel, I was unable to elicit spasm, but the slightest 
touch upon the nerve at once produced it. These experiments, which were 
recently repeated by Kashida in Tokio, show most distinctly that the phe- 
nomenon is the result of nerve irritation. This is also favored by the observa- 
tion that unilateral compression will often produce bilateral spasm, and that 
contractions are sometimes caused by pressure at other points in which there 
is no large vessel (for example, upon the ankles) ; thermal irritations may 
produce spasm (Kashida). 

To produce this phenomenon the upper arm should be spanned with the 
hand, and the four fingers should be pressed with considerable force into 
the region of the plexus. Sometimes the spasm appears immediately, at other 
times after one to three minutes; instead of compression, we may constrict 
the arm with a bandage or a piece of rubber tubing. 

In addition, there are cases of " latent tetany " in which, besides Trous- 
seau's phenomenon, we note electric and mechanical hvperirritability without 
spasm. 

(3) Electric Hyperirritability (Erb's phenomenon; Hoffmann's phenome- 
non) of the motor nerves, to which Benedikt and Kussmaul had previously 
called attention, was first accurately studied by Erb. The first galvanometric 



916 TETANY OF ADULTS 

measurement was made by Nathan Weiss. In 1887 I published the first 
thorough study of the condition of the muscles, at that time little understood. 
Simultaneously with these appeared Hoffmann's reports concerning the motor 
and sensory nerves. 

Since then it has been frequently remarked that no other disease so in- 
variably runs its course with marked hyperirritability ; the nerves of the upper 
extremity, especially the ulnar nerves, are the ones which reveal the well 
known phenomenon. It is chiefly galvanic irritability which causes these con- 
spicuous changes. Cathodal closure contraction is often produced by the 
feeblest currents, 0.05-0.1 milliampere; and cathodal closure rapidly induces 
tetany. The anodal contractions are soon evoked. While, in the norm, we 
can never produce anodal closure or anodal opening tetany, no matter what 
the strength of the current employed, in tetany it can be caused even by 
relatively feeble currents; occasionally we observe a cathodal opening con- 
traction which never occurs in the norm — and, in rare cases, even cathodal 
opening tetany. The constancy of this increased irritability to the galvanic 
current is equalled by its inconstancy to the faradic — a peculiarity to which 
I called attention in 1887, and which has since been frequently confirmed. 
In many cases we observe the phenomenon studied by Chvostek, Jr., and 
described by Hoffmann: on galvanic irritation of the sensory and motor 
nerves, even with very feeble currents, the patient has distinct sensations 
which are often radiating; i. e., they produce paresthesia, which follows the 
anatomically familiar distribution. Both Chvostek, Jr., and I reported in- 
creased irritability of the auditory nerve. 

(4) Mechanical Hyperirritability (ChvosteVs phenomenon, Hoffmann's 
phenomenon). — Chvostek, Sr., first pointed out that, in tetany, lightning- 
like contractions can be produced in the face by tapping over the course of 
the facial nerve; sometimes they can be evoked in other motor nerves, but 
not with the same constancy. This sign furnishes a most significant guide. 
In typical cases contraction is caused by a slight tap in front of the ear, and 
occasionally by mild stroking of the cheek (Schultze). In less marked cases, 
contraction of the angle of the mouth and the ala nasi on the same side can 
usually be produced only by a percussion blow below the pons zygomaticus; 
occasionally it will appear only in the angle of the mouth. But it must be 
emphasized that Chvostek's symptom may be absent, particularly in chronic 
tetany; inversely, as I showed in 1886, and as Schlesinger, Schultze and others 
agree, it is by no means pathognomonic; it may sometimes be demonstrated 
for years in normal persons, and more frequently in those who are neuras- 
thenic, hysterical, or epileptic ; nevertheless, in those not suffering from tetany 
it never assumes the marked degree which is common in so many — although 
not in all — of those afflicted with this disease. 

Hoffmann pointed out a similar condition in the sensory nerves: tapping 
upon those areas which in neuralgia often appear as pressure points will not 
infrequently produce a powerful sensation which may radiate along the course 
of the nerves. I have often evoked this sign in patients with tetany — occa- 
sionally also in those who did not suffer from it. 

(5) The Sensorium. — In the malady under consideration consciousness is 
usually retained unless there is a severe, underlying affection; for example, 
severe gastrointestinal disease. 



SYMPTOMS 917 

In 1889, when connected with Meynert's Clinic, I published for the first 
time three cases of acute psychosis in tetany — a condition which is theoretically 
interesting; similar ones were reported by Kraepelin, Hochhaus, Westphal, 
and Hirschel. Besides these severe psychoses we now and then observe a 
transitory state of excitement. 

(6) The organs of special sense are but little affected; spasms in the 
muscles of the eye are occasionally observed, and were closely studied by 
Kunn ; sometimes there is cataract formation. Now and then narrowed pupil 
and dilated pupil have been emphasized. Some cases of pupillary rigidity 
have been reported. Inflammation of the optic nerve has been described as 
an exceedingly rare occurrence. 

Pain is the most prominent sensory disturbance. Although some cases 
run their course without this symptom, nevertheless it frequently accompanies 
the spasm, and often persists longer. Sometimes the pain is of great in- 
tensity, sometimes it is slight — often there is only a more or less unpleasant 
paresthesia. This symptom is by no means rare, and in time we learn to 
recognize a forme fruste of this affection, the only subjective symptom being 
formication. In contrast to the common appearance of the symptoms men- 
tioned is the rarity of objective sensory disturbances; most cases run their 
course without these. If present, there is, as a rule, only slight hypesthesia 
of the finger-tips or the fingers, perhaps of the hand, rarely of the entire upper 
extremity. The large nerve trunks are occasionally sensitive to pressure. 

(?) x\s already stated the temperature is sometimes increased, even dur- 
ing the attacks which are not due to an underlying and acute febrile disease; 
subnormal temperatures also are now and then observed. 

(8) Occasionally there are disturbances of the circulatory and respiratory 
organs which are attributable to muscle spasms. In the forms running an 
afebrile course tachycardia is sometimes noted. 

(9) In adults I have never observed disorders on the part of the urinary 
organs, but a few cases in children have been described. Polyuria and polydip- 
sia, albuminuria and transitory glycosuria are rare occurrences. 

(10) Trophosecretory anomalies are more common. In some cases there 
are outbreaks of sweat and reddening of the skin, as well as slight edema. In 
occupation tetany the patient's face is often slightly swollen. Herpes and 
urticaria have been observed. Alopecia and changes in the nails are more 
interesting, since they reveal a transition into myxedema. Change in the 
nails is a conspicuous symptom, which, however, is seen only in severe and 
chronic cases. Cataract of the lens has been reported by Meinert, Peters, 
"Wettendorfer, and others. Upon prolonged observation a few cases show 
atrophy of individual muscle groups (Hoffmann and Max Weiss). 

(11) In many cases the tendon reflexes are normal; sometimes they are 
increased, but the absence of the patella tendon reflex during the parox} r sm 
has also been observed. Absence of the reflexes may continue after the cessa- 
tion of the paroxysm. 

(12) We not rarely meet tetany patients who suffer from typical epileptic 
spasms; in some cases this is certainly a mere coincidence, but it occurs so 
frequently that we are forced to conclude that it is not always accidental. 
An internal connection is strongly favored by the fact that tetanic and epilep- 
tic spasms have been observed after strumectomy in persons who were 



918 TETANY OF ADULTS 

previously free from nervous disease, and that animal experiment occasionally 
reveals similar sequels. Here it may be emphasized that some authors include 
among the symptoms of tetany the hysterical attacks now and then observed. 

DIAGNOSIS 

The diagnosis of well developed tetany in the adult is extremely easy; 
the characteristic spasms with a free sensorium, the demonstration of Trous- 
seau's sign, and the electric and mechanical hyperirritability remove all doubt. 
As an aid to the diagnosis, we note at certain times and in definite localities 
that special underlying conditions (severe gastrointestinal affections, strumec- 
tomy, maternity) and the peculiarity of the occupation (of cobblers and tail- 
ors, most of whom are still young) are factors. We can, therefore, be brief 
in discussing the differential diagnosis. 

Some decades ago much was written of the differentiation from tetanus, 
but nowadays, when both affections are so thoroughly understood, it is need- 
less to consider this at length: the etiology of tetanus is trauma, a factor 
which plays no role in tetany. In tetanus the muscles of mastication and of 
the nape of the neck are chiefly affected while the muscles of the hand remain 
intact; reflex irritability is greatly increased — a peculiarity not noted in 
tetany — while in the former affection we do not find Trousseau's phenom- 
enon nor electric and mechanical hyperirritability. The differential diagnosis 
from epilepsy will rarely cause perplexity because in this disease symptoms 
appear which are foreign to tetany: the characteristic aura, loss of con- 
sciousness, clonic contractions, incontinence, post-paroxysmal confusion, etc. 
But it must be borne in mind that there are mixed forms of epilepsy and 
tetany. 

There is actually but one disease — hysteria — which causes difficulty in 
diagnosis. Kecent studies have shown that the previously mentioned neurosis, 
in which everything may be simulated, may produce a picture closely resem- 
bling tetany; here I refer to the reports of Blazizek, Schlesinger, Krafft- 
Ebing, Nikolajevic, and Curschmann. The last author has recently devoted 
two very interesting articles to this question. If patients with this affection 
(and they are almost invariably females) suffer from tonic tetanic spasms, 
and if the accompanying triad of phenomena is absent, the differential diag- 
nosis will occasion no perplexity. But in some cases investigators have also 
observed mechanical hyperirritability and Trousseau's phenomenon; the for- 
mer symptom is of limited importance, as it is not rare in other affections, and 
is quite common in hysteria. 

The diagnosis is much more obscure when symptoms resembling Trous- 
seau's sign are present, but these symptoms differ so widely from the genuine 
sign that Krafft-Ebing has referred to them as the " pseudo-Trousseau sign." 
H. Curschmann gives the following points of differentiation: in pseudo- 
tetany this phenomenon does not appear slowly and gradually, but immedi- 
ately at the moment of compression. The nature of the spasm is peculiar; 
for example, there may be a paradoxical and spasmodic position of the fist, 
with tonic contraction of a few of the muscles supplied by the median nerve. 
In most cases suggestive measures (for example, pressure on the ovary) may 
produce the same effect as the above mentioned methods ; but in genuine cases 



PROGNOSIS 919 

we never see electric hyperirritability — a fact which is calculated at once to 
decide any doubtful case. 

In conclusion, a word in regard to the differentiation from myotonia con- 
genita (Thomsen's disease), which must be considered because, as already 
stated, intention spasms now and then occur in tetany, and because, as 
Schultze has recently shown, electric and mechanical reactions of the nerves 
and muscles may occasionally be produced which resemble Thomsen's disease. 
But the facts that in tetany muscular hypertrophy is absent, and that the 
symptoms which remind us of myotonia are very rare, while in myotonia 
spontaneous spasms, Trousseau's phenomenon, and marked electric hyperirri- 
tability of the nerves are wanting, will render the diagnosis easy. Thomsen's 
disease is congenital, and usually progressive. Tetany is acquired late and 
is often curable, and those symptoms which remind us of Thomsen's disease 
often disappear simultaneously with the spasms. 

This discussion of the differential diagnosis is based upon the supposition 
that we are considering a weU-deu eloped form of the affection. There are, 
however, formes frustes. One of these is marked by the appearance of pares- 
thesia, of Trousseau's phenomenon, and of electric and mechanical hyper- 
irritability. 

The conditions are less clear in an affection which I described in the year 
1887 as " tetanoidie." In the spring of this year, in which an uncommonly 
violent epidemic prevailed, I saw four cases — mostly in young workmen — 
with paresthesia of the fingers, and occasionally with severe facial nerve phe- 
nomena, in none of which was there spontaneous spasm, nor could this be 
produced by pressure, and only the appearance of extreme galvanic hyper- 
irritability distinctly revealed the connection; similar cases were subsequently 
reported by many authors. That such cases may not rarely be confounded 
with other conditions — for example, with neurasthenia, hysteria, acropares- 
thesia, occupation neuroses, etc. — is quite conceivable. If an epidemic prevail 
in a city in which there is usually more or less tetany, and a patient come 
to us with the foregoing subjective symptoms, the occupation (that of cobbler 
or tailor) or an underlying condition (maternity, strumeetomy, or severe 
gastrointestinal affection) may alone lead us to suspect tetany. "When this 
suspicion is once awakened, the demonstration of Chvostek's sign and Erb's 
symptom will soon clear the situation. 

PROGNOSIS 

In turning to the prognosis we must first state what we know of the 
duration of tetany. There are cases in which the spasms occur but once, and 
last for only a few hours; usually they appear acutely and have a duration 
of weeks, during which time the attacks do not always occur daily, but are 
noted at intervals. In the stage of onset rigidity lasts merely a few hours, 
subsequently for many hours; in the intermissions the person feels quite well 
or complains merely of lassitude or paresthesia. Although months may pass 
in which the health is perfect, yet relapses occur, frequently during the months 
of February and March ; these cases have been grouped by v. Jaksch under the 
name of " acute relapses." In examining such a patient whom I had asked 
to call upon me after some vears, I was struck with the fact that very few 
60 



920 TETANY OF ADULTS 

of them entirely recover. The majority, even between the paroxysms, are not 
perfectly well. They suffer from paresthesia and a feeling of constriction, 
and not rarely present Chvostek's, Trousseau's, or Erb's symptom; sometimes 
there is cataract, alopecia, or shedding of the nails, as well as general nervous- 
ness. Some show the picture of chronic tetany, which has already been 
described. 

The mildest cases are those which follow acute infectious diseases and 
intoxications. In the tetany of workmen very mild attacks are common, but 
a large contingent of these patients not infrequently have relapses into chronic 
forms. In the tetany of maternity we often observe a fulminant onset, some- 
times the attacks are brief, at other times more prolonged, and parturition 
often terminates the aifection. Belapses are not unusual, especially if the 
woman again becomes pregnant, also after labor, or while nursing her child. 
A chronic condition in this form is not rare. Two fatal cases have been 
reported. 

The tetany of gastrectasis occupies a unique position: here the attacks 
are usually very severe. As a rule, death occurs after a few days, more rarely 
in the course of weeks — perhaps not on account of the tetany, but because of 
the underlying grave condition. Occasionally such patients recover. It has 
already been mentioned that tetany following total strumectomy is often fatal. 
In other cases the attacks are merely transitory, and there is complete restora- 
tion to health. In a third group tetany occurs with myxedematous symptoms, 
or myxedema follows a transitory tetany, and in these cases spasms appear 
only rarely or not at all. 

PATHOLOGY 

Pathologico-anatomically the findings in the adult are not convincing. 
Ignoring the earlier conclusions which were only macroscopically arrived at, 
we must state comprehensively that the few positive findings implicate merely 
the spinal cord. For instance, N. Weiss found changes in the ganglion cells 
of the anterior horn, Langerhans described vascular changes; these semi- 
positive findings are offset by the negative ones of prominent authors. It 
may be mentioned parenthetically that recent investigators have also postu- 
lated spinal cord changes in the tetany of children. The origin of this affec- 
tion in the spinal cord is favored by the symmetrical bilateral appearance of 
tonic spasms and the increase of the reflexes which is occasionally demon- 
strated. Implication of the brain is denoted by the spasm of certain cranial 
nerves (the facial, the motor trigeminal, and those supplying the ocular 
muscles), intercurrent psychosis, and epilepsy. 

TREATMENT 

The prophylaxis of tetany is self-evident. True prophylaxis can come 
into question only in tetania strumipriva ; total extirpation should be avoided, 
and, in accordance with recent advice, the epithelial bodies should not be 
excised. In women who suffer from tetany during pregnancy, conception 
should be avoided; should they again become pregnant, we should advise 
them to remove from the city in which tetany prevails. This advice should 
also be given to young workmen who suffer from the acute relapsing form. 



TREATMENT 921 

Those predisposed to tetany should give careful attention to the digestive 
tract — a rule which also applies to those in whom the affection has already 
appeared. In gastric tetan}^ internal treatment is the most satisfactory. 
Lavage may be cautiously employed, but Fleiner particularly warns us against 
considering any operation. Nursing women should wean the child, and when- 
ever tetany threatens during pregnancy the latter, under all circumstances, 
must be interrupted. For obvious reasons much was expected from thy- 
reoidin; although the results have so far not been satisfactory, yet in doubtful 
cases I should at once make a trial of this form of medication. Our treat- 
ment, therefore, is still symptomatic. In desperate cases morphin cannot be 
avoided; chloral hydrate and the bromids also come into question. The 
salicylates, antipyrin, pyramidon, and similar remedies are useful to relieve 
the pain. Undoubtedly a symptomatic value attaches to electrotherapy: 
galvanization of the medulla spinalis by means of medium currents (4 to 5 
milliamperes) and labile galvanization of the extremities sometimes appear 
to be beneficial. The pain and paresthesia are often mitigated by the faradic 
brush. I have seen relief from protracted warm baths, and now and then 
from packs. Lukewarm or cold douches, bandaging the extremities at night, 
and the application of ice bags to the back, may also be tried. Abandoning 
the occupation sometimes produces a remarkable and very rapid improve- 
ment ; absolute rest in bed is necessary in the serious cases. 



THOMSEN'S DISEASE (MYOTONIA CONGENITA) 

By L. v. FRANKL-HOCHWART, Vienna 

L. K., a servant, aged 28, is the patient whose history will now be detailed. Upon 
the 10th of May, 1904, she was admitted to NothnagePs Clinic, and reported that her 
mother had died in the preceding February from inflammation of the lungs. The 
mother was said to have suffered during childhood from the same disease which this 
patient presented, but her symptoms were much less marked. At fifteen the mother 
was attacked with articular rheumatism, also with a disease of the bones which pro- 
duced curvature of the legs. At thirty she suffered from an attack of apoplexy with 
right-sided paralysis of the extremities, and shortly before her death had a second 
stroke of paralysis affecting the left side. The father of the patient was an alcoholic, 
and was said to suffer from hematemesis. The patient's aunt had epilepsy; the mater- 
nal grandfather was neurotic, but the patient knew nothing definite as to the nature 
of his disease. 

In childhood the patient herself had diphtheria, scarlatina, and a pulmonary in- 
flammation; she did not begin to walk until 4 years of age, and until then was quite 
feeble. Later she developed more rapidly, and while attending school was a com- 
paratively robust child. 

As far back as she could remember she had suffered from muscular stiffness which 
hindered motion, and made her incapable of any work which required skill, such as 
embroidering, sewing, etc. If she grasped an object firmly with her hand she could 
not immediately let go of it, and after doing so the fingers were only slowly extended 
and as if they were flexed by a spasm. If she knelt upon the floor, or sat for some 
time upon a chair, she was scarcely able to rise; her knees would be quite stiff and 
she could not extend them. The legs were like wood, and could be moved only with 
difficulty, so that her first steps were very awkward and uncertain. Gradually the 
gait would become normal, and would continue so provided the patient did not move 
about too long. After walking on an even surface for about half an hour she felt a 
tired sensation which rapidly increased and forced her to sit down in order to avoid 
falling. The ascent of steps was extraordinarily difficult because it was almost impos- 
sible for her to lift up her legs, and in descending she felt uncertain, and was con- 
stantly afraid of pitching forward. During excitement the patient performed all move- 
ments in a perfectly normal manner, and as if she had never been ill. In occasional 
quarrels with her playmates she fell down on the slightest push. 

During cold weather she noted an aggravation of the ailment; she was always 
better in summer than in w 7 inter ; during the menstrual periods and in the mornings 
she was less well than at other times. When she rose early, she could hardly drag 
herself from bed because of the stiffness in her limbs. She was worse at night only 
when she had been very active during the day; otherwise she was comparatively well 
in the evening. During the periodic exacerbations of her illness, speech and mastica- 
tion were occasionally impeded, particularly if her mouth had been for some time 
closed, in which case syllables and words Avere at first enunciated with difficulty, for 
her tongue was as heavy as lead and appeared as if paralyzed; sometimes she was 
obliged to chew very slowly because she had to overcome rigidity of the jaw which 
sometimes continued as long as fifteen minutes. 

The mental power as well as the memory of the patient was good so that, in spite 
of her infirmity, she advanced at school, and helped with the housework at home as 
922 



THOMSEN'S DISEASE 923 

far as she was able. For a few years before coming under observation she had been 
employed in a button factory where easy work was assigned her. She had to regu- 
late the lever of a metal press, but even this simple task was difficult for her to per- 
form as it always required some exertion for her to remove her hand from the machine 
quickly and at the right time, and there was danger of her hand being caught in 
the machinery. 

According to the patient's report, in the course of years her condition gradually 
became worse, that is, the symptoms had of late become more noticeable. She came 
to the hospital because of frequent attacks of syncope and spasms during the preced- 
ing three months. She attributed these to grief over the death of her mother, because, 
in three days after this occurred, she was several times attacked by spasm of the vocal 
cords which lasted several minutes (seven). The throat felt as if suddenly constricted. 
Her face became red, then blue, and speech was impossible. Eight days later, after 
some further excitement, she was attacked by general tremor and extensor spasms. 
While these lasted, the teeth were pressed firmly against each other, but the tongue 
was not bitten ; there was loss of consciousness, but neither vomiting nor incontinence ; 
the attacks lasted five minutes, after which there was great lassitude ; memory re- 
turned after the lapse of half an hour. After that time there were four attacks of 
syncope, always after excitement. Immediately prior to the attack the patient several 
times had a sensation of fulness in the stomach and throat; it appeared to her as if 
bands were rising from the stomach. There was dyspnea, cold perspiration appeared 
on the face and hands, and loss of consciousness soon followed. There was neither 
paralysis nor disturbance of speech after the attacks, there was no derangement of 
the organs of special sense, no sensory disturbance, no pain; exceptionally slight pres- 
sure in the head, vertigo, and palpitation of the heart were noted. The menses 
appeared during her fifteenth year, but nothing anomalous was noted. 

We pass to the examination of the patient. She was of medium size and grace- 
ful, with a moderate development of fat: her skin was pale, but she showed no other 
anomaly. The internal organs were normal. The urine was free from pathologic con- 
stituents; nothing of importance could he detected in the bones. It may he stated 
briefly that the organs of special sense functioned normally, and that no disturbances 
were revealed hy the most minute sensory tests. Mentally the patient was normal, but 
was inclined to slight irritative conditions. She had no attacks of syncope while in 
the hospital. All of the tendon reflexes were normal. Dermographia was noted. We 
now turn to the apparatus which the patient most complained of as being disturbed 
— the muscles. 

The muscles of the nape of the neck, particularly the trapezius and the sterno- 
cleidomastoid, were powerfully developed, and the shoulders were very muscular. Con- 
sidering the graceful structure of the body, the development of the muscles of the 
back was conspicuous: These were by no means too plump, but the contours were 
as prominent as in an athlete under training. The arms also showed unusual devel- 
opment for a woman. The circumference of the upper arms was 27.5 cm. ; the promi- 
nence of the biceps and the deltoid was quite conspicuous. The muscles of the fore- 
arm were just as markedly developed, and the thenar group was extremely prominent. 
The shoulder could be raised and the scapula* approximated very rapidly. The arm 
could be quickly raised to its full height above the shoulder-joint. Testing the motor 
power, it was at once evident that the arm could be depressed without meeting resist- 
ance. Upon repetition the power increased, so that on a fifth or sixth test the arm 
could be depressed only by exerting considerable force. The lateral movement of the 
arm was excellent, the fall being sudden. The elbow-joint could be moved the more 
readily the more it was exercised. If the arm at the elbow-joint was extended to its 
maximum, and the patient was told to flex it, she could do so only very slowly as if 
overcoming strong resistance. Upon repetition of this exercise there was improve- 
ment, so that on the third or fourth attempt flexion could be performed with ease 
when the arm was extended to its maximum. 

If the forearm was flexed to its utmost for some time, and the patient was then 
directed to extend it, this was promptly done and without difficulty. The same result 
attended the movement of the elbow- joint and the wrist- joint. 

The fingers could be well extended, and were rapidly flexed from a position of 
extreme extension. But when flexed to an extreme degree, they could be extended only 



924 THOMSEN'S DISEASE 

slowly and with great effort as if controlled by a spasm. If the patient held an object, 
she could let go of it only slowly and after considerable effort. 

In the vertebral column there were no pathologic peculiarities. 

In the lower extremity the hyper-development of the muscles at once became con- 
spicuous. The vasti and the gastrocnemii were especially prominent, and were very 
hard to the touch. The circumference of the middle of the right thigh was 50, of the 
left 49.5 cm. ; the circumference of the calves bilaterally was 36 cm. The patient could 
quickly rise from a recumbent to a sitting posture, and if she were placed upon the 
floor could easily rise. The lifting of the leg extended at the hip- joint was at first 
very slow but was quicker on repetition, and gradually became normal; the power 
which was slight at first constantly increased; when vigorous movements were in- 
tended, slight spasm appeared; like symptoms occurred on all movements. 

The test of the mechanical irritability by means of the percussion hammer was very 
interesting. Tapping the nerve trunks produced no reaction. But the results were 
very different on making short, sharp taps on the muscles. On the trapezius this soon 
caused a deep groove to form which, three to five seconds after stimulation had ceased, 
slowly and sluggishly disappeared. Similar results were noted in the deltoid, the biceps, 
and in the muscles of the forearm; there was no response in the triceps. This phe- 
nomenon was very conspicuous in the hyper-developed thenar muscles; on tapping 
these the thumb showed opposition, grooves being observed which in disappearing 
produced slight waves. Similar conditions were observed in the rhomboid, the infra- 
spinatus, and in the vasti, and were especially marked in the gastrocnemii. 

In the tongue, which was not hypertrophied, contractions appeared on firm depres- 
sion but there was no rigidity. In the muscle groups innervated by the cranial nerves 
(the muscles of the face, the eyes, and of mastication) no pathologic changes could 
be determined. The pupils functioned normally. Electrical tests revealed the follow- 
ing: Different nerves, such as the crural, the facial, the peroneal, the median, etc., 
could be stimulated both by galvanism and faradism. Minimal contractions were 
everywhere produced by currents corresponding to the normal ; galvanic irritation with 
brief induction quickly produced contraction in the muscles, but this lasted no longer 
than normal. In a few nerves a remarkably prolonged contraction could be produced 
with somewhat stronger currents; for instance, if the ulnar nerve were periodically 
stimulated the hand at once assumed its characteristic position. On opening the inter- 
ruption electrode, the extremity did not at once return to its normal position but for 
some time retained its first position, as if stiffened; the same effect was seen upon gal- 
vanic stimulation, but these tests did not evoke reaction in all nerves. In some of 
the muscles prolonged contractions were produced, and these were quite remarkable. 
On faradic stimulation of the thenar muscles a groove slowly developed which was 
only sluggishly compensated for after withdrawing the stimulation. This groove was 
even more distinct upon galvanic irritation. 

With a current of 5 milliamperes a deep groove was produced in the deltoid; this 
persisted for from five to ten seconds after the removal of the electrode, and disap- 
peared gradually. There was a similar reaction in the trapezius, in the biceps, in the 
extensor carpi radialis, in the antithenar, and in the interossei muscles. Here and 
there an undulating motion was perceptible, but, notwithstanding all my efforts, I was 
unable to produce true rhythmic waves over entire muscle groups. 

When the tongue was stimulated by galvanism the contraction was sluggish, and 
disappeared gradually. Here also I was unable to produce waves. 

Any one who has seen a similar case, or has read of these symptoms, will 
at once understand that we are dealing with a rare case of Thomsen's disease 
(myotonia congenita) ; this was even a greater rarity, for it was noted in a 
woman, whereas the condition is usually seen in men. 

Here we note heredity — the mother appears to have had a similar affec- 
tion. Here was a muscular development decidedly disproportionate with the 
otherwise graceful structure of the body; the history and the statements of 
the patient make it plain that it was difficult for her to produce movements, 
and the rigidity of the movements on sudden intention was demonstrated; 



ETIOLOGY 925 

the patient had suffered since childhood from these conditions. We also 
succeeded in evoking the remarkable electrical reactions which are designated 
as myotonic, and this effect of mechanical irritation makes the diagnosis clear 
beyond all doubt. 

I shall now discuss the entire symptom-picture, and will take this oppor- 
tunity to explain other details of the case. 

Our knowledge of the disease dates from Thomsen's noteworthy publica- 
tion in 1876. We find that Bell (1832) and Leyden (1874) had previously 
reported cases which agreed perfectly with the symptom picture. Thomsen 
reported in his article that'he had suffered from the affection, and that sim- 
ilar conditions had been traced in his family through four generations. Soon 
afterward Seeligmiiller, Peters, Bernhardt, Strumpell, Moebius and Westphal 
also reported interesting cases. In 1886 and in 1889 Erb published his funda- 
mental reports, and first demonstrated the electro-diagnostic phenomena of 
this disease, the mechanical irritability of the muscles, and their histologic 
relations, which he had carefully studied. 

Microscopic results of an autopsy were detailed by Dejerine and Sottas, 
and Schiefferdecker and Schultze recently extended these researches; a num- 
ber of authors have complemented the clinical picture, and have especially 
portrayed the myotonic symptom picture in combination with other nervous 
diseases. Briefly sketched, the symptom picture might be described as 
follows : 

Males who are members of families in which the ascendants have suffered 
from similar conditions are most frequently affected; they are usually born 
with the affection; rarely do they acquire it later. The most prominent 
symptom noted is the colossal muscular development which is especially 
marked in the extremities; yet the muscular power is slight. The stiffness 
which appears upon intended movements, and which upon gross movements 
increases to spasm, particularly inconveniences the patient. On mechanical 
irritation, grooves form in the muscles and only slowly disappear. Strong 
electric stimulation with direct and indirect irritation often causes tonic 
evidences of contraction; faradic and galvanic irritation produce grooves in 
the muscles which only slowly disappear. In some cases galvanic waves have 
been observed. 

The symptoms are chiefly noted in the muscles of the extremity, rarely 
in the trunk, and still more rarely in those muscles supplied by the cranial 
nerves. Sensation, the sensory sphere, and the reflexes are not involved. 

ETIOLOGY 

If we attempt to amplify this scheme we must first consider the etiology. 
We know of but one actual factor in this — heredity. There are reports of 
families four generations of whom have suffered from the affection, but the 
disease also attacks persons who do not come from myotonic families. It is 
interesting to note that other nervous affections have appeared in the ascend- 
ants, and sometimes also in the descendants, such as psychosis, neurosis, and 
alcoholism. This will recall the history of our patient. 

We know that the malady is usually congenital; but few cases have been 
reported in whieh it appeared in later youth. We know the prevalence of 



926 THOMSEN'S DISEASE 

the affection in the male; briefly, there is remarkable, perhaps quite uniform, 
material which indicates that we are closely approaching the solution of this 
enigma. In some cases it has been maintained that fear or trauma had an 
etiologic effect. I believe these factors to be rather predisposing than etiologic. 

SYMPTOMS 

Among the conspicuous symptoms is hyper-development of the muscles. 
In our patient this peculiarity was evident, but the case could not be regarded 
as classic. In other cases I have seen over-development of the entire muscu- 
lature. The muscles stood out plump and strong, and reminded us of the 
proportions of the Farnese Herakles. 

The extremities are usually the chief seat of the affection, but the most 
varied locations have been reported. Thus, Oppenheim mentions a case in 
which the muscles of mastication and the muscles of the lower extremities 
were implicated while the arms were exempt. The motor power fails to 
correspond with the mass of the muscles: The patients are often only mod- 
erately strong or may even be regarded as weak. The stiffness of which they 
complain is even more disagreeable than the weakness. This becomes espe- 
cially troublesome when they intend to perform a movement, the patients 
then feeling that they must overcome some resistance, and the muscles actually 
do resist the impulse of the will. If sitting, it is impossible for them to rise, 
if they are standing and desire to walk seconds pass before they can make 
the first step, which is slow and laborious, but after walking for one or two 
minutes movements often become quite normal. One of my patients reported 
that, when climbing a ladder as a school-boy, on the first attempt he always 
lagged behind the other boys. After he overcame this, he could reach the top 
sooner than the others. If, while he was sitting, he was told to rise he could 
do it only slowly and with effort. When he was ordered to step out, it 
required a few seconds for him to take the first step, after which he slowly 
proceeded forward as though dragging heavy chains after him. Gradually 
this condition so improved that after three or four minutes he could run 
with uncommon rapidity, and even succeeded in executing some quite agile 
jumps. 

Besides this condition, which may be designated as intention rigidity, 
actual intention spasms, such as Seeligmiiller described, are observed in 
almost all cases. Movements strongly intended often increase the spasm. If 
such a patient closes his hand tightly, and then tries to open it, this is abso- 
lutely impossible; after prolonged effort he succeeds in opening it slightly, 
then gradually more, but only spasmodically. Several minutes may pass be- 
fore he can again extend his hand; similar conditions are usually observed 
in the lower extremity, often in the muscles of the trunk and the nape of the 
neck, in some cases also in the diaphragm. The muscles supplied by the 
cranial nerves are rarely implicated; probably the tongue is most frequently 
affected; the patients often complain of difficulty in moving this organ, and 
this symptom may very often — but by no means always — be the chief cause 
of the difficulty in speech. Some observers have noted myotonic disturbances 
of the muscles of the eye; for example, with strong intention a hindrance to 
the action of the bulbi. Oppenheim reported an interesting case of this in 



SYMPTOMS 927 

one of his patients in whom the myotonic disturbance was distinct only in 
the orbicularis palpebrarum, while the other symptoms could be demonstrated 
in the remaining muscles of the eye. In isolated cases we also note disturb- 
ances in the muscles of mastication and of the face. Severe spasm is pro- 
duced not only by marked intention; sometimes severe shock, such as a fall, 
may cause rigidity. Often such persons are for several minutes unable to 
rise, and the body is as stiff as if it were made of wood. 

Excitement often decidedly increases the symptoms. In the morning and 
when the patients are alone, they are able to walk quite well and to work, 
but movements are decidedly hindered when they are under observation or 
are excited. As school-children they suffer greatly, also as young men during 
military service. Mental stimulation which is not exciting and pleasant 
influences often lessen the disturbance. In contrast with this, in our patient, 
excitement often had a beneficial effect. Hard physical labor, general somatic 
affections, the immoderate use of alcohol, are all deleterious factors. Cold 
has a particularly unfavorable effect, and in some cases this is the only factor 
which causes any disturbance. 

Jensen made minute myographic studies into the nature of the muscular 
contraction, and determined that the first contraction of the myotonic muscle 
consumes a much longer time than normally, and that it is chiefly the coor- 
dinates of the decrescents which appear to be so markedly slowed. Accord- 
ingly the myotonic muscle very slowly relaxes, the process consuming more 
than 8 seconds, while the normal decrescent requires but about 0.2 of a second. 

The curves show that it is the terminal portion of the process of relaxa- 
tion which is chiefly slowed, this latter portion usually but not always ap- 
pearing to be quite sharply defined in comparison with the first, which, how- 
ever, also shows a distinctly abnormal slowing. These abnormalities in the 
course of the myotonic curves gradually lessen, and after about ten contrac- 
tions there is a return to a normal condition. The reaction of the motor 
nerves and muscles to mechanical and electric stimulation has been described 
in the history of our case. We concur in the classical description of Erb. 
We were unable to demonstrate the waves noted by this author, but this 
symptom appears to be quite rare in Thomsen's disease, for later investigators 
were often unable to demonstrate it. 

The mechanical irritability of the muscle is never increased, the faradic 
resistance to minimal contractions is normal, and no prolongation appears. 
With stronger and longer continued currents there is a prolongation, but brief 
stimulations do not produce it. Galvanic currents applied for a short time 
elicit prompt reaction from the nerve but no prolongation; labile currents 
undoubtedly evoke prolonged tonic contractions. There are no peculiarities 
in the form of the contraction. 

In the muscles after mechanical stimulation Erb describes contractions 
similar to those we have mentioned. " Even the pressure of the tip of the 
finger upon the muscle, and still more so tapping with the percussion ham- 
mer, will produce sluggish tonic contractions in the muscle bundle, and, 
according to the position and size of the muscle, these appear as deep grooves, 
furrows, or rolls. The contractions are decidedly prolonged, and, according 
to the strength of the stimulation and the tendency of the muscle, last from 
5 to 30 seconds." 



928 THOMSEN'S DISEASE 

All muscles respond readily to faradism; with brief currents which pro- 
duce minimal contraction, or with a single opening contraction, there is no 
prolongation; with somewhat stronger currents the contractions may last for 
20 seconds. In some cases, when individual muscles are included between the 
two electrodes, a peculiar, irregular wave of the entire muscle mass may now 
and then be observed (Petrone, Bernhardt, Erb). Direct galvanic contraction 
is occasionally increased. The anode is said to act stronger than the cathode. 
In our case the latter symptoms could not be determined; the contractions 
were peculiarly sluggish. This qualitative peculiarity was only manifest 
after strong currents, never after weaker ones. If the intensity was increased, 
a depression slowly appeared, remained for from 5 to 10 seconds, and dis- 
appeared gradually and sluggishly. 

The phenomenon of rhythmic wave-like contractions with a stabile cur- 
rent (which could not be demonstrated in our case) is, according to Erb, 
best evoked if the electrode is not placed directly upon the muscle but upon 
the end of the tendon, or somewhat deeper. If the current is sufficiently 
intense, contraction first occurs, and is succeeded by an irregular, undulating 
movement of the muscle, and this soon merges into an ordered, regular wave 
movement. More or less extended waves of contraction follow more or less 
rapidly : They begin at the cathode and move toward the anode. The electric 
phenomena are sometimes labile, but are subject to variations. 

This exhausts the list of the principal symptoms. It should, however, 
be mentioned that the tendon reflexes are occasionally diminished, sometimes 
increased. The sensory functions and those of special sense are normal. 
Ataxia has not been described. There is never marked mental deterioration, 
but the patients have a peculiarly anxious and shy expression. They are 
ashamed of their affection, never speak of it, and, owing to this peculiarity, 
often do not consult a physician until after the lapse of years. 

It is readily understood that every possible nervous condition may be 
combined in these individuals who belong to neurotic families. A number of 
hysterical peculiarities were manifest in our patient. 

It is interesting to note that bladder and rectal disturbances are never 
mentioned, although the external sphincters contain transverse-striped mus- 
cles, and the implication of almost all other transverse muscle groups has 
been reported. This intactness of the external sphincters is conspicuous also 
in myasthenia and in the tetany of adults, which reminds us of the peculiar 
fact, also referred to by Oppenheim, that these muscles often have the phys- 
iological action of smooth muscles and even absolutely resist curare (as A. 
Frohlich and I first pointed out). 

A number of other nervous symptoms may be due to the fact that the 
affection is combined with various organic nervous diseases. Chief among 
these is muscle atrophy. As Hoffmann strongly maintains, this affection 
seems to undergo a direct change into muscle atrophy. In his opinion, this 
is favored by the fact that in a few cases myotonia preceded the muscular 
atrophy, that in the case described by Nogues et Sirol the patient's father 
suffered from myotonia and his son suffered from myotonia and muscular 
atrophy, and that the three children reported by Pelizaeus also had myotonia 
but did not all have muscular atrophy. On the other hand it is not clear 
that muscular atrophy always precedes myotonia, but in all cases that have 



PATHOLOGIC ANATOMY 929 

come under observation myotonia was found at the first professional examina- 
tion, also muscular atrophy, although usually as a forme fruste, i. e., to a 
mild extent and not noticeable to the patient. It was determined not only 
in the atrophied muscle alone, although most distinct in this, but was widely 
distributed in muscles of great volume and good power. Finally, in all the 
cases except Hoffmann's the localization of the atrophy differed more or less, 
it neither corresponded to a conspicuous spinal type nor did it belong to the 
well known myopathic varieties. Other isolated cases (Dana, Deleage, Bett- 
mann, Hoffmann) seem to show that muscular atrophy accidentally occurred 
in patients with myotonia. We shall revert to these conditions in the differ- 
ential diagnosis, and shall then more minutely discuss the question of tetany. 

Combinations of this affection with tabes have been described by Hoff- 
mann and Xalabandoff — but these may have been due to accidental conditions. 
Kaiser reports a peculiar case which shows a combination of myotonia and 
athetosis. 

Oppenheim once saw the disease combined with a congenital muscular 
defect, at another time with a congenital defect in the formation of the 
fingers. 

PATHOLOGIC ANATOMY 

Pathologic anatomy indicates that we are dealing with a primary dis- 
ease of the muscles : that is, the only autopsy, which was in the celebrated 
case reported by Dejerine and Sottas, gave entirely negative results in so 
far as the nervous system was concerned. 

The first investigators also found negative conditions in the muscles, but 
Erb succeeded in demonstrating hypertrophy ; a number of other authors 
confirmed this remarkable discovery, and contributed additional details, par- 
ticularly Fischer, Dejerine and Sottas, Martius and Hansemann. The latest 
and most comprehensive study we owe to Schiefferdecker and Schultze. The 
foundation of all these cases is the following: Hypertrophy of the primitive 
fibers; increase of the sarcolemma nuclei. As to the most recent methods of 
investigation, a quotation from Sehiefferdeekers report will best present these. 
Examination of the deltoid muscle from a person attacked by myotonia 
showed a marked distribution of the fibers; the sarcoplasma presented an 
extremely characteristic and remarkable change in that, after fixing in formol 
(Jores), it was seen to be more or less extensively permeated by small and 
peculiar nodules which have thus far been found in no other muscle, either 
of man or animals. This granular condition was most likely the expression 
of a specific disease of the sarcoplasma; the number of nuclei was much 
above the normal (128 to 290). The volume of the nucleus had been de- 
cidedly increased without its composition undergoing change; it is important 
to note that the bulk of the nucleus had remained unaltered. This observa- 
tion corresponds with that made by Dejerine and Sottas that the first phe- 
nomenon of myotonia congenita is the hyperplasia of the nuclei, which is 
naturally attributed to disease of the sarcoplasma. The fibrillaB of this muscle 
show peculiar changes in which apparently the transverse fibers are chiefly 
implicated, but which probably depend upon disease of all the fibrillae, and 
the cause of which is most likely to be found in disease of the sarcoplasma. 
This change in the fibrillae seems to start at the margins of the fiber and 



930 THOMSEN'S DISEASE 

gradually proceeds toward the center, indicating a change in nutrition which 
proceeds from the periphery; it does not favor the supposition that, perhaps 
as the result of marked thickening of the fiber, the nutrition no longer suffices, 
since, otherwise, the first changes in the fibrillae should take place in the 
center of the fiber. In regard to the clinical observation that myotonia 
undergoes a transition into progressive muscular atrophy, Frobmann makes 
the interesting report that he succeeded in demonstrating numerous atrophic 
fibers between the hypertrophic ones. 

The nature of the disease is still obscure. Erb ascribed it to an anatomical 
(histologico-chemical) change in the transverse striped muscles. This did 
not solve the question whether we are dealing with pure myopathy or with a 
muscular change due to nervous trophic influences and disturbances. In 
favor of the first view is the negative necropsy finding in the nervous system, 
such as Dejerine and Sottas found nine years after the publication of Erb's 
book. As favoring the possibility of nervous influences the following might 
be adduced: 1. That there are no spasms in the primary muscular affection; 
2. That those affected often belong to families in which degenerative nervous 
diseases prevail, the patients themselves suffering from all varieties of nervous 
affections (like the patient whose history I quoted). 

A few authors attempted to solve the enigma by attributing the disease 
to an anomaly of metabolism. Moltschanow showed that in his case the 
amount of urea in the urine was decreased as well as chlorin and uric acid. 
Wersiloff found a great diminution of creatinin. Bechterew demonstrated in 
one case a decrease of urea, phosphoric acid and the chlorids, while the quan- 
tity of uric acid was at one time decreased, at another time conspicuously 
increased. The latter author formulated the hypothesis that myotonia is a 
peculiar derangement of the metabolism in which toxic products are set free 
in the organism and have a poisonous effect upon the muscular tissue. 

DIAGNOSIS 

In typical cases the diagnosis is very easy. The heredity, the congenital 
nature of the affection, the hypertrophy of the muscles, the peculiar electro- 
mechanical reactions, the intention spasms, are all so characteristic that doubt 
can scarcely arise. It must be mentioned, however, that the affection has 
sometimes developed only in late youth, and cases have been observed in 
which, although there was no muscular hypertrophy, all the other symptoms 
of the clinical picture could be demonstrated. The diagnosis becomes per- 
plexing when myotonic symptoms are combined with muscular atrophy. 

In 1888, in the Zeitschrift fiir Tclinische Medicin I reported for the first 
time a typical muscular atrophy with intention spasm, and periodical, true 
muscular hypertrophy (which could be miscroscopically demonstrated), also 
a sort of myotonic reaction; the spasms subsequently disappeared. When, 
years later, H. Schlesinger saw the case partial sensory paralyses had ap- 
peared ; this author mentions in his monograph, " Syringomyelia," that in 
this disease myotonic symptoms not rarely develop in individual muscle 
groups, and he refers to a myotonia syringomyelitica which should be differ- 
entiated from true myotonia. A. Fuchs recently demonstrated a case with 
myotonic phenomena in a symptom-picture which revealed the characteristics 



DIAGNOSIS 931 

of amyotrophic lateral sclerosis. When we recall that Hoffmann accepts the 
view of the transition of Thomsen's disease into muscular atrophy, and if we 
bear in mind that localized muscular atrophy may be combined with my- 
otonia, we can readily understand that there may be transitional stages 
between these symptoms; one investigator may in one case make a diagnosis 
of myotonia undergoing transition into muscular atrophy, while another in- 
vestigator may diagnosticate a isotonic symptom-complex combined with 
muscular atrophy. 

We have recently been considering another extremely interesting com- 
bination. This is tetany with myotonic symptoms. Schultze was the first 
to call attention to this peculiar association, later Kasparek, Bettmann, Hoff- 
mann, v. Yoss and I; recently the subject has been minutely studied by 
Schultze. In these cases we refer merely to myotonic symptoms in tetany, 
not, however, to a combination of the latter affection with Thomsen's disease, 
because neither clinical nor histologic investigation has ever revealed muscular 
hypertrophy. Tetany has been ascribed to various conditions. We know of 
cases which followed extirpation of goiter, also of gastrointestinal tetany, and 
the so-called idiopathic workman's tetany. Xotwithstanding the fact that I 
saw many cases of tetany in Vienna, the symptoms described were only twice 
observed by me, and were then typical intention spasms. Other authors have 
reported the same. Occasionally there may be some rigidity on an attempt 
at movement. Schultze and others have demonstrated a mechanical myotonic 
reaction of individual muscles, particularly of the tongue; in other cases 
electric reactions resembling myotonia were evoked. In proof of the view 
that these are not combined forms of true Thomsens disease is the circum- 
stance that a decrease of the myotonic symptoms is sometimes observed simul- 
taneously with a decrease in the symptom of tetany. 

In a differ entio-diagnostic respect it may be interesting to mention that 
intention spasms (without myotonic reaction) occur also in other diseases 
of the nervous system, usually in severe spinal affections. In these cases the 
spasms not only occurred in the muscles which were actively tense, but were 
also noted in distant groups; similar conditions have occasionally been ob- 
served in hysteria. In a patient convalescing from enteric fever Xothnagel 
once saw general spasms of the flexors and extensors upon flexion of the 
elbow. In examining the patient whose history I have related, spasms were 
sometimes observed not only in the muscles which act simultaneously, but 
also in the antagonists. 

Some vague resemblance to intention spasm is found in spastic occupation 
neuroses, but minute investigation will at once lead to a correct diagnosis. 

In concluding our differentio-diagnostic study, we must describe the con- 
ception of paramyotonia. Eulenburg designated by this name a disease which 
resembles Thomson's disease, being a hereditary or congenital form of " mus- 
cular idiosyncrasy," which has been traced through six generations of one 
family. In the individuals affected, this anomalous action was evident 
throughout the entire muscular system, and produced marked phenomena of 
spasm and of rigidity in individual muscular regions, and in others an in- 
hibition of movement. A spasmodic rigidity followed exposure to cold; in 
some muscles it disappeared rapidly, in others more slowly, to give place 
either to the normal condition or to a paresis-like immobility which persisted 



932 THOMSEN'S DISEASE 

for some time. No myotonic reaction could be demonstrated. Hlavacek re- 
ports a combination of paramyotonia with myotonia. 

As far as life is concerned, the prognosis of the disease — I am now speak- 
ing of typical myotonia — is absolutely favorable. In genuine cases no cure 
has as yet been observed, but remissions and improvements are occasionally 
reported. As a rule, the progress of the disease is not rapid. 

Therapy has as yet yielded no brilliant results. Systematic massage and 
gymnastics appear to form the best mode of treatment. In the patient whose 
history was related the former method had no marked effect. Some authors 
maintain the beneficial effects of hydrotherapy, of spa treatment, of carbonic 
acid springs. Galvanism and faradism have frequently been tried. In regard 
to internal treatment, iron, arsenic, strychnin and sodium iodid may be of use. 



LOCALIZED SPASM 

By E. REMAK, Berlin 

For practical purposes spasms limited to individual muscles or muscle 
groups are best considered in one article, since only the most minute differen- 
tial diagnosis will enable the physician to group the case that he observes under 
one or the other pathologic form. 

Although, in accordance with an old custom, text-books still include limited 
muscular spasms among diseases of the peripheral nerves, it has been for 
some time recognized that they are only rarely due to this cause, and even 
then there must be a reflex stimulation and increased irritability of the ner- 
vous centers and tracts, either of the spinal cord or of the brain. Clinico- 
anatomical experience in organic diseases has led us to some conclusions as 
to the parts of the nervous system implicated in localized spasms, for they 
are accompanying symptoms of such diseases. Among these we rarely find 
neuritis, but frequently meningitis (opisthotonos), among spinal diseases 
those of the anterior gray substance (fibrillary contractions), next of the 
pyramidal lateral column tracts (spasms of spastic spinal paralysis and 
amyotrophic lateral sclerosis), of the posterior columns (athetoid contractions 
of tabes), among cerebral diseases those of the pons (contractures in the 
course of the facial nerves and trismus), and especially of the motor area of 
the cerebral cortex. The experimental and clinical findings of the last three 
decades which indicate the exact localization of the motor centers in the 
anterior central convolution, and their relations to definite muscular regions 
in cortical spasm or so-called Jacksonian (partial) epilepsy, have chiefly 
shown that they may begin and run their course locally, according to their 
point of origin. In these investigations of the point of origin of localized 
spasms in cases in which there was no organic disease, and also concerning 
the various forms of spasm produced, an attempt was made to locate the gan- 
glion centers, etc., and to localize and describe the form of the partial spasm. 
An important guide was the distribution (whether unilateral or bilateral, 
whether throughout one or several nerve tracts), as well as the form of 
the spasm itself. The symptomatologic and minute classification lately pro- 
posed for the differentiation of the muscular spasms distributed throughout 
the body, and for the recognition of several special pathologic forms of quite 
different pathogenesis, namely, of myokymia, myoclonia, myotonia, tetany, 
chorea electrica, chorea rhythmica. and even of tic, is the result. All of 
these affections must, in the individual case, be considered as localized spasm, 
because they occasionally appear as limited spasms and remain so for some 
time, which is also true of the initial stage of tetanus, of some forms of 

933 



934 LOCALIZED SPASM 

tremor, especially of paralysis agitans, and of chorea and athetosis in which 
involuntary movements are occasionally spasmodic without being for this 
reason strictly designated as localized spasms. The etiologic relations of the 
most common general neurosis, hysteria, are sometimes more intimate than 
those of the former neuroses. Aside from the fact that hysteria produces 
spasms and contractures of typically hysteric character, this " grande simula- 
trice " may also resemble other forms of localized spasm. Authors hold dif- 
ferent opinions as to whether some of the general spasm neuroses (myoclonia, 
chorea electrica, chorea rhythmica, and tic) are merely special expressions or 
are forms of hysteria. Lately, however, the psychogenous (mental) origin of 
some localized forms of spasm other than hysterical has been urged by French 
authors, and their strict differentiation as tic from spasms due to organic 
or functional disturbance has been declared to be important, because in these 
psychical treatment alone is said to be effective. 

DIFFERENTIATION OF FORMS 

This review shows how necessary is an exact classification in every case 
of localized spasm — for prognosis as well as for treatment. Only when the 
obvious symptoms of localized spasm are recognized, does the diagnosis begin. 

Muscular spasm may be broadly denned as an involuntary, therefore ap- 
parently spontaneous, muscular contraction which is not due to external, 
mechanical, electric, or thermic stimulation. Muscular spasms, such as inten- 
tion spasm and occupation spasm, may be associated with voluntary move- 
ments. Myotonia is the spasmodic arrest of the voluntary contraction of the 
muscles ( Thomsen's disease ) . 

All muscular contractions do not necessarily lead to a shortening of the 
muscle, and need not even implicate the entire muscle. Distributed in naked 
persons who are cold, localized in progressive muscular atrophy which is of 
spinal origin, and in amyotrophic bulbar paralysis, the fibrillary contractions 
may involve only isolated bundles of muscles. Widely distributed and con- 
tinuous, wave-like, fibrillary, muscular contraction, previously described by 
Kny, was designated by F. Schultze in 1895 as myokymia. Hoffmann (1896) 
and others have observed its local occurrence. 

Spasms are strictly differentiated, according to the rapidity of their course, 
as tonic and clonic. Clonic spasms which last but a moment have been desig- 
nated as lightning-like; for example, those in the chorea electrica of infancy, 
as Henoch called it, and in myoclonia (polyclonia) which are probably iden- 
tical. Clonic contractions have not rarely appeared as fibrillary muscular 
contractions; for example, in so-called idiopathic facial spasm. 

Contractions of restricted distribution, in more or less rapid rhythmical 
sequence, and affecting the antagonistic muscular groups, produce various 
tremors which are not included among muscular spasms; only their most 
severe forms lead to so-called shaking palsy. 

When muscular contractions follow one another in such rapid succession 
that the muscle does not have time to regain its position of rest, these spasms 
are designated as clonic. When the contractions last for a considerable time 
we speak of them as tonic, and, if extremely violent, as tetanic spasms. Lim- 
ited but painful tonic spasms, especially those so common in the calf of the 



DIFFERENTIATION OF FORMS 935 

leg, are designated as cramps. Extensive and violent spasms of several mus- 
cle groups of a member are called convulsions; epileptic or hysterical spasms 
are types of these. 

Permanent, tonic, muscular spasms lead to contracture. In so far as this 
is an accompanying phenomenon of organic diseases (for example, cerebral 
hemiplegia and spastic spinal paralysis), also of hypertonia and increased 
tendon phenomena, we are not here concerned with it, nor with the contrac- 
tures of paralysis agitans, of catalepsy, etc. In a differentio-diagnostic re- 
spect the frequently localized hysterical contractures must be considered. 
Contractures which persist after severe peripheral paralysis, particularly of 
the muscles of the facial nerve which were formerly paralyzed, do not depend 
upon muscular spasm but upon interstitial retraction and induration. But 
to these, clonic contractions may be added. Usually these occur as conjoined 
movements of voluntary or of involuntary muscles, especially in the face and 
in the eyelid. Voluntary movements often produce a local attack of spasm; 
for example, mastication and speaking evoke attacks of idiopathic facial 
spasm. Some forms of spasm are caused only by definite coordinate move- 
ments (writer's cramp, etc.). 

Between clonic and tonic spasms and contractures we observe transi- 
tional stages. Clonic spasms may show frequent alterations, first becoming 
tonic, then clonic. Tonic spasms sometimes lead to contractures. 

None of these forms of spasm produces voluntary movements, but simpler 
ones which differ from the voluntary by being arrhythmic, sometimes rhyth- 
mic, occurring in succession, and often reminding us of reflex contractions. 
Even when peripheral irritation and organic diseases of the reflex tracts can- 
not be determined, we may assume that the localized spasms are due to an 
irritative condition at some point in the reflex arc, but it frequently remains 
doubtful whether the motor nuclei of the spinal cord, of the medulla oblon- 
gata, or of the pons must be considered the reflex center, or whether higher 
cerebral ganglia, even those of the cortex, are implicated. Spasms of cortical 
origin are characterized by marked contractions usually of slow rhythm, 
which repeatedly, often paroxvsmally, pass gradually from one muscle group 
to another, and consist of clonic contractions of synergistic muscles (for 
example, the muscles of the arm, the extensors and flexors of the finger, and 
of the elbow, the abductors and adductors of the shoulder) ; these do not, 
however, give us the impression of being true coordinate movements. As the 
cortical spasms are perhaps due only to irritation of the so-called sensory 
spheres of the cerebral cortex, for which the not uncommon initial pares- 
thesias may be clinically utilized, it is not impossible that the cortical spasms 
are also to be attributed to reflex cause. 

Following the example of the French authors (Gilles de la Tourette, 
Charcot and Guinon, Brissaud and others), for more than twenty years 
mala die des tics or tic convulsif has been differentiated as a spasmodic affec- 
tion which runs its course with apparently voluntary, systematic, coordinate, 
complicated, and often grotesque movements, the contortions appearing in the 
face as grimaces. It is probably quite correct to assume that, perhaps in conse- 
quence of some transitory peripheral irritation, these developed from habit, 
and owing to an absence of inhibition were repeated under constraint (automat- 
ically) ; at all events, they originate from psychomotor coordination centers. 



936 LOCALIZED SPASM 

If these psychogenous spasmodic forms, which may also be local, are 
designated as " tic," the nomenclature at once becomes perplexing, since the 
term " tic convulsif " now means something quite different from what it for- 
merly signified. In contrast to tic douloureux (neuralgia of the fifth nerve) 
we mean by tic convulsif the common, unilateral, idiopathic, facial spasm. 
To prevent confusion Jolly, in 1892, proposed designating this as " tic im- 
pulsif." For the same reason, Marina had previously (1888) proposed that 
we distinguish myospasia impulsiva from myospasia simplex. 

But in tic impulsif, during the periods between the attacks, coordination 
of voluntary movements is as little disturbed as in intermittent localized 
spasms. This enables us to differentiate all of the localized spasms, including 
the tics, and is in contrast to chorea, in most of the forms of which chorei- 
form unrest (folie musculaire) persists in the periods between the attacks 
and disturbs the coordination of voluntary movement to a still greater extent. 
This is also true of posthemiplegic chorea occurring in organic hemiplegias 
and diplegias, especially of that subdivision described by Hammond in 1871 
as athetosis, which is characterized by continuous motility, in the main in- 
creased by voluntary movements, and usually located in the ends of the 
extremities, especially the fingers. Idiopathic bilateral athetosis also becomes 
especially noticeable upon voluntary movement. 

If neither chorea nor athetosis can be grouped with localized spasms, 
nevertheless these affections may be combined. In 1898 F. Schultze showed 
that contractions and lightning-like spasms of the individual muscles (mono- 
clonia) are observed in chorea as well as combined with choreic movements. 
In 1888 I reported the case of a man, aged 39, who had suffered from a left- 
sided hemiplegia ever since he was a child of eighteen months, and who, be- 
sides a left-sided, posthemiplegic hemiathetosis, was also afflicted with persistent 
rhythmic, clonic contractions of the left platysma myoides occurring from 
96 to 120. times per minute. 

To the above rule that muscular unrest in chorea is increased by voluntary 
movement, progressive (and usually hereditary) Huntingdon's chorea is an 
exception. In this affection the choreic movements in the limbs are volun- 
tary, and sometimes cease, that is, are inhibited, by voluntary movement, 
which may cause a superficial resemblance to tic impulsif. Since we are in 
this malady invariably dealing with a general disturbance, a neurosis, there 
is no necessity for a minute description. 

On the other hand, chorea rhythmica must be grouped with localized 
spasms. French authors especially, perhaps erroneously, have designated as 
chorea rhythmica the rhythmical, forcible, and usually bilateral contractions 
of synergistic muscle groups. In this affection, probably because of hysteria, 
coordination of voluntary movements is quite undisturbed during the periods 
between the attacks. This is also true of Henoch's chorea electrica, the iden- 
tity of which with polyclonia (paramyoclonus) was previously stated to be 
wry likely, as well as that it may occur locally. 

SYMPTOMATOLOGY 

In most forms of localized spasm there are no pathognomonic symptoms 
except spasm. Aside from the local contractions reflexly due to neuralgia, 



SYMPTOMATOLOGY 937 

even pain is not usually an accompanying symptom, and it is prominent only 
in the well-known cramps in the calves, some forms of myokymia, and in 
attacks of tetanus and tetany. Localized clonic, and even tonic, spasms and 
contractures are generally painless ; but the feeling of contraction may become 
annoying. 

Spasms lasting for years, for example, of the facial nerve, may be un- 
accompanied by paralysis or muscular atrophy. But after long-continued 
and violent spasms, especially of the muscles at the nape of the mck and in 
other parts of the neck (spastic torticollis) there may be slight hypertrophy 
of the same muscles, especially of the sternocleidosmastoid and trapezius. 
A transitory paralysis or paresis after localized spasmodic attacks is character- 
istic of cortical spasms, and on repeated attacks cerebral monoparesis or 
monoplegia may result. Quite exceptionally, especially when peripheral 
cranial nerves have been subjected to pressure from basal tumors, it has 
been noted that peripheral paralysis, for example, of the facial, is preceded 
by the irritative symptom of localized contractions. 

A typical combination of spasm (contracture) and paralysis of periph- 
eral nature is shown by Bose's head tetanus which follows an infection of 
wounds of the face by tetanus bacilli, and first appears as trismus with uni- 
lateral paralysis in the region of the facial nerve (of the same side), and also 
with symptoms of contracture. 

Changes in the mechanical and electrical irritability of nerves and mus- 
cles are rarely evident in localized spasms, even though of long continuance. 
A pathognomonic increase of irritability is noted only in tetany, the attacks 
of which are characterized by an obstetric position of the hands, most marked 
in the region of the ulnar nerve. I refer the reader to the article upon 
tetany as to the increase of mechanical, electrical, and particularly of gal- 
vanic, nerve irritabilitv, even at the onset of the contractions (AnOC and 
CaOC, also AnOT). 

According to all the nerve specialists of Berlin, tetany of adults is a 
rare affection in that vicinity, while in Heidelberg and Vienna, for example, 
it is common. It is therefore remarkable that infantile tetany (in which 
Escherich, Ganghofner, Mann, and Thiemich have also demonstrated an in- 
crease of galvanic nerve irritability which, according to Finkelstein and 
Japha may be associated with or may be without spasm of the glottis) should 
in Berlin so frequently be almost latent, and manifested only by a galvanic 
hvperirritability of the ulnar nerve and relieved by a change of food (discard- 
ing cow's milk). This condition, revealed only by an increase of the electric 
contractility, is unknown in the adult. On the contrary, according to my 
own and foreign researches it may be considered the rule that in localized 
clonic and tonic spasms persisting for years the electric contractility is 
normal; this may be shown, for example, in idiopathic unilateral facial 
spasm, by careful comparison with the healthy side. The same is true of 
so-called occupation spasms, particularly of writer's cramp. Only myotonia, 
which is characterized by the spasmodic continuance of every voluntary move- 
ment (mostly congenital) and increased mechanical irritability .of the muscles 
with groove formation on percussion, as well as the contractions which per- 
sist with a sluggish course after faradic and galvanic irritation, have been 
shown to be myotonic reactions. 



938 LOCALIZED SPASM 

A diminution of faradic and galvanic irritability as well as DeR of the 
muscles is noted in localized spasms only when they complicate a degenerative 
neuritis or are dependent upon it. I saw this complication in 1889 in a 
case of occupation neuritis with milker's spasm, and I have mentioned a sim- 
ilar case of traumatic neuritis in my work on "Neuritis" (p. 94 et seq.). 
This decrease in contractility and the spontaneous contractions or constrained 
movements which appear with contracture in cases of peripheral facial paral- 
ysis that are not yet cured are typical instances of neuritic and muscular 
degeneration and regeneration. The same is true of the athetoid spontaneous 
contractions of polyneuritis described by Lowenfeld, E. Eemak, Korsakow, 
and others, which I have explicitly described in my book (p. 380 et seq.). 

When sensory disturbances of the shin or of the articular sense are per- 
ceptible with or after localized spasm, these do not depend upon the spasm, 
but upon the cause of the affection. If they follow cortical spasms, for 
instance, they may be due to organic disease. 

When associated with neuritic contractions and spasms, they may be per- 
ceived in the diseased nerve areas, as in my case of milker's spasm. The 
majority of hysterical spasms and contractures run their course with sensory 
disturbances, especially hemianesthesia. 

In localized spasm reflex irritability is often normal. It is sometimes 
so far altered that spasms may be readily evoked at definite pressure points. 
Occasionally, however, they may be prevented by this means, as in some forms 
of facial spasm known since the time of v. Graefe and E. Eemak. In hyster- 
ical spasms these pressure points are also detected in the area of the spasms 
as well as in remote regions, for example, in the so-called " ovarie." Dis- 
tinctly abnormal cutaneous reflexes, such as Babinski's toe reflex, may be 
recognized in spasms of the lower extremity which depend upon organic 
foundation (disease of the lateral columns). 

In localized spasms the tendon reflexes are by no means invariably altered. 
Sometimes they are increased, especially in myoclonia, tic, and hysteria. 
Unilateral increase or decrease of the tendon phenomena on the same side 
as localized spasm almost always favors an organic disease. After cortical 
attacks a unilateral increase of the tendon reflexes, especially the foot phe- 
nomenon (dorsal clonus), may denote the development of monoparesis or 
hemiparesis. 

ETIOLOGY 

From this description it becomes evident that there is no uniform etiology 
of localized spasm. It is clear, however, that if in the so-called idiopathic 
forms we exclude a peripheral irritative factor or an organic nervous disease, 
we may frequently determine a certain spasmophilic, neuropathic predisposi- 
tion. Not rarely nervous headache and severe hemicrania have preceded; 
emotion, care, and sorrow are sometimes incidental causes. Over-exertion 
and an improper method of carrying on their work will often lead to occu- 
pation spasms. The family history of patients suffering from myoclonia and 
tic frequently indicates a neuropathic predisposition. 



TREATMENT 939 



COURSE 



The course of localized spasm is far less readily outlined than that of 
peripheral paralysis; when definitely localized a further distribution is likely. 
For instance, if there is localized spasm with succeeding paresis, during 
a subsequent attack other regions may be involved in a form of cortical 
epilepsy due to focal disease, in encephalitis, in meningitis, or even in a begin- 
ning progressive paralysis. Trismus makes us dread an outbreak of tetanus, 
a tonic spasm of the ulnar nerve one of tetany. Myoclonia which is at first 
localized may develop into polyclonia; a localized unilateral tic may become 
a bilateral or general tic. A hysterical and localized spasm or a contracture, 
even if it ceases, frequently recurs or may implicate other regions. Even 
in the spasmodic attack strictly localized to a nerve region a favorable course 
can be expected only in those rare cases in which peripheral irritation is rec- 
ognized and removed. Usually the spasm becomes chronic with remissions; 
I have known it to remain quiescent for decades. Although we cannot promise 
recovery in such cases, we may comfort the patient with the well-founded 
assurance that the further extension of his distressing affection is impossible. 

TREATMENT 

Provided no organic disease can be detected, the treatment of localized 
spasm must be directed to the cause; if an underlying disease be found, it 
must receive appropriate treatment. If, for example, a cortical disease has 
been diagnosticated, and this, owing to its nature, cannot be relieved by 
internal medication (iodin preparations, etc.), as soon as its exact seat has 
been determined the operation of trephining is indicated. If peripheral irri- 
tation cannot be relieved by absorbents, we must resort to surgical measures; 
for instance, extraction of a tooth, or, in cases of incarceration of sensory 
nerves, the pressure from the cicatrix or callous mass must be relieved by 
neurolysis. On the other hand, stretching of a nerve, particularly of a motor 
nerve, has not fulfilled the hopes at first entertained concerning it. There 
is relief so long as the paralysis lasts, but this often torments the patients 
more than the preceding spasm; when the paralysis disappears the spasm 
usually returns. Therefore, with spasm in a muscular area the function of 
which is not too important resection of the motor nerve is preferable. As, 
however, the motor innervation of spasmodic muscles is frequently supplied 
by several nerve branches, or even by nerve trunks, here also our treatment 
often fails. It is probably for this reason that Kocher and others have been 
so successful, especially in stubborn cases of spasm of the muscles of the nape 
of the neck, their method being the successive severing of the spasmodic 
muscles. 

Before deciding upon operative measures, however, we should try both 
by systematic internal and external treatment to diminish the tendency to 
localized spasm. Among internal remedies the preparations of bromin, 
valerian, belladoma, arsenic, and tincture of gelsemium, have not, as a rule, 
been markedly beneficial. Curare, atropin, antipyrin, etc., have been tried 
subcutaneously. Of external methods electricity, usually the stabile galvanic 
current, on prolonged use has given the most satisfactory results. The best 



940 LOCALIZED SPASM 

method is to apply an anode having a diameter of 10 to 15 centimeters with a 
strength of current of from one to two milliamperes either to the painful pres- 
sure point, to the pressure point which inhibits the contraction, or at the exit 
of the motor nerve. Although success is by no means certain, the frequency 
and intensity of the attacks will often be diminished. Other methods, such 
as applying the induced current to the antagonists not affected by the spasm, 
have fallen into disuse, and should at most only be resorted to for the relief 
of hysterical contractures when the suggestive element in eleetrotherapeutic 
treatment is likely to have an effect. 

Brissaud and his pupils, Meige and Feindel, have proposed a special 
treatment for tic impulsif, namely, psychomotor education, which in the main 
is identical with Oppenheim's inhibitive treatment. It consists of practice in 
maintaining a position of rest; first, the patient is taught by the aid of a 
mirror to keep the affected area immovable, second to practise on command 
and before the mirror slow, correct movements of the muscles involved in 
the tic. 

In the cure of occupation spasms, especially of writer's cramp, practice 
in new coordination movements, the method being alternated with gym- 
nastics, massage, and electrotherapy, has proven most beneficial. At all 
events, these modes of treatment are more rational than hypnotic suggestion, 
success from which has been reported in isolated cases. 

CLINICAL VARIETIES 

In passing to the description of localized spasm in various muscular 
regions, we must refer to our previous description both for their symp- 
tomatologic and pathogenic classification as well as for their treatment. After 
examining the patient, and taking into account according to the rules of 
neuropathologic diagnosis, all the symptoms that have appeared, we must 
first decide the cardinal question, whether or not the spasm is the symptom 
of an organic disease (cerebral, bulbar, spinal, meningitic, or neuritic). 
Next we must consider whether it is reflex or idiopathic; in the latter case, 
whether it is fibrillary, clonic, or tonic, whether it indicates contracture, or 
is limited to a definite nerve region. If these points are decided in the nega- 
tive, we must next ascertain whether tic impulsif is present, also whether the 
localized spasm represents its onset or that of general spasmodic neurosis in 
which hysteria especially plays a part. It is at once obvious that with rare 
exceptions occupation spasms implicate only the upper extremity. 

Group I. — Spasm of the muscles of the face in its various forms is the 
most common of all localized spasms. Among my 244 cases of all kinds of 
localized spasm, in 109, therefore 44.6 per cent., the facial muscles were 
affected. 

(1) Bernhardt in 1902 described as myokymia of the facial nerve a per- 
manent spasm of the contracted left half of the face in a woman aged 27; 
this disappeared within 3 months. Newmark and Vitek have since reported 
analogous cases which were right-sided, and also terminated in recovery. In 
Newmark's case, however, the symptoms of multiple sclerosis soon afterward 
appeared, and this proved fatal. In 1886, among the early symptoms of 
multiple sclerosis in an engineer, then 21 years old, who was under my ob- 



CLINICAL VARIETIES 941 

servation for ten years, I saw various muscular contractions in the forehead, 
eyelid, cheek, and lips of the left side without any sign of contracture. 

(2) In a few cases of severe trigeminal neuralgia of the second branch 
I observed at the height of the attack undoubted reflex clonic and tonic con- 
tractions of the muscles of the eye and cheek with retraction of the mouth. 
Such localized spasmodic attacks from pain are, however, not always strictly 
limited to the region of the facial nerve. For instance, in a girl aged 5, who 
had toothache with inflammatory swelling of the gums, I afterward saw clonic 
contractions of the left angle of the mouth and of the muscles of the fore- 
head. Simultaneously the eyes turned to the left and spasm occurred in the 
shoulder. On improvement in the gums, the attacks ceased. 

(3) Painless, unilateral, "idiopathic" spasm of the muscles of the face, 
which has several times been mentioned {tic convulsif in the earlier sense) 
is the prototype of an attack strictly confined to a nerve region (the facial 
nerve) in which clonic and tonic spasms occur. I have reports of 46 cases 
among which there were 17 males and 29 females. The predominance of 
females has already been commented upon by Gowers and others. In my 
cases of both sexes spasm was more common upon the left side (11 males, 
18 females). My youngest patient was a boy aged 9, my oldest a woman, 
aged 66, who had suffered from the attack for six months. In several of the 
older patients, however, the affection had begun 20 years or more previously. 

The contractions, as a rule, first appear in the muscles of the eyelid 
as a unilateral blepharospasm, which is the most common form of partial 
facial spasm. Later they involve the muscles of the cheek (the zygomatic, 
the levator anguli oris, etc.) and subsequently those of the forehead and the 
chin. In fully developed cases earlier authors as well as I have observed 
contractions in the muscles that move the lobes of the ear, also in the platysma 
myoides, and in one case a patient complained at the height of the attack 
of a grinding sensation in the ear which I attributed to a contraction of the 
stapedius muscle. But the spasm never extends beyond the region of the 
facial nerve, and is always limited to the side first attacked. 

. The duration of the individual attacks varies from a very brief time to 
15 minutes or longer. When prolonged, the clonic contractions at the acme 
of the attack pass into tonic spasms, the face during the interval becoming 
perfectly passive. Nevertheless, in a few instances in which the attacks were 
frequent I have observed a slight increase of the nasolabial fold and a nar- 
rowing of the palpebral fissure. The contractions are very irregular. I have 
already stated that they are frequently produced by chewing, swallowing, and 
speaking. After it has begun^ true tic convulsif (idiopathic facial spasm) 
cannot be controlled by any exercise of the will. 

Since the spasm never attacks the muscles of the tongue, the neck, or the 
shoulders, as do cortical spasms, it is likely that the irritative process which 
causes idiopathic facial spasm runs its course in the peripheral neuron of the 
facial tract alone. Some necropsy reports, for instance, those of F. Schultze, 
have shown that direct irritation of the facial nerve by an aneurysm of the 
vertebral artery may produce facial spasm. But a reflex irritation in the 
tract of the trigeminal nerve is usually much more likely, because, with the 
exception of the previously mentioned rare cases of basal tumors pressing on 
the facial, in which a rapid but transient spasm introduces a succeeding 



942 LOCALIZED SPASM 

paralysis, such an irritation has never been observed after idiopathic facial 
spasm which persisted for years. In a case of endocranial tumor of the 
anterior cranial fossa which had injured the first branch of the trigeminal 
nerve, Oppenheim saw facial spasm on the same side. 

Only very rarely do peripheral irritations (conjunctivitis, irritation of a 
tooth, affection of the ear, etc.) prove clinically to be of etiologic importance. 
The pressure points in the face and in the transverse processes of the cervical 
vertebrae which v. Graefe, R. Remak, and others described, and from which 
attacks may be produced or aborted, I have scarcely ever found to be active, 
in spite of much labor expended upon repeated tests. It has already been 
stated that nervous headache and attacks of migraine occasionally precede 
facial spasm; but hemicrania does not always occur upon the same side as 
the facial spasm. 

What has been previously stated of the treatment of localized spasm par- 
ticularly applies to true facial spasm. Actual cure only exceptionally occurs; 
more often there is merely improvement; exacerbations and relapses are 
common. 

(4) Post-paralytic spasm of the muscles of the face is not infrequently 
an accompanying phenomenon of severe or moderately severe peripheral facial 
paralysis which has not yet quite yielded. While the face is at rest there is 
usually a slight contracture, probably due to interstitial myositis of the for- 
merly paralyzed half of the face, which reveals itself by a narrowing of the 
palpebral fissure and the marked prominence of the nasolabial fold. The 
voluntary and involuntary movements, which to some extent reappear, cannot 
be evoked in the various portions of the face without implicating the other 
muscles, but on closing the eye the nasolabial fold is increased by the tonic 
and conjoint action of the zygomatic muscles, etc., and in puckering the mouth 
(as in whistling) the eye is more nearly closed. In such cases we not infre- 
quently observe more or less conspicuous and apparently spontaneous con- 
tractions, particularly of the muscles of the cheek, occasionally of the chin, 
whose synchronism with the physiologic wink of the eyelid I have several 
times demonstrated (1881 and 1898) and have observed to be invariable. 
When the patient voluntarily suppresses the closing of the lid these contrac- 
tions cease. I have therefore regarded these spontaneous contractions as 
clonic, constrained movements due to the physiologic closure of the eyelid. 
The irritation which Hitzig and others assume to be conveyed to -the nucleus 
of the facial nerve seems reasonable, and in the cases observed by R. Remak, 
Hitzig, Bernhardt, and Miiller is undoubtedly true of the post-paralytic facial 
spasm which also implicated the non-paralyzed half of the face, sometimes 
to a marked extent. 

Treatment for these conditions is of but little avail. The contracture of 
the cheek is best combated by stretching and galvanization with the anode. 
I also advise that the patient practise the movement of individual muscles 
before a mirror, and that he suppress conjoined movements as far as possible. 
Emotional movements of the face must be refrained from in order to prevent 
distortions. 

(5) Tic (impulsif) and 

(6) Myoclonia of the facial muscles can be differentiated only with great 
difficulty, and some authors, for instance, Meige and Feindel, group them 



CLINICAL VARIETIES 943 

together. If we consider as tic those combined movements which resemble 
voluntary ones, and as myoclonia the clonic, lightning-like, individual con- 
tractions of muscles, we find by experience that both forms of contraction 
actually occur in the face side by side. 

Of 43 cases in which the face was solely or chiefly implicated, 26 cases 
occurred in early life (4 to 20 years of age) and 30 cases were females. The 
high ratio of 69 per cent, of females probably depends upon hysteria which 
must often be taken into account. A woman whose case I reported came 
to me in 1894 on account of hysterical stuttering, but she also presented tic 
in the muscles of the face, for, owing to almost rhythmical contractions of 
the occipitofrontal, transverse folds formed in the forehead, the ears were 
occasionally moved, sometimes also the muscles which raise the upper lip, 
and the platysma myoides contracted. Certainly these cases are most common 
in neuropathically predisposed individuals. Fright and trauma (especially 
of the head) were in a few cases the accidental cause, and tic of the face, 
especially nictitatio and blepharospasmus duplex, has been observed by myself 
and others as an accompanying phenomenon of traumatic neurosis. Whether 
onanism, which is frequently observed in children, may be regarded as the 
etiologic factor, or whether this is simultaneously due to a nervous predis- 
position, cannot at present be decided. 

It is characteristic of tic as well as of myoclonia of the muscles of the 
face that the contractions are not limited to one side but are usually bilateral, 
are not always strictly confined to the region of the facial nerve but occa- 
sionally implicate the external muscles of the eye, the tongue, and the 
muscles of the vocal cord, as may be proven by directing the patient to make 
slight sounds. Sometimes they also implicate the muscles of the neck and 
the nape of the neck. In cases of apparent myoclonia of the face, after the 
patient removes his clothes we now and then note lightning-like contractions 
of the trunk and extremities which had previously been overlooked (polyclonia, 
chorea electrica). 

Unlike idiopathic facial spasm the patient may occasionally suppress these 
contractions at will, as is shown by the fact that during the examination 
there are no contractions, but these are frequent when the patient believes 
himself to be unobserved. A relatively common localization of facial tic, 
sometimes due to photophobia, is a frequent closure of the eye (nictitatio) 
or a conspicuous blepharospasmus duplex, occasionally an involuntary lateral 
rotation of the eyes. In addition to tic of the eyelids, Meige and Feindel 
differentiate mimical tic, tic of the nose (snuffling tic), tic of the lips (suck- 
ing tic), and tic of the chin. But they quite correctly lay stress upon the 
frequent combination of these tics with each other, with clonic contractions 
of the fronto-occipitalis, the muscles of the chin, and the platysma myoides. 
As I observed in the case of a girl aged 16, contractions of the fronto-oc- 
cipitalis may be so severe as to shake the hat. Clonic and tonic spasms 
limited to the platysma myoides or to the muscles of the chin, I have several 
times observed to be bilateral, once only unilateral, and in rhythmical se- 
quence, and have also seen these as individual attacks in hysterical women. 
In a case which was under my care for ten years, these paroxysmal contrac- 
tions of the platysma were a partial phenomenon of tabes in conjunction with 
hysteria. The rhythmical platysmic contractions occasionally mark a transi- 
61 



944 LOCALIZED SPASM 

tion into the previously mentioned chorea rhythmica (hysterica), and they 
also occur with rhythmic bilateral contractions of the muscles of the shoulder 
and arm. 

The course of myoclonia and of tic is irregular, and varies inasmuch as 
when the contractions cease in one area they may appear in another or may 
become general. Nevertheless the symptoms may sometimes disappear, but 
relapses are common. 

In treatment, especially of the young, the removal of deleterious or 
psychical sources of irritation is indicated, possibly also a stay in a sanatorium. 
Soothing measures, for example, warm baths, are generally advisable. Cold 
applications are contraindicated. In some cases there have been apparent 
results from the adminstration of moderate doses of arsenic and simultaneous 
and systematic galvanization of the nape of the neck by the anode (3 or 4 
milliamperes) ; in 1881 I found this treatment beneficial in a few cases which 
were diagnosticated as Henoch's chorea electrica, but what its therapeutic 
value is I cannot state. Lately we have been inclined to expect more from 
psychotherapy, but as this necessitates both the good will and cooperation of 
the patient, like the inhibitive therapy previously described (psychomotor 
education), to carry it out in children demands the utmost patience on the 
part of the physician. 

(7) Hysterical contracture of the facial muscles may be introduced by 
clonic contractions, but may appear without this, and may persist unilater- 
ally or bilaterally in a more or less unchanged condition. The diagnosis of 
hysteria should be made only after every other form of spasm has been ex- 
cluded, especially that due to organic cerebral disease (for example, of the 
pons) as well as post-paralytic and tetanic contracture. Hysterical con- 
tracture of the muscles which close the eye has been several times described. 
This also may be unilateral and, according to Parinaud and Charcot, Nonne 
and Beselin, Wilbrand and Sanger, may resemble ptosis (ptosis spastica 
pseudo paralytica) — a point which I cannot here discuss. A unilateral and 
usually slight hysterical contracture of the lower portion of the face is a 
partial phenomenon of typical hysterical liemispasmus glossolabialis, to which 
I shall refer when describing spasm of the tongue. In this condition, as 
Charcot, Brissaud and Marie have shown, and as I stated in 1892, unilateral 
contracture resembles paresis of the lower branches of the facial nerve which 
supply the mouth on account of the deepening of the nasolabial fold and the 
contractions of the lips. A light passed in front of the apparently paretic 
side of the mouth is not blown out, as in true paresis, upon this side, but 
after it has passed the median line and is on the side of the contracture. 

An isolated, unilateral, hysterical contracture of the lower half of the face 
without contracture of the tongue has so rarely been described (except by 
Ollivier, Bourneville, Binswanger) that I will mention a typical case of my 
own. 

A woman, aged 21, who for more than 6 months had been subject to hysterical 
crying, had attacks in which, after several contractions of the left cheek, the mouth 
and the left nasolabial fold were markedly drawn to the left, and remained so for 
one to three days. Upon admission to the hospital she presented a peculiar appear- 
ance. While the upper portion of the face down to the middle of the nose was quite 
symmetrical, the left ala nasi was drawn to the left, and the left angle of the mouth 
to the left and downward, the left nasolabial fold and the fold of the chin being 



CLINICAL VARIETIES 945 

strongly marked. In speaking and on opening the mouth there was no change. The 
tongue was protruded in the median line, and was freely moved. There was no differ- 
ence in the faradic contractility of either branch of the facial nerve. Faradization of 
the muscles of the right side brought the mouth to its correct position, but it subse- 
quently returned to the previous one. Nevertheless, the deviation of the face was 
corrected after faradization had been repeated several times in the course of a few days. 

(8) We can regard as occupation spasms only those exceedingly rare cases 
of spasm of the facial muscles which (analogous to true writer's cramp) are 
produced by definite movements of the face, and solely by these. To this 
group belongs a case reported by Zenner as auctioneer's spasm, in which, at 
first upon repetition of a number, subsequently in ordinary speech, spastic 
contractions appeared in different muscles of the face, particularly the orbicu- 
laris oris. In the case of a trumpeter observed by Oppenheim, who felt a 
contraction in the orbicularis oris as soon as he placed his instrument to his 
mouth, true spasm could not be determined. Bernhardt mentions a case of 
reading spasm which Duchenne described as occurring in a young man: As 
soon as he began to read, a contracture of the frontalis elevated the eyebrows, 
and the eyelids were spasmodically closed. On the other hand, Toby Colin 
reported as watchmaker's tic a continuous, typical, clonic-tonic spasm of the 
entire left half of the face supplied by the facial nerve, this occurring in a 
watchmaker whose work had for years required him to press a magnifying 
glass to his eye, and who had developed a spasm of the muscles supplying the 
left eye, a true blepharospasm. 

Before concluding the subject of localized facial spasm I must mention 
that since Hitzig's celebrated case of traumatic abscess in the right anterior 
central convolution, in which the focal symptoms began with clonic spasm 
in the region of the left facial nerve, particularly about the mouth and nose, 
but in which also the tongue showed transitory signs of paralysis, many sim- 
ilar cases of cortical facial spasm have been reported. Although the attacks 
may appear during perfect health, in which case they are of short duration, 
nevertheless the signs may point to a severe cerebral affection (tumor, etc.). 
A correct diagnosis is of the utmost importance. For example, a tuberculous 
man, 60 years of age, consulted me because in the preceding four weeks he 
had had five attacks, of at most two minutes' duration, in which the mouth 
was retracted to the left, the lower jaw became rigid and somewhat retracted, 
the tongue was immovable so that he could not speak, yet consciousness was 
not lost. The objective examination of the nerves gave absolutely negative 
results ; not even paresis of the left branch of the facial supplying the mouth 
could be determined, and this is of pathognomonic significance. For a time 
after the administration of a mixture of iodin and bromin the attacks lessened 
in severity; six months later the house plrysician informed me of the'lethal 
outcome of the case. The attacks had been intensified, and left-sided hemi- 
plegia had finally occurred. 

In the initial stage of these cases the diagnosis is frequently difficult, for, 
as the following case shows, hysteria may simulate the picture of cortical 
spasm. 

A girl, 13 years old, the child of nervous parents, was brought to me because 
for about eight months preceding and at irregular intervals she had had numerous 
attacks, lasting about five minutes and accompanied by a feeling of anxiety, during 



946 LOCALIZED SPASM 

which the muscles of the left cheek and of the eye moved to and fro. The tongue 
was drawn to the left side of the mouth so that she could not speak, she saw a play 
of colors, and her left hand felt numb. No contractions were observed in the head 
or arm, and consciousness was retained. The objective findings were entirely negative. 
Under psychical and faradic treatment the attacks ceased, and four months later there 
had been no recurrence of the attacks. 

Group II. — Except for fibrillary contractions, localized spasm of the 
tongue is very rare, while, on the contrary, the tongue is frequently impli- 
cated in general spasmodic neuroses, chorea, epilepsy, and tic. 

(1) The continuous, wavy, fibrillary contractions which are observed in 
the atrophic tongue in amyotrophic bulbar paralysis, and bilaterally and uni- 
laterally in hemiatrophy of the tongue due to traumatic or neuritic hypo- 
glossal paralysis, may be designated as myokymia of the tongue. These con- 
tractions correspond to the so-called paralytic oscillations which have been 
studied by physiologists. They must not be confounded with the tremor of 
the quiet or protruded tongue which is frequently observed in progressive 
paralysis, in alcoholism, in melancholia, etc. • 

(2) A few cases have been described of reflex spasm of the tongue due 
to painful affections of the mouth, as from caries of the teeth (Mitchell), 
after .extraction of a tooth (Fere), or in trigeminal neuralgia which impli- 
cates the lingual nerve (Eomberg). Keflex irritation may also originate in 
the nasal cavity (Wendt) or in even more distant nerve areas. Gallerani and 
Pacinotti described a case of spasm of the tongue, of the lips, and of the 
pharynx, which was manifest in the speech chiefly as aphthongia, and gradu- 
ally disappeared after the excision of a small piece of porcelain from the 
occipital nerve. It appears that reflex spasm of the tongue is even less strictly 
localized than the idiopathic form. 

(3) Idiopathic spasm of the tongue, according to the observations of 
0. Berger, Dochmann, E. Eemak, Erlenmeyer, Bernhardt, Hirt, Personali, 
Lange and Sanger, appears without obvious cause, occasionally in nocturnal 
attacks, usually with bilateral, and sometimes clonic, contractions which are 
more marked upon one side, and which may be preceded by a sensory aura 
such as paresthesia of the tongue. In 1883, I presented before the Berlin 
Medical Society the case of a man, aged 33, whose tongue, resting upon the 
floor of the mouth, by a decrease and flattening of its surface was moved 
45 to 50 times in the minute, while corresponding rhythmical contractions 
of the muscles of the tongue could be externally felt between the lower jaw 
and the hyoid bone. The tip of the tongue never struck against the teeth. 
The contractions became stronger when the tongue was depressed with an 
instrument; then it became markedly arched, especially toward the left side. 
The tongue was protruded rhythmically, and even forcibly. The contractions 
were increased, and invariably produced, by mastication. In this case the 
tongue was frequently caught between the teeth or pressed against the palate 
by a tonic spasm which prevented deglutition. In the more violent attacks 
produced by mastication, the spasm subsequently implicated the lower half 
of the left side of the face, and caused severe tonic and clonic contractions 
of the muscles of the cheek and the area surrounding the mouth, while the 
upper branches of the facial nerve were not involved. A paresis of the left 
branch of the facial supplying the mouth, which was previously demonstrated, 



CLINICAL VARIETIES 947 

was more profound after these major attacks. This affection, which had 
developed within four weeks, declined markedly within eight days under the 
administration of potassium iodid and potassium bromid, and the daily anodal 
galvanization of the inframaxillary region; it has never reappeared. There 
was no paresis of the facial nerve when I examined the man two years later. 
In my case, and also in Erlenmeyer's, the spasm implicated the region of the 
right facial nerve. In Hirt's case spasm of the muscles of mastication always 
preceded spasm of the tongue. While in my case the attacks were chiefly 
produced by the act of mastication (Romberg's masticatory lingual spasm), 
in other instances nothing of this kind was noted, and in Lange's case the 
tonic spasm of the tongue ceased when the patient began to chew, so that 
during the day he constantly chewed something in order to keep his tongue 
in his mouth. Closer analysis of the reported cases confirms the view that 
the pathologic picture of idiopathic spasm of the tongue is not so uniformly 
and strictly confined to the region of the hypoglossal nerve as is, for example, 
idiopathic facial spasm; therefore it seems unlikely that the pathogenesis 
of idiopathic spasms of the tongue is invariably the same. If they are in 
any sense true spasms it is doubtful whether they are due to affection of the 
nucleus of the hypoglossal or of the cortical region of the tongue. In either 
case we can understand the transference of the effect to the tract of the 
lower facial and the motor trigeminal nerves. As paresis of the facial nerve 
could be demonstrated in my case, I assumed a partial cortical epilepsy. 

Whether the treatment I employed brought about a cure cannot be stated 
with certainty, but, at all events, in other cases (for example, that of Berger) 
galvanic treatment has been effective. Personali saw good results from 
antipyrin in increasing doses of from 0.8 to 4.6 grams per day. Sanger 
found iron and arsenic to be beneficial. 

(4) According to Meige and Feindel, lingual tic (tic impulsif of the 
tongue) rarely occurs alone. They characterize as licking tic the tic move- 
ments in which the tongue moistens too profusely the free border of the 
lips; as clicwer's tic those in which the tongue rolls about between the teeth 
and the inner surface of the cheeks. On account of their psychogenous 
origin, the cases described by Erb, Seppilli, and Oppenheim probably belong 
to the tics of the tongue, cases of spasm of the tongue accompanied by hal- 
lucinations and other psychical anomalies. 

(5) Hysterical spasm of the tongue is noted as one of the phenomena 
of hemispasmus glosso-labialis, but also on protuding the tongue and without 
a simultaneous facial contracture as a unilateral tonic spasm in this affection. 
Not rarely it appears with hemianesthesia, usually of the other side. In 
the typical case of hemispasm of the right side, which I analyzed in 1892, 
the tongue lay straight upon the floor of the mouth or, perhaps, deviated 
slightly to the right. With irregular rotary movements it was pushed strongly 
to the left, then in a convex arch to the right side, so that it was held almost 
entirely in the right half of the mouth, and deviated more than it ever does 
in hemiatrophy or in cerebral hemiglossoplegia. In this deviating position 
the patient was able to move the tongue up and down, but to the left only 
to the median line. As soon as I attempted to move the organ to the left 
the muscles on the right side of the tongue offered strong resistance. When 
this was overcome, and the tongue was brought to the left angle of the mouth, 



948 LOCALIZED SPASM 

it did not deviate as soon as the pressure was relaxed, but only after con- 
siderable time and as if by voluntary movement. Even when the tongue was 
returned to its normal position by faradic stimulation of its left half, on 
interruption of the current it did not deviate until after some time had 
elapsed. 

Hysterical contractions of the tongue may accompany other contractions 
of the face, of the neck, etc. For instance, I saw severe attacks in a case 
of hysteria virilis occurring in a man, aged 28, who presented right-sided 
hemiparesis and hemianesthesia, and in whom persistent, almost rhythmical, 
and painful contractions of the right-sided muscles of the cheek and forehead 
distorted the face. His tongue was protruded paroxysmally, the contractions 
of the face increasing, and extending to the right platysma myoides and to 
the right arm. 

(6) Articulator!) lingual spasm which only appears on an attempt to 
speak, and at first prevents speech, which Fleury (1865) designated aph- 
tliongia, was observed by Fleury, Vallin, Mossdorf, Ganghofner, and Steinert, 
usually in excitable young people from mental emotion (fright, sorrow, 
anxiety). Upon attempting to speak there is a tonic intention spasm of the 
muscles of the tongue which usually forces the tip of the tongue against 
the teeth and its root against the palate. Even this form of lingual spasm 
is rarely limited to the region of the hypoglossal nerve, but usually implicates 
the lower muscles of the face, the platysma myoides, the sternocleidomastoid, 
the abdominal muscles, and, by causing the patient to make a fist (Steinert) 
or by contraction of a lower extremity (Ganghofner), even the muscles of 
the extremities. These authors have warned us not to confound this condition 
with severe stuttering, in which extensive constrained movements may also 
occur. In the treatment of these spasms, it is advisable to resort to psychical 
influences, and, judging by the favorable results of Mossdorf, to anodal gal- 
vanization of the upper part of the nape of the neck. 

(7) Other occupation spasms of the tongue have been only rarely re- 
ported, by v. Strumpell in a clarionet player, and by Turner in a cornetist. 
When he played with the band, the latter was unable to move his tongue for 
the production of certain notes, while at home he could produce them at will. 

Group III. — In passing we may mention localized spasms of the palate 
which usually run their course as rhythmical contractions. They have been 
noted as the consequence of organic diseases, bilaterally in tumors of the 
cerebellum (Oppenheim), and unilaterally in aneurysm of the vertebral artery 
(Oppenheim and Siemerling). They are more common when purely func- 
tional (hysterical) in origin (Williams, Scheich, Goldflam, Meyerson, Peyser, 
Avellis, Bernhardt and others). The various forms of spasm of the glottis 
need no special mention. 

Group IV. — Localized spasm of the muscles of mastication mainly affects 
the muscles innervated by one nerve, the motor branch of the fifth; these 
muscles are the temporal, masseter, pterygoid, mylohyoid and digastric. Au- 
thors usually differentiate only clonic and tonic (trismus) forms of this 
spasm. Meige and Feindel, however, described a tic of the lower jaw (biting 
and chewing tic) and a psychogenous trismus. 

(1) Chattering of the teeth from exposure to cold and from chill in 
fever is due to clonic rhythmic contractions of the muscles of mastication. 



CLINICAL VARIETIES 949 

Similar clonic contractions limited to the muscles of mastication now and 
then occur in hysteria. Clonic spasms of the muscles of mastication as a 
partial phenomenon of general spasms are more common. It is well known 
that these cause epileptics to bite the tongue, and in some cases of paralysis 
agitans they produce rhythmical horizontal contractions of the lower jaw 
(pterygoid muscles). 

(2) A tonic spasm of the muscles of mastication (trismus) can be assumed 
only after excluding the fact that the teeth are locked because of an anky- 
losis or pseudo-ankylosis of the joints of the jaw. The palpable demon- 
stration of tonic tension of the masseter and temporal muscles is decisive. 
This may readily be due to some painful irritation affecting the third branch 
of the fifth nerve as well as by painful affections of the teeth, and is especially 
prone to occur in children from the cutting of teeth. We must always bear 
in mind, however, that trismus may be the first symptom of tetanus, and 
a simultaneous paralysis of the facial nerve is an indication of head tetanus. 
The preceding injury to the face may have been so slight as to have been 
forgotten. For example, in 1883 I saw a case which was later described by 
P. Giiterbock, a fatal case in a man aged 34. As the lockjaw was apparently 
preceded by bilateral pain in the region of the jaw, I erroneously assumed 
a reflex origin in the oral cavity, and sent the patient to a surgeon for 
examination under anesthesia. In a case of head tetanus in a boy aged 9, 
diagnosticated by me in 1901 and described by Jolly, a diagnosis of menin- 
gitis was made at his home. As a matter of fact, at the onset of the affection 
a diagnosis may be extremely difficult, especially if, as P. Richer, v. Eisels- 
berg, Jolly, and Binswanger have observed, there is also hysterical trismus 
which resembles beginning tetanus. Such cases may be identified with the 
psychogenous trismus described by French authors, which may also be a 
symptom of marked psychosis. The diagnosis is much less obscure when 
trismus occurs as a symptom of organic cerebral disease (meningitis, acute 
bulbar paralysis, tumor of the pons or cortical disease), because the accom- 
panying symptoms of irritation as well as the general signs point to the true 
nature of the disease. In conclusion, the rigidity of the muscles of the jaw 
in mastication, which is peculiar to Thomsen's disease (myotonia), may also 
be mentioned. 

(3) According to Meige and Feindel, tic impulsif of the muscles of mas- 
tication (hiting and chewing tic) occurs in predisposed individuals in conse- 
quence of an erosion of the lips or of a slight periostitis of the jaw, and is 
shown by the fact that mastication produces sensation in the affected area. 
On the other hand, constrained tic movements in the lower jaw may become 
so severe that the lips are bitten (cheilophagia). Onychophagy which, as is 
well known, is a sign of degeneration, may also be regarded as biting tic. 

Group V. — Next to spasm of the muscles of the face, I have found local- 
ized spasms of the muscles of the neck and nape of the neck to be the most 
common form (in about 50 cases they formed 20 per cent.). I do not group 
among these painful and chronic torticollis (caput obstipum or torticollis rheu- 
maticus) in which there is extreme intolerance even of passive motion of the 
stiff, cervical vertebral column, this depending chiefly upon inflammatory, 
and partly upon reflex, contraction of the sternocleidomastoids, especially of 
one, in consequence of cold to the nape of the neck or inflammation of the 



950 LOCALIZED SPASM 

cervical vertebrae. This will be described, as well as congenital torticollis 
which is usually due to a shortening of the muscles. If we confine ourselves 
wholly to the clonic-tonic spasms of the muscles of the neck and nape, a 
sharp differentiation of the various forms becomes very difficult, much more 
so than in the forms of spasm previously described. 

(1) Nodding, salaam, or greeting spasms (spasmus nutans) occur in 
early childhood, usually in the period of dentition, and are characterized by 
a pagoda-like twitching or nodding of the head which occurs several times 
in a minute and depends upon clonic, and usually rhythmic, contractions of 
the deep muscles of the neck (splenius, biventer, recti et obliqui capitis), 
sometimes also of both sternocleidomastoids. These contractions are not in- 
frequently associated with nystagmus, which is most apt to occur when the 
head is firmly held or, as I have noticed, when the child while looking at an 
object periodically interrupts the movement of the head. This form of spasm, 
as I recently had an opportunity of observing in the case of twins, lessens 
after some time, but it may also be the first symptom of a grave cerebral 
affection. 

(2) In predisposed children six years of age and upwards myoclonia and 
tic are sometimes, although not exclusively, localized bilaterally in the muscles 
of the neck and the nape. I have records of 13 such cases occurring chiefly in 
females (10). In irregular sequence, here and there, brief individual con- 
tractions of the superficial muscles of the neck were noted, especially of the 
platysma myoides (myoclonia). Or the head was rapidly thrown backward 
or shaken (tic) by brief and usually s} T mmetrical, combined, spasmodic con- 
tractions of the muscles of the nape. In a case of Henoch's chorea electrica 
which I demonstrated in 1881, that of a girl aged 10, the head was forced 
backward 60 times in a minute by rhythmical contractions of the muscles 
of the neck and palpable contractions of the splenii capitis, while spasms of 
the platysma were also visible; as a rule nictitation and tic-like contractions 
of the muscles of the face reveal the nature of the affection. This rarely 
persists to advanced age. But a spinster, aged 52, had from earliest child- 
hood, and particularly when walking, been subject to brief posterior move- 
ments of the head and, simultaneously, bilateral and visible contractions of 
the platysma. Ehythmical spasm of the platysma in hysteria has already 
been mentioned; this cutaneous muscle is supplied by the facial nerve. 

(3) Clonic and tonic rotary spasms of the neck {torticollis spasticus) 
I have observed in 24 cases ranging in age from 13 to 66 years (11 males 
and 13 females). In only two cases did it appear before the twentieth year; 
in the overwhelming majority it was after the thirtieth. As a rule the 
occiput (region of the ear) on the same side as the spasm is forcibly and 
paroxysmally drawn downward, the face is turned to the opposite side, and 
the chin is elevated, while contractions of the sternocleidomastoid are evident 
both to inspection and palpation. As the trapezius of the same side fre- 
quently and simultaneously becomes tense and draws the head backward, 
raises the shoulder, and draws the shoulder-blade toward the vertebral column, 
we were formerly inclined (according to the traditional teaching that local- 
ized spasms originate from individual peripheral nerves) to explain rotary 
spasm simply as a spasm of the spinal accessory. It did not, however, escape 
the notice of vigilant observers that the muscles of the nape of the neck 



CLINICAL VARIETIES 951 

which are supplied by the upper cervical nerves, such as the longissimus 
capitis, the splenius capitis, and the transversus spinalis of the same side, 
are implicated in the spasm; that, especially with persistent intermittent 
spasms, contractions of the antagonistic muscles at the other side of the neck 
are also alternately noted. These combined spasms of the muscles of the 
neck are explained by Seeligmuller and others as arising because, in conse- 
quence of a spasm of the spinal accessory, the antagonists of the corresponding 
muscles of the other side make a constant effort to keep the head in an erect 
position, and are thus themselves contorted by spasms. From my own ob- 
servations I have come to the conclusion that a pure spasm of the spinal 
accessory is very rare, although I am willing to admit with Bernhardt that 
it does occur. In fact, close study of paralysis of the trapezius has shown 
that the cervical nerves are certainly implicated in the innervation of this 
muscle, and that when the trapezius is more decidedly involved in the spasm 
it is a mistake to consider merely a strict localization in the region of the 
spinal accessory. This is of practical importance, inasmuch as it explains the 
numerous failures after division of the spinal accessory in cases of so-called 
spinal accessory spasm. 

But in most cases not only the limitation of rotary spasm of the neck 
to the region of the spinal accessory but also the assumption of a true spasm 
has been doubted. Brissaud and his pupils, Bompaire, Meige and Feindel, 
regard it as a psycliogenous tic {torticollis mental). Xeuropathically pre- 
disposed or psychically influenced persons, from an insignificant irritation 
in the region of the nape of the neck or from their occupations, are said to 
contract a habit of turning the head which subsequently develops into a con- 
strained, psycliogenous tic (impulsif). As proof of the tic nature of the 
spasm, it is maintained that when the attention of the patient is distracted 
the spasm sometimes ceases, and that they can sometimes suppress the con- 
tractions, as I proved in several cases, by making slight pressure with the 
finger against the chin, the contractions being previously so violent that the 
investigator was unable to check them by any means. Xevertheless Meige 
and Feindel hold that the differential diagnosis between spasm and .tic of the 
neck is not easy, and they base their diagnosis of tic essentially upon the 
demonstration of psychical anomalies which are peculiar to all tic patients 
(peculiarity in or change of disposition, childish mental state). 

From the investigation of my own clinical cases I believe it to be abso- 
lutely impossible to differentiate cases of true spasm and psycliogenous tic, 
and I certainly believe that there are many transitional stages between them. 

As rotary spasm of the neck is a most conspicuous and distressing affec- 
tion which not only renders the >patient unable to work but is apt to occur 
during walking, etc., and therefore interferes with all social life, the moody, 
disagreeable nature of these patients is sufficiently explained. But Oppen- 
heim and others, as well as I, have observed marked psychical disturbances 
(hallucinations, etc.). A man, aged 25, in whom persistent torticollis spas- 
ticus had been observed, was arrested a few years later for exhibitionism. 
Scotoma is frequently noted. A woman, aged 47, who was under my observa- 
tion for seven years, had left-sided spasm of the spinal accessory which 
improved and left but the merest trace; but within a year she was disturbed 
by continuous photopsia though the eyes were in a normal condition. On 
62 



952 LOCALIZED SPASM 

several occasions simultaneously a bilateral blepharospasm revealed the tic 
nature of the affection. In 1881 I examined a woman, aged 52, who had 
peculiar rotary movements of the eyes. This case, which had been success- 
fully treated by my father in 1862, was of etiologic interest because the 
patient had for 13 years been employed at reeling wool which compelled her 
constantly to move her head from one side to the other. This occupation 
etiology, lately urged by Walton, appeared also in six others of my cases (in 
a balancer, a piano-teacher, two locksmiths, a velvet smoother, and in a 
statistician whose work was the comparison of rows of figures). In all of 
these cases spasm was introduced by disagreeable sensations in the nape of 
the neck (usually, but by no means always, pressure points could be demon- 
strated in the transverse processes of the cervical vertebrae) and this suggested 
a reflex genesis. Nevertheless, nothing conclusive can be stated in regard to 
the starting-point and the pathogenesis of rotary spasm of the neck. Oppen- 
heim believes it likely that this spasm originates in the kinesthetic centers for 
the muscles of the neck in the cortex of the brain. 

A markedly tenacious course and the inefficacy of all antispasmodic therapy 
is almost an invariable rule in this form of spasm. Nevertheless, under rest 
and galvanic treatment of the nape of the neck (especially of the transverse 
processes), persisted in for a year and longer, in four cases I saw such an 
improvement that there was but a slightly oblique position of the head. 
Among these was one case that had been under observation for eight and a 
half years. But the permanence of the recovery is always doubtful, and here 
psychical influences play a great role. A woman, aged 28, was attacked at 
the age of 16 with right-sided spasm of the spinal accessory, but between 
the ages of 21 and 25 and while she was engaged to be married she was 
entirely exempt. After the breaking of her engagement, a relapse occurred 
and the spasm persisted for three years. A patient, aged 66 (according to 
a history taken by my father who made a diagnosis of alternating spasm of 
the spinal accessory), who for thirty years had followed his occupation as a 
school-teacher, suffered a severe relapse after a profound mental shock. 

Psychomotor education, which for this form of spasm is especially lauded 
by Meige and Feindel, may be employed alone or as an auxiliary to other 
forms of treatment, particularly galvanism. Hasebrock lately brought about 
improvement in the position of the head by the use of elastic rubber bands 
fastened to strips of plaster, which aided the action of the healthy antag- 
onists. 

According to de Quervain's compilation of the results of Kocher's opera- 
tions, one method which consisted in the severing of all the muscles impli- 
cated in the spasm should be considered in especially severe cases. Kocher 
attaches great importance to subsequent gymnastic exercises. An engineer, 
aged 40, suffering from severe spasm which was at first left-sided and then 
bilateral, and who at that time was unsuccessfully treated by me, was oper- 
ated upon by Kocher eleven months later, and six years afterward I had an 
opportunity of confirming the absolute success of the operation. The func- 
tional disturbance was slight, and the deep transverse cicatrix on the nape 
of the neck did not greatly disfigure him. 

(4) Aside from the previously mentioned painful and partly reflex caput 
obstipum, bilateral contracture of the muscles of the nape of the neck which 



CLINICAL VARIETIES 953 

appears as stiffness of the nape of the neck or is markedly developed as 
opisthotonos, is a symptom of tetanus or organic disease of the posterior 
cranial fossa (tumor, meningitis, etc.). In 1890 I demonstrated in a tuber- 
culous patient slight contracture of the muscles of the neck and more decided 
contracture of the muscles of the shoulder and upper arm with excessively 
increased tendon reflexes in this region; the autopsy, conducted by Gold- 
scheider, revealed a myelomeningitis cervicalis anterior which appeared to 
have irritated the anterior cervical roots. 

In paralysis of the serratus complicated with partial paralysis of the 
trapezius, unilateral contracture of the single muscle of the nape of the 
neck which passes to the shoulder-blade (levator anguli scapulae) and of the 
rhomboidei causes the so-called rocking position of the scapula. In some 
cases reported by A. Eulenburg, Xove-Josserand, Bernhardt, P. Manasse, 
and others, an acquired high position of the scapula was attributed to per- 
manent and idiopathic tonic contraction of the levator anguli scapulae and 
of the rhomboideus. In a case reported in 1903 by P. Manasse, in which 
all other methods of treatment were for a long time unsuccessful, repeated 
division of the contracted muscles led to recovery. It is noteworthy that 
during the operation, and while the patient was under anesthesia, clonic con- 
tractions of the muscles occurred. 

Group VI. — Of all the localized spasms of the upper extremity the most 
numerous are occupation spasms, particularly writer s cramp. As occupation 
neuroses (of which occupation spasm is a common form) will be described 
in a separate chapter, I shall not discuss them here; I reported my own ex- 
periences in an article in the " Eeal-Encyklopiidie " in 189-1. I shall merely 
remark that while, as a rule, writer's cramp is noted exclusively during the 
act of writing, yet when the affection is severe it may last longer than this, 
and may exceptionally present itself also as intention spasm during other 
occupations. For instance, in a book-keeper whose hand showed spasmodic 
dorsal flexion, and deviated toward the ulnar side on every attempt to write, 
the same spasm was produced whenever he held small objects in his hand 
(for example, playing cards), and this partial and tonic radial spasm was 
frequently visible when he held nothing. L. Laquer reported a substantive 
tonic radial spasm in connection with writer's spasm. Another patient, 
under my observation with writing spasm, was seized with a -flexor spasm 
of the right wrist-joint and fingers on every attempt at writing; this con- 
tracted the hand into a perfect ball shape. The spasm, like that of Gowers's 
case which chiefly affected the region of the median nerve and amounted 
to a flexor spasm, also occurred when, on making an effort to grasp any- 
thing, the thumb was placed in apposition to the fingers. When the spasm 
was more markedly developed, not only were the fingers spasmodically con- 
tracted but the hand also showed spasmodic volar flexion, and the elbow- 
joint was flexed. The view of a cerebral origin for such forms of spasm is 
confirmed by the history in the following case of symmetric irradiation of 
occupation spasm. An official, who had suffered for 15 years with severe 
writer's spasm and painful stiffening of the joints of the right hand, 
and who for 4 years had written with his left hand, complained that for 
2 years of this latter period the right thumb by a constrained movement 
had been forced under the other fingers. He could only resist this pain- 



954 LOCALIZED SPASM 

ful spasm by grasping some object with the right hand while writing with 
the left. 

Peripheral irritative factors may produce, presumably by way of the 
reflexes, secondary occupation spasms of the upper extremity similar to my 
previously mentioned case of milker's spasm in a milk-maid, who, after pro- 
longed milking, at first suffered from nocturnal painful spasms of the fingers, 
but in the second week after abandoning her occupation and while under 
observation had painful drawing sensations lasting for 15 minutes; these 
began in the fingers and passed to the shoulder when flexor spasms of the 
first three fingers (spasms of the median nerve) occurred, and also actual 
pain. In this case there was bilateral neuritis of the median nerve with a 
corresponding disturbance of sensation in this region, also in the area of 
the right superficial radial nerve, as shown by the reaction of degeneration. 
As other cases of occupation neuritis are not usually accompanied by such 
local spasms, perhaps these depend upon a special " spasmophilic " predis- 
position based on hysteria. This may also explain why localized attacks of 
spasm, rhythmical, clonic, tonic contractions, or tonic spasms, are so much 
more rarely observed after over-exertion, no matter of what nature, than 
so-called occupation paralyses. After exhaustive gymnastics Hochhaus saw 
in the case of a boy, aged 9, contractions of the right triceps and supinator 
longus which occurred 40 to 50 times per minute. I saw in a girl, aged 10, 
rhythmical contractions of the right biceps and supinator longus after climb- 
ing. In a workman who had frequently lifted heavy loads of iron Bern- 
hardt (1901) observed clonic contractions in the left pronator teres which 
recurred about 150 times a minute. Brissaud (1902) saw in cork-cutters 
a permanent spastic retraction of the last two fingers of the left hand, such 
workmen being compelled to keep these fingers in one position. 

After trauma, but only with a special predisposition (with or without 
external wounds), localized (hystero-traumatic?) spasms of the arm of vary- 
ing extent may be observed. The painful muscular contractions which occa- 
sionally are felt in stumps after amputation are familiar to us from the 
descriptions of Eomberg, S. Weir Mitchell, and others. Injuries without an 
external wound or cicatrix are often sufficient to produce these. A patient, 
aged 18, who sustained a fall upon the right elbow- joint, suffered for two 
years from contractions in the right thumb as soon as he placed it in a 
position of medium apposition. A girl, aged 9, a few weeks after a dis- 
tortion of the right wrist was asked to write; immediately afterward her 
right hand contracted into a fist and could not be opened for twelve hours. 
A boy, aged 9, three days after being struck by his brother a blow on the 
right arm with a stick, had permanent rhythmical contractions of this arm 
which persisted for two years, and then suddenly ceased, leaving a tremor 
which appeared only when he tried to write. A girl, aged 18, had daily 
four or five attacks of clonic contractions in the right hand which, closed 
to a fist, assumed a position of dorsal flexion, the elbow-joint being flexed 
and the upper arm spasmodically adducted; neither the head nor the facial 
nerve was implicated in this spasm. It was noticed that the spasm could 
be produced at will by pressure upon a painful spot on the back of the fore- 
arm. There was gradual improvement after anodal galvanization of this 
area. This was probably hysterical brachial hemispasm, and was attributed 



CLINICAL VARIETIES 955 

to the pressure of an exostosis. In children especialty, even without trau- 
matic etiolog}', probably owing to hysteria and particularly after emotional 
disturbance (fright and the like), various forms of spasm of the upper 
extremity occur which, because of their apparently psychogenous develop- 
ment and the variations in their mode of appearance, are difficult to 
describe systematically; they frequently undergo a transition into hysterical 
contracture, chorea rhythmica, chorea hysterica, and tic impulsif. A girl, 
aged 11, was under treatment two years for a grapho-tremor ; there was a 
contracture of the left arm accompanied by pain in the left elbow-joint, and 
this resembled a cerebral spastic hemiplegia running its course simultaneously 
with almost rhythmical contractions of the adductors of the shoulder. Under 
the administration of arsenic and the use of galvanism partial recovery oc- 
curred in three months, the tremor persisting. A boy, aged 11, after being 
frightened by a locomotive, was subject to contractions which spasmodically 
adducted the arm to the thorax, the hands approximating to each other. 
Occasionally contractions were noted in the legs when elevated or the patient 
was forced to stamp if sitting. The head was occasionally rotated to the side. 
Improvement followed the employment of valerian and the faradic brush. 
A very hysterical girl, aged 13, had attacks in which the right arm, and 
occasionally the foot, was drawn up to the head. Pressure in the ovarian 
region evoked spasmodic contractions of the hands. In adults also rhythmical 
and extensive spasms of the arm are observed after accidents. To this cate- 
gory belongs a case described by A. Eulenburg in 18DT as fright neurosis, 
in which hemianesthesia and rhythmical contractions of the left pectoralis 
major, the clavicular portion of the deltoid, and individual bundles of the 
serratus were observed in a patient who had suffered from an accident. 

The differentiation of such cases of pure functional spasms of the arm 
from other forms is most important, but is not always easy. I can merely 
touch upon the affections to be considered, which are as follows: 

(1) The forms of true epilepsy which begin with a sensory and motor 
aura in the upper extremity and are chiefly due to irritation from a cicatrix, 
usually after injury to the finger; 2. Cortical spasms of the arm which are 
ushered in with rhythmical clonic contractions, do not extend beyond the 
arm, do not necessarily produce unconsciousness, and are usually followed 
by more or less temporary paralysis; 3. Attacks of tetany which, especially 
in the region of the ulnar nerve, are usually bilateral, but can only be demon- 
strated as such when the characteristic electro-diagnostic symptoms are evi- 
dent, because spasm of the ulnar nerve and contractures may be due to other 
causes. For instance, in a girl aged 11, after an attack of indigestion with 
vomiting, I saw hysterical intention spasms in the region of the left ulnar 
nerve upon trying to grasp any object or when extending her arm backward. 
There was marked analgesia of the arm. The attacks disappeared upon 
application of the faradic brush. Another girl, aged 11, suffered for three 
months, at first only on writing, from a transitory and finally permanent 
contracture in the right ulnar region; this was cured by labile galvanization 
of the ulnar nerve. In a man, aged 39, who for several years had slept in 
the open air in Texas, I observed a gradual and permanent but painless 
spastic contracture in the left ulnar region, especially of the flexor carpi 
ulnaris, yet there was no disturbance of sensation or of the mechanical or 



956 LOCALIZED SPASM 

electric contractility. In the morning • the contracture was only slight, but 
it increased during the day until it resembled ulnar tetany. The benefit from 
galvanization was here only transitory. 

I need not refer to the various forms of trembling which occasionally 
increase to a shaking spasm, particularly in paralysis agitans, chorea, athe- 
tosis, tetanus, and myotonia, and which are most common in the upper 
extremity. That tic impulsif also affects the upper extremity has been men- 
tioned. Meige and Feindel described a scratching tic, also the irresistible 
impulse to pick the nails of the fingers and toes. 

Group VII. — Localized spasm of the transverse striped muscles of the 
trunk is noted in the muscles of the chest, of the diaphragm, and of the 
abdomen. 

Spasm of the muscles of the chest is comparatively rare, is usually bi- 
lateral, and is probably due to hysteria. In a lady, aged 24, who was very 
hysterical, I saw a rhythmical succession of paroxysmal contractions of the 
pectoralis and the inward rotators of the arm which occurred about 70 times 
a minute, and did not cease when the arm was raised nor in walking. Such 
cases have also been called chorea rhythmica hysterica. 

Singultus (hiccough), which is often very disagreeable, is due to clonic 
spasm of the diaphragm. Violent contractions of the diaphragm without a 
simultaneous dilatation of the glottis cause the inspiratory murmur. The 
affection is relieved by derivatives and blisters, by faradization of the scrobicu- 
lus cordis, by the administration of antispasmodics, and by diverting the 
attention of the patient from his condition. Prolonged clonic spasm of the 
diaphragm is comparatively rare. A lady, aged 65, who had marked kypho- 
scoliosis, suffered for three years, apparently after influenza, from pain in the 
region of the epigastrium, and rhythmical contractions of the entire upper 
part of the body which were evidently contractions of the diaphragm (48 to 
54 in a minute) ; Litten's diaphragm phenomenon was also observed as 
well as constrained movements of the erector trunci and right quadrati 
lumborum. Under anodal galvanization of the nape of the neck and back, 
this tachypnea was decreased from 36 to 30, and the contractions gradually 
ceased. 

Tonic spasm of the diaphragm, usually of hysterical nature but also oc- 
curring in tetanus and tetany, was described by Duchenne and others as a 
very distressing affection, for the vaulting of the epigastrium and hypochon- 
drium may greatly increase the dyspnea. Powerful rubefacients applied to 
the lower thoracic region and hot baths are useful in this condition. 

Isolated spasms of the abdominal muscles seldom occur except in tetanic 
attacks, epileptic spasms, and in polyclonia, and in all of these hysteria is 
probably the chief etiologic factor. A man, aged 31, who had been subject 
to headache, suffered for four weeks from clonic contractions of the abdom- 
inal muscles accompanied by pain in the scrobiculus cordis and anorexia; 
when he was in the recumbent posture these contractions became paroxysmal 
and arrythmic, and involved both recti and obliqui so markedly that the whole 
trunk trembled. They could invariably be produced by pressure upon the 
epigastrium. Neither respiration nor the diaphragm were implicated. A 
conspicuous, girdle-like, cutaneous anesthesia extending from the scrobiculus 
cordis to below the navel indicated the hysterical nature of the spasms. 



CLINICAL VARIETIES 957 

Under faradism of the skin and galvanic treatment the patient recovered in 
three weeks. 

Even unilateral contractions of the abdominal muscles have been described 
by Duchenne and Bernhardt. 

Group VIII. — Localized spasms in the lower extremities are more or less 
rare, but they occur in the same forms as in other regions. 

(1) Myokymia was described by Kny and Fr. Schultze as most prone to 
affect the muscles of the calf, and in a few instances painful spasms of the 
muscles of the calf were periodically observed. In a case of J. Hoffmann's 
they were localized in the region of the sciatic, and Bernhardt and I ob- 
served them in cases of bilateral or unilateral sciatica. It is noteworthy that 
in all of these instances the tendo Achillis reflex was absent, therefore neu- 
ritis probably existed. 

That myokymia in the lower extremity may develop chiefly from over- 
exertion, and may gradually disappear after rest, was taught me by the fol- 
lowing case: 

A chemist, aged 24, had suffered ever since he was 2 years of age from infantile 
spinal paralysis of the right leg (chiefly of the crural region, but also of the sartorius 
and of the tibialis and peroneal regions with the exception of the tibialis anticus) and 
there was decided shortening of the leg ; from long standing in the laboratory and exces- 
sive use of the leg there was considerable hypertrophy, and simultaneously a persistent, 
painless, muscular contraction of the left leg. On standing, and when the muscles 
of the left leg were tense, the entire musculature formed waves, and when in the 
recumbent posture instantaneous and wavy contractions of varying intensity appeared 
in the muscles of the calf, the thigh, and also on the extensor surface. The electric 
contractility of the left side was normal ; the galvanic contractions were lightning-like. 
Galvanism and a course of treatment at W ildbad had a beneficial effect. But even 
after the patient changed his occupation the contractions continued for a long time, 
and when I saw him nine years later he stated that they had disappeared only three 
years previously. 

(2) (Ionic contractions in the region of the crural and obturator nerve 
are apparently of reflex origin, and are occasionally produced by irritative 
factors in the cavity of the pelvis. In 1874 I saw a girl who, after a gunshot 
wound of the pelvis from which the bullet had not been extracted, suffered 
from persistent clonic contractions in the muscles of the thigh of the same 
side. 

To this category probably belong the contractions noted in diseases of the 
sexual organs, of the bladder, and of the rectum, as well as in inflammations 
of the hip-joint. In a case of hernia described by Bernhardt in 1901, three 
weeks after a bilateral radical operation clonic contractions occurring about 
120 times per minute were noted in the adductor muscles of the right thigh. 

A. Eulenburg, Fr. Schultze, and Xiebergall have described analogous cases 
following exertion, long standing, or marching; clonic and tonic spasms of 
the ilio-psoas and rectus femoris, without assignable cause, have been reported 
in neurasthenics (Klemperer, Koch, and others). 

Occasionally, as I observed in a boy aged 10, the lightning-like contrac- 
tions of myoclonia are chiefly confined bilaterally to the muscles of the thigh, 
but the contractions may also be sometimes noted in the abdominal muscles. 

When paroxysmal and unilateral clonic contractions of the thigh appear, 
it should always be borne in mind that they may be due to cortical epilepsy. 



958 LOCALIZED SPASM 

In a case which I described in 1899, and in which Oppenheim found at the 
autopsy a left-sided tumor of the cortex of the brain, I saw rhythmical con- 
tractions of the right extensor quadriceps which took place at the rate of 20 
within 10 seconds. The muscles of the calf were also involved in the spasms. 
The attacks were at first confined to the right leg, so that the patient dragged 
the leg for one or two days after an attack. In subsequent attacks, the con- 
tractions were again in the right thigh, but now attacked also the right half 
of the abdomen and the right shoulder, the head was drawn to the right, the 
shoulder elevated, and well developed cortical spasms were produced. In a 
case of cortical epilepsy after trauma in a boy, aged 11, I saw contractions 
of the left thigh (extensor quadriceps) which occurred rhythmically but 
not very frequently in the abdominal muscles, the sternocleidomastoid, the 
platysma, the muscles of the shoulder and the pectoralis. Immediately after- 
ward there was flaccid paralysis of the left ankle and of the muscles which 
moved the toes. This form of cortical spasm in the leg is by no means 
common. 

In a case reported by me in 1902, I made a diagnosis of cortical epilepsy 
on account of paresis of the right leg, the foot reflex, and Babinskr's sign, 
although the attacks invariably began with contractions in the great toe of 
the right side, next in the leg, and occasionally also in the thigh, usually 
radiating no further. While, in such cases, hemiparesis and monoparesis are 
preceded by clonic contractions, these are much less frequently observed as 
a post-hemiplegic phenomenon. A case of right sided hemiplegia which Bern- 
hardt reported in 1891 is especially noteworthy because in its course a true 
plantar clonus of the foot appeared, and when the foot was in the equino 
varus position the muscles of the calf and the tibialis posticus contracted 
more than 200 times per minute. This plantar clonus was regarded as a 
variety of so-called post-hemiplegic chorea and athetosis. But only occasion- 
ally do slight clonic contractions of the extensors of the toes produce the so- 
called athetoid spontaneous movements which are observed in polyneuritis, 
and sometimes also in tabes. We find in literature reports of idiopathic and 
functional rhythmical contractions of some of the peroneal muscles, usually 
in young persons (Jobert, Bernhardt). 

(3) The very common and painful cramp in the calf of the leg, which 
is of brief duration, and usually occurs after exhaustion, but also under nor- 
mal circumstances and, as a rule, at night, is a tonic spasm, and is caused 
by any severe or unusual movement (plantar flexion), generally during sleep, 
and is limited to one side. The calf becomes as hard as a board, is very pain- 
ful upon pressure, and for a considerable time after. Whether varices play 
a role in its development is very doubtful. But it is certain that an abnormal 
blood mixture is a predisposing cause, because spasm of the calf of the leg is 
observed in chorea, in enteric fever, in diarrhea, in diabetes, in alcoholism 
[and gout], also after pregnancy in which, perhaps as a symptom of stasis, 
it is particularly common. Local pain favors the peripheral origin of these 
cramps. 

Much more rare are tonic, idiopathic, and extensive muscular spasms of 
the leg which chiefly occur in walking. A locksmith, aged 31, who came 
under my observation, suffered for six months when walking or when carry- 
ing loads from attacks of clonic spasm in the right tibial region which began 



CLINICAL VARIETIES 959 

with brief contractions of the flexors of the toes. In the attacks which I 
saw, the tendo Achillis was contracted to its maximum so that passive dorsal 
flexion of the foot was absolutely impossible. Besides this tonic spasm I saw 
brief contractions of the flexors of the toes, and as the spasm in the calf of 
the leg ceased after anodal galvanization these were also noted in the peroneal 
region. The attacks lasted almost three hours. The tendon reflexes were un- 
changed, but the mechanical irritability of the muscles seemed to be increased 
without increase of the electrical contractility. Under rest and galvanization 
for two months, the attacks became less numerous and of shorter duration. 
This case may be grouped with occupation spasms of the lower extremities such 
as Duchenne saw in a lathe worker, Eulenburg in silversmiths, Stuertz in 
tanners, and Schultze in dancers in consequence of toe-stepping. 

These cases of tonic spasm which appear on walking or similar move- 
ments must be differentiated from those described by Bamberger, Guttmann, 
Frey, and others as so-called saltatory reflex spasm. In this affection, when 
the foot is placed upon the floor, clonic contractions of the lower extremity 
are produced and cause the whole body to make a jumping movement. As 
the tendon reflexes in such cases are always greatly increased, Erb holds that 
an extraordinarily increased reflex irritability of the spinal cord is the cause. 
Oppenheim regards the disease as a rare form of hysteria. As a rule, the 
prognosis is favorable. 

(4) Several forms of tic impulsif of the lower extremities (walking and 
jumping tic) have been described by Meige and Feindel ; for instance, cases 
in which the foot is turned toward the other leg, cases of stamping tic, knee 
flexor tic, and jumping tic. They admit that tic of the lower extremity alone 
is very rarely met with. 

A girl, aged about 7 years, who had been treated by me for hysterical 
chorea, was again brought to me at the age of 14 because for several years 
she had hopped with the right foot while walking, and this movement had 
been constantly growing more frequent. After a few normal steps her gait 
would be interrupted by a kind of polka step with the right foot, this having 
the appearance of a voluntary movement. Faradization relieved it within 
eight days. But a year later she returned with marked tic impulsif, shaking 
of the head, bilateral nictitatio, etc. 

This varied realm of localized spasm has been discussed largely from the 
standpoint of my own experience rather than from the reports in literature. 
A bibliography of the subject will be found in the second part of Bernhardt's 
article, " Diseases of the Peripheral Nerves," in the second edition of Noth- 
nagel's collection which appeared in 1904, as well as in Binswanger's 
"Hysteria" (1904) in the same work, and in Meige and Feindel's "Tic, 
Its Nature and Treatment." 



THE PRESENT STATUS OF GRAVES' DISEASE 
(EXOPHTHALMIC GOITER. BASEDOW'S DISEASE) 

By A. EULENBURG, Berlin 

When the Merseburg physician, Karl von Basedow, about 1840 published 
in Casper's Wochenschrift his article, " Exophthalmos from Hypertrophy of 
the Cellular Tissue in the Orbital Cavity," he could scarcely have dreamed 
that he had immortalized his name — still less so that he had left to the world 
the difficult task of ascertaining the cause of the disease which was named 
after him. 

The nosographic priority and the perpetuation of the name will probably 
be upheld by German physicians. For although the celebrated Dublin clin- 
ician, Robert James Graves, had in 1835 recognized and described a disease 
which in England and America is called after him " Graves' disease," this 
consisting in a complication of goiter and exophthalmos (exophthalmic 
goiter), and the younger Charles Parry of Bath had reported in 1825 five 
cases of "enlargement of the thyreoid gland combined with enlargement or 
palpitation of the heart" (only one of which cases showed exophthalmos), 
Basedow undoubtedly deserves the credit of having first called attention to 
the triad of symptoms consisting of palpitation (tachycardia), enlargement 
of the thyreoid, and exophthalmos, and of having raised this syndrome from 
a mere coincidence to the dignity of a nosologic entity, to a clinical thera- 
peutico-pathologic conception. Naturally he gave us merely a clinical foun- 
dation; the , structure, which as we shall see is yet far from complete, is 
very different from anything the author of the first article (and of a second 
which appeared eight years later) could possibly have foreseen. Although 
we are still laboring over some of the problems of this remarkable symptom 
triad which he and many subsequent observers and investigators worked upon, 
yet in the course of time they have become more clear, and our insight into 
their nature, our recognition of the internal connection and the common root 
of the pathologic structure, has been greatly facilitated and is almost perfect. 
This at once becomes obvious if we cast a retrospective glance over the views 
concerning Basedow's (Graves') disease which in the course of time have 
been gradually evolved. This shows that, upon the whole, there are but three 
f imdamental views, and these have been periodically remodelled into different 
forms which determine or dominate our clinical conception, as well as our 
theoretical and practical views of Basedow's disease. Under a few speculative 
heads these have been massed into theories to which collective designations 
have been applied, hence we have at present hematogenous, neurogenous and 
thyrogenous theories of Basedow's disease. It is very instructive to note how 
960 



SYMPTOMATOLOGY 961 

these points of view, according to the difference in theory, all culminate in 
the predominant opinions which, even to-day, are incomplete and inconclu- 
sive^ — a circumstance which at first appears to indicate that each of them 
contains a kernel of truth. 

SYMPTOMATOLOGY 

Before discussing these questions, however, it is advisable clearly to define 
our conception and the nature of Basedow's disease, for our present position 
concerning actively discussed theoretic as well as practico-therapeutic ques- 
tions will naturally depend upon our conception of Graves' disease. As is 
well known, the original and apparently precise limitation of the symptom 
triad — cardiac palpitation (or, better, tachycardia), goiter and exophthalmos 
— in the course of time was subject to many changes, which sometimes tended 
to complete or transform, sometimes to limit or narrow, the pathologic pic- 
ture. Above all, a series of valuable and clinically remarkable differentio- 
diagnostic symptoms were subsequently included as common although not in- 
variable accompanying phenomena of these three cardinal symptoms. Among 
these were the eye phenomena known as Graefe's, Stellwag's and Moebius's 
symptoms; the muscular weakness, Marie's tremor of the members, the de- 
crease of galvanic resistance determined by Vigouroux and Charcot (which 
Martins, Kahler and myself designated as a rapid loss of power of resistance 
and a diminution of the relative minimum of resistance) ; the symptoms of 
rapid metabolism, the sweating, the gastric crises, the copious watery diar- 
rhea, the sensations of heat, the abnormal pigmentation and discoloration of 
the skin, leukoplakia, alopecia, amenorrhea and dysmenorrhea, polyuria, al- 
buminuria and alimentary glycosuria which Chvostek first recognized as very 
common (not so regarded by H. Strauss), the typical emaciation and cachexia; 
in a word, that entire group of symptoms indicative of neurasthenia and 
hysteria, and consisting of nervous psychical disturbances (occasionally de- 
veloping to complete psychoses!), which became so predominant in the patho- 
logic picture that some authors on this account regarded Graves' disease as 
in the main merely a localized and peculiarly modified form of hysteria. On 
the other hand it did not escape the attention of the earliest observers of the 
disease that one or even two of the three " cardinal symptoms " may for a 
time or permanently be absent — in cases in which, otherwise, the clinical 
course was typical of Graves' disease. Exophthalmos is most often absent, 
more rarely goiter and tachycardia — in very rare cases exophthalmos alone 
is found, but it then manifests the characteristics of the exophthalmos of 
Graves' disease (so-called "exophthalmos cachectique," after Fischer). Nat- 
urally it must be observed whether the absence of individual and leading 
symptoms is only transitory, whether it is observed in an early stage of the 
disease, or whether it persists throughout the entire course of the affection so 
that the pathological picture is incomplete ("abortive" or "rudimentary" 
form). It has become the custom, following Trousseau, to designate these 
cases by the term this author invented, " maladie dite fruste par 1' absence du 
goitre et de l'exophthalmie," and these cases were later designated by Char- 
cot as the " forme fruste " of Graves' disease. The expression " fruste " 
(which is derived from coins whose impress is indistinct from use) does not 



962 PRESENT STATUS OF GRAVES' DISEASE 

actually apply to the cases of rudimentary development. It is better adapted 
to those in which, in the course of the disease, spontaneously or under thera- 
peutic influence, the chief symptoms either disappear or improve and thus 
obscure the typical character of the affection. At all events this teaching of 
a " forme f ruste " has led to many errors, and, in particular, cases have been 
designated as Graves' disease and treated by operation in which there is grave 
doubt as to the diagnosis. Also under the modern appellations of " sec- 
ondary " or " symptomatic " Graves' disease, in contrast to primary or gen- 
uine, " iodin thyreoidism " and " iodin Graves' disease," etc., many a sus- 
picious case may have been treated. 

DIAGNOSIS 

How shall we meet these difficulties in diagnosis ? We must start from the 
fact that there may be either constant, specific, or pathognomonic symptoms 
of Graves' disease — that, therefore, the diagnosis can never be based upon 
the presence or the absence of a single symptom; on the contrary, when the 
cases are at all doubtful the diagnosis should be based upon the general 
habitus, or the entire course of the disease. Compensation for the three 
cardinal symptoms by others perhaps less important may be of value in 
determining the signs of the disease. If, for instance, there is no trace of 
exophthalmos but very conspicuous psychical (neurasthenic or hysterical) 
symptoms and secretory trophic disturbances, such as sweating, diarrhea, 
amenorrhea, emaciation, alimentary glycosuria, edema, etc., or the distinctly 
developed symptom of a galvanic loss of resistance, we can hardly doubt that 
the case is one of Graves' disease; if, on the other hand, neuro-psychical 
and secretory trophic anomalies common in this affection are absolutely and 
permanently absent, even though we note the concurrence of the three cardinal 
symptoms, and especially if one of these be absent, the diagnosis cannot be 
regarded as positive. 

It is evident that the diagnosis does not depend so much upon the mere 
presence of the most important symptoms of Graves' disease as upon the char- 
acteristic manner of their appearance — a circumstance which in modern 
casuistry is not always sufficiently appreciated. The mere presence of goiter 
is not enough but it must show the specific composition of the goiter of 
Graves' disease, must be of a soft, elastic consistence, must be almost uni- 
formly distributed, and accompanied by thrills, pulsation, and a loud, roaring, 
often systolically increased murmur — briefly, by all the peculiarities which 
from the onset clinically distinguish the hyperemic, vascular goiters due to 
hypertrophy and hyperplasia, attended by degeneration of the thyreoid gland, 
from cystic degeneration and malignant tumors. I do not here consider the 
question in how far such differentiations are justified from the standpoint 
of the pathologic anatomist — which, from the recent findings of Lubarsch, 
Renant, Goffroy, Greenfield and others, may be doubted, inasmuch as the 
goiter of true Graves' disease is to some extent a simple vascular goiter un- 
dergoing the hyperplastic change of the thyreoid, and to some extent an 
"autonomous neoplasm" (Lubarsch) with the formation of irregular, greatly 
dilated cavities which usually contain no colloid and very little or no gran- 
ular and coagulated fluid. Nor does the mere demonstration of exophthalmos 



THEORIES CONCERNING GRAVES' DISEASE 963 

suffice, but this must be bilateral and uncombined with dilatation of the 
pupils, while the coincidence of certain other disturbances of the eye, of 
Graefe's, Stellwag's or Moebius's sign, as well as persistent nutritive dis- 
turbances of the bulbus, may under some circumstances help to verify the 
diagnosis. [Unilateral thyreoid enlargement and unilateral exophthalmos 
are not rare in undoubted cases. — Ed.] Finally, tachycardia and cardiac 
palpitation in themselves are not sufficient, but we may expect with some 
degree of regularity not only a paroxysmal but a persistent and almost hab- 
itual appearance of this cardiac neurosis which may exist with a normal or 
but slightly altered blood pressure, 1 with or without anemic murmurs or other 
anomalies in the vascular apparatus, and with the signs of consequent cardiac 
insufficiency, dilatation, etc. The other symptoms, which, like the tremor, 
the mental state, etc., reveal to the eye of the practised observer much that 
is characteristic, are analogous, and in doubtful, atypical, and rudimentary 
cases often aid materially in the diagnosis. 

THEORIES CONCERNING GRAVES' DISEASE 

With these preliminary remarks we turn to the consideration of the three 
principal theories in a historical sense, the earliest of which is the " hema- 
togenous theory " which emanated from Basedow himself, and corresponded to 
the humoral pathological view of that period, that the disease was due to a 
faulty, anemic, and chlorotic admixture of the blood (accurately depicted in 
his second report in Casper's Wochenschrift, 1848). Subsequently many 
authors concurred in this opinion or have held similar ones (Helfft, Lubarsch, 
Cooper, L. Gros, Prael, Fischer, Hiffelshein, Mackenzie, Taylor, Warburton 
Begbie). Among the arguments in its favor was the overwhelming frequency 
of the disease among females, its usual appearance during youth, its very fre- 
quent association with menstrual disorders, particularly with amenorrhea, the 
favorable influence of pregnancy in isolated cases, and later reports of its 
frequent coincidence with enteroptosis (Schwerdt). Other observations, such 
as the hereditary tendency of the disease, its often acute development after 
trauma, severe somatic and psychical shock, and the like, might with equal 
justice point to a neurotic origin of the affection. Still others, such as its 
combination with severe intestinal affections which has recently been much 
emphasized, with jaundice, etc., might indicate an autointoxication from the 
intestinal tract changing the secretory activity of the thyreoid, which we shall 
later consider. At all events, the arguments which favor the hematogenous 
theory are opposed by many others, and that an anemic, chlorotic, altered 
blood mixture is the etiologic cause is at least exceedinglv doubtful. Aside 
from the facts that the disease may occur in men (although decidedly less 
often), that it is met with in children and in women during the climacterium, 
that pregnancy, as a rule, has an unfavorable rather than a benign effect 
upon the disease, this hypothesis does not even explain the three chief symp- 
toms, since exophthalmos does not appear in ordinary chlorosis, goiter is 
rarely present, and when it is it differs decidedly from the goiter of Graves' 
disease, and tachycardia is neither markedly developed nor so persistent. 

i Compare " Die Untersuchungen von J. Donath," Zeitschrift f. Tclin. Med., Bd. 48, 
Heft 1 und 2. 



964 PRESENT STATUS OF GRAVES' DISEASE 

The other symptoms of Basedow's disease show no intimate relation to the 
ordinary symptoms of chlorosis and chlorotic anemia. Moreover, Graves' 
disease attacks those who are neither anemic nor chlorotic, and examinations 
of the blood carefully and frequently made according to modern and exact 
methods, the estimation of the hemoglobin, the counting of the blood-cor- 
puscles, etc., have given varying and contradictory results. Hence, according 
to the earlier views, an anemic and chlorotic state of the blood cannot be re- 
garded as the true cause, the specific pathogenic agent; there must still be 
an unknown quantity. On the other hand, it shows that the hematogenous 
theory cannot be completely set aside, but that it must be regarded as a 
factor in the newer conception of a thyreogenous autointoxication, and must 
be included within this. 

The neurogenous theory may, in a certain sense, be attributed to the 
earlier authors, particularly to the Driburg physician, Briick, who in Casper's 
Wochenschrift (in 1840 and also in 1848) also called attention to the simi- 
larity of these nervous disturbances to hysteria, and even disignated the exoph- 
thalmos in Basedow's disease as "buphthalmos hystericus." But, instead 
of following further this true and fruitful conception, which only a few at- 
tempted, authors lost themselves in byways, such as the one-sided and ex- 
aggerated pursuit of animal experiments which then and long afterward 
afforded abundant opportunities for application to human pathology. Al- 
though Benedikt more than thirty years ago declared very ingeniously that 
nothing was so dangerous as " to find a narrow physiologic bridge just wide 
enough to stand upon," yet, for a long time many authors considered the 
sympathetic bridge, others the vagus bridge, and still others the bridges in- 
cluding the medulla, to be broad and firm enough to support weighty and 
cumbersome theoretic loads. As a matter of fact, in a retrospective survey 
after this lapse of time, we can scarcely suppress the feeling that the greatly 
lauded advance of experimental physiology at that period occasionally ex- 
erted a most unfavorable influence, pathologic facts frequently being dis- 
torted into one-sided and erroneous conclusions which brought many disagree- 
able disappointments to their enthusiastic adherents and sympathizers for 
which physiology was not to blame! Thus was evolved, not a single neurog- 
enous theory of Graves' disease but, on the contrary, a consecutive series of 
theories which disproved one another, and which we may designate as the 
sympathetic theory, the vagus theory, the spinal, bulbar, and cerebral theories, 
last of all the constitutional, neuropathic, or neurosis theory. I shall not ex- 
plicitly present these "theories," most of which, like other speculations, ter- 
minate in unsolvable contradictions, but shall limit myself to a rapid enu- 
meration of their fundamental principles (some of which are accepted even 
to-day). 

With the exception of the unfortunate view of Koeben (1855) which was 
based upon the irritation of the sympathetic by the pressure of a preexisting 
goiter, the sympathetic theory depended upon Claude Bernard's celebrated 
experiment upon the cervical sympathetic. Aran (1860) and Trousseau 
(1862) were pioneers in the study of the symptoms of Basedow's disease, but 
conflicting opinions arose because some of the symptoms appeared to be due 
to experimental irritation, others to the division of the sympathetic of the 
neck; for instance, tachycardia and exophthalmos were produced by irrita- 



THEORIES CONCERNING GRAVES' DISEASE 965 

tion, arterial dilatation and goiter by section. Many ingenious attempts were 
made to solve these contradictions by proving either that tachycardia and 
exophthalmos were symptoms of paralysis, or, inversely, that goiter was the 
result of irritation (from stimulation of the vasodilator nerves, according to 
Benedikt and Ch. Abadie). All this reasoning was more or less artificial, 
and these forced hypotheses led to nothing convincing. Geigel (1866) tried 
another method, for, instead of utilizing the sympathetic border column in 
explanation, he took the vasomotor and oculo-pupillary centers of the sym- 
pathetic of the neck in the cervical and upper thoracic cord, and postulated 
a paralytic condition in the one, and an irritation in the other of these centers, 
which, however, by no means solved the difficulty but merely transferred it 
to another point. Others, in opposition to clinical proofs, assumed irritative 
and paralytic conditions in succession (Horner and Nicati) or they used the 
vagus to explain the tachycardia resulting from paralysis of the regulatory 
pneumogastric fibers, as was previously done by Friedreich in paralysis of 
the vasomotors of the coronary arteries running in the sympathetic. When 
Filehne's experiments were made known (1879), and were confirmed and 
supplemented by Durdufi, and subsequently by Bienfait and Troitzki, every- 
thing seemed for a time to favor the bulbar theory, especially of the areas 
of the fourth ventricle and corpora restiforme, injury to which caused an in- 
crease in the pulse rate (from exclusion of the regulatory pneumogastric 
center) as well as moderate and often unilateral exophthalmos and — in iso- 
lated cases — hyperemia of the thyreoid. A few positive autopsy findings 
(Mendel, Leube, later Kedzior and Zanietowski, 1 Dana 2 — to which numer- 
ous negative ones were opposed, as was the case with the preceding sympa- 
thetic and pneumogastric hypotheses — appeared to confirm the bulbar theory, 
which for a considerable time found some noted advocates (such as Brissaud 
at the Bordeaux Congress, 1895). But the inconstancy of the results of 
investigation, the inequality of the points of attack found by different ex- 
perimenters, and the want of analogy with most of the symptoms of Graves' 
disease, led to reflection; for example, it was noted that true goiter never 
developed in the animals experimented upon! 

The results of this theory did not come up to the expectations, and re- 
peated attempts to trace the origin of the disease to the cerebrum, and per- 
haps even to localize it in the cortex, also failed. Careful observation of the 
clinical facts and their consideration made it certain from the onset that all 
attempts at localization within the central nervous system must necessarily 
fail to explain the manifold symptom-complex of exophthalmic goiter; hence, 
as was clinically justifiable, nothing remained but the constitutional neuro- 
patliic or neurosis theory mentioned at the beginning of this article, and in 
various quarters this was accepted — lately with special force by Buschan. It 
cannot be denied that there is much to favor this theory; among other con- 
siderations the previously mentioned etiologic factors, the hereditary and 
family occurrence of Graves' disease, its acute development after .trauma and 

i Neurol. Central!)!., 1901, Nr. 10, p. 438. " Atropine des linken Corpus restiforme. 
Bei Lebzeiten rechtseitige Strumectomie und Ligatur der linken Schilddriisenar- 
terien." 

2 New York Med. Journ., June 14, 1903, " Changes in the Bulbar Nuclei, especially 
of the Pneumogastric." 



966 PRESENT STATUS OF GRAVES' DISEASE 

severe psychical shock, especially after fright, its coincidence with other con- 
stitutional neuropathies, with hysteria, neurasthenia, hemicrania, epilepsy, 
chorea, paralysis agitans, etc., and even with conspicuous functional psychoses 
(melancholia, mania). Nevertheless, many of these conditions can be other- 
wise explained; certainly the neurosis theory does not embody the "entire 
and full " truth ; like all other neurotic hypotheses it leaves unsettled the ques- 
tion as to the origin of the disturbance in the mechanism of the nerve, the 
exciting and specific cause of the disease, and the pathogenic agent to which 
the nervous system succumbs. Here, as in the hematogenous theory, we are 
confronted by an unknown X whose satisfactory solution appears to be prom- 
ised by the third theory, the thyreogenous. 

This theory, in so far as it attempts to deduce the other symptoms of the 
disease from a previous and primary local affection of the thyreoid gland, 
is not of recent date. In the form of the mechanical thyreoid gland theory 
it was first proposed by Koeben in 1855, then in the sixties by French authors 
(especially Piorry and Cros) ; according to this view, tachycardia and exoph- 
thalmos are pressure phenomena due to the enlargement of the thyreoid 
gland, but the obvious and just objections to this theory of course made it 
untenable. What, however, may be justly alleged against this mechanical 
theory of the thyreoid gland nowise applies to the later chemico-toxic (auto- 
intoxication) theory we exclusively hold at this time. On the contrary, this 
is absolutely the product of morphologic, physiologic and clinico-therapeutic 
facts which, as is well known, have recently led to a complete change of 
opinion regarding the function and importance of the thyreoid gland in the 
normal organism as well as its functional anomalies in certain severe and 
general cachexias.* The important relations of these functional anomalies to 
the pathologic conditions of cachexia strumipriva and myxedema were made 
clear in 1883 by the pioneer labors of Kocher and others, and, what is espe- 
cially fortunate and invites further research in this realm, these experiments 
yielded valuable therapeutic results in the form of organotherapy. These 
facts in regard to cachexia strumipriva and myxedema were the basis of the 
new chemical thyreoid gland theory of Graves' disease. Gauthier (1886) ap- 
pears to have been the first to emphasize that the disturbed chemism of the 
thyreoid gland in preexisting goiter is an active factor in the development of 
Basedow's disease, especially the cachexia; but this was purely incidental and 
led to no decisive conclusions in a pathologic respect. On the other hand 
Moebius, who designated Basedow's disease as an " intoxication of the organ- 
ism from the pathologic activity of the thyreoid gland" in laying the founda- 
tions of his view called attention to the contrast which appeared to exist 
anatomically as well as symptomatologico-clinically between myxedema (as 
well as cachexia strumipriva) and Graves' disease. In the one case there is 
aplasia and atrophy of the thyreoid gland, in the other enlargement and 
hyperplasia; in the one case there is slowing of the pulse, narrowing of the 
palpebral fissure, an impairment of the psychical functions, an increase in 
weight, coldness and thickening of the skin ; on the other hand there is tachy- 
cardia, exophthalmos, psychical excitement, general emaciation, loss of struc- 
ture, and increase of the cutaneous temperature. This might be further 
complemented by the differences in regard to the excretion of phosphoric 
acid proven by Wilhelm Scholz (working under Kraus) between myxedema 



THEORIES CONCERNING GRAVES' DISEASE 967 

and Graves' disease. Nevertheless such a diagrammatic comparison is always 
somewhat artificial, inasmuch as the almost invariable and cardinal symptoms 
of one disease are offset by the inconstant and secondary symptoms of the 
other. But, aside from this, there are many other difficulties that preclude 
a satisfactory understanding of Graves' disease upon the basis of this theory 
which appears so plausible. 

In cachexia strumipriva and in myxedema it may be regarded as certain 
that atrophy or total absence of the gland and the consequent absence of the 
glandular secretion bear a direct causal relation to the symptoms of the dis- 
ease, presumably because the absence of the product of the normal gland, 
which is necessary for the integrity of all the vital functions, or a certain 
auto-intoxication produced by the intermediary metabolism of the body, 
changes or neutralizes these symptoms. The latter view, according to which 
the thyreoid gland neutralizes the action of toxic products, is regarded by 
some authors (F. Blum) to have a better foundation than the experimen- 
tal theory, while the former is clinically more convenient, but is objec- 
tionable because the giving off of a glandular secretion to the circulation has 
not yet been proven. In the study of Basedow's disease it is certainly very 
difficult to understand the symptoms while we hold to the fact of a hyperemia 
(and subsequently hyperplasia) of the thyreoid gland and a correspondingly 
increased glandular secretion. We cannot at once determine how an increase 
in function and an increased secretion of the thyreoid gland cause " hyper- 
thyreosis," and have such a damaging effect upon the organism as to pro- 
duce the severe cachexia peculiar to Graves' disease; for, in contrast to the 
influences which produce cachexia strumipriva and myxedema, nothing comes 
into question but an increased formation of those specific products of secre- 
tion which are said to promote health, or to have an antitoxic effect, or to 
increase an antitoxic effect. The similarity between presumable intoxication 
from excessive doses of thyreoid gland preparations and the symptoms of 
Basedow's disease is too vague. A sharp contrast between Basedow's disease 
and myxedema, anatomically as well as clinically, appears to be contestable 
since resemblances and differences between these conditions, as well as transi- 
tions into each other, may be recognized; even the secondary development 
of myxedema, with or after preceding Basedow's disease, has been observed 
in a few cases (Kowalewski, Sollier, Baldwin, Babinski, v. Jaksch, Hirschl, 
Faure, Ulrich and myself). On the other hand, this circumstance points to 
the fact that apparently complicated relations exist between the two forms 
of the disease. At all events, we do not attain our goal with the mere 
assumption of an increase in function and a quantitatively increased secretion 
of the thyreoid gland in Basedow's disease, but must also assume a qualita- 
tively changed secretion, and resort to other hypotheses to explain it. Obvi- 
ously an anomalous nervous influence upon the thyreoid gland should be held 
responsible for this, similar to certain anomalies of secretion of the salivary 
glands (for example, the paralytic saliva of the submaxillary gland after 
division of the chorda) which has been experimentally proven. This view is 
contradicted by Hiirthle's investigations into the thyreoid gland, which dem- 
onstrate that experimental stimulation of the nerves in animals — faradic irri- 
tation of the isolated nerves of the gland or of the pneumogastric and sym- 
pathetic trunks in the thoracic cavity — exerts no appreciable influence upon 



968 PRESENT STATUS OF GRAVES' DISEASE 

the activity of the gland. These results can hardly be regarded as final ; on 
the contrary, later experiments favor the view that in the pathogenesis of the 
altered glandular function — in so far as the cardio-vascular symptoms are 
concerned they demonstrate the influence of an activity of certain nerve tracts 
(according to E. Cyon) — the accelerating fibers running in the sympathetic, 
those from the vagus, and the regulatory fibers from the depressor especially 
come into consideration. The " thyreogenous " theory in this respect calls 
for further neurogenous confirmation and completion. 

But the experiments of Hiirthle offer the possibility of another explana- 
tion. According to these, the physiologic as well as the pathologic production 
of a secretion in the thyreoid gland, of a definite composition of the blood 
flowing to and circulating in the gland, depends upon the absence or de- 
ficiency of certain products in the blood. From this the idea may have 
originated that in Graves' disease certain primary changes in the composition 
of the Hood, the nature of which is at present unknown, produce a glandular 
stimulation, acting upon the glandular function, the secretion, the growth 
of the follicles, upon the degeneration in the cellular processes, and thus 
produces the peculiar, specific, and pathogenic intoxication of the secretion. 
Quantitative as well as qualitative changes in the composition of the blood 
of the thyreoid gland probably here act together. The quantitative effect is 
shown at once in the marked arterial congestion and hyperemia of the gland. 
But a simultaneous qualitative change in the composition of the blood favors 
certain facts ; not only, as has been previously stated, that the blood mixture 
resembles chlorosis, but also that the appearance of the affection combined 
with acute infectious diseases or other debilitating conditions, such as hemor- 
rhages, marked loss of fluids, etc., as well as its connection with severe gastro- 
intestinal disturbances and jaundice, which has lately been emphasized by 
various authors, favor this view. The last-named conditions remind us of 
Htirthle's experiments, according to which the tying of the biliary channels 
and the jaundice thereby produced always changes the activity of the thyreoid 
gland, and especially increases colloid formation as shown by the abundance 
of colloid cells and of colloid substance within the lymph spaces and in the 
epithelial cells. Hiirthle maintains that there is no other explanation than 
the following: The constituents which pass into the blood in biliary stasis 
produce an irritation of the gland which stimulates it to the increased pro- 
duction of colloid. 

That such an increased colloid formation may be combined with an in- 
creased excretion of a toxic product, a toxalbumin — the so-called thyreoproteid 
of Notkin — seems not unlikely from Notkin's researches regarding this sub- 
stance. Usually thyreoproteid, which originates from the blood by a normal 
enzyme of the gland — through the " collective substance " (Blum) in which 
the iodizing process of the thyreoid gland plays a particular role — is trans- 
formed and rendered permanently innocuous. Why this does not occur in 
Graves' disease, or to only a decreased extent, may be seen from Hiirthle's 
investigation as well as from some confirmatory reports of French authors. 
Besides the change in the production of the secretion, an altered and in- 
creased discharge of the secretion from the gland and its direct transference 
to the circulation by venous absorption, avoiding the lymph tract, appears 
here to play a part. Whether the colloid substance collected in the dilated 



THEORIES CONCERNING GRAVES' DISEASE 969 

intercellular spaces leaves the gland by the efferent lymph vessels or is in 
part directly absorbed by the veins of the thyreoid gland is still questionable 
— according to Hurthle's investigations the latter view is by no means im- 
probable. If we consider that, as Renaut has shown, pathologic changes 
often destroy to a great extent the lymph tracts of the gland and that, like 
the arteries, the capillaries and veins of the gland are also increased and 
dilated (the veins even more than the arteries, Dinkier states) — this further 
confirms such view. We may therefore with some justification conclude that 
in Graves' disease the secretion formed in abnormal amount and of altered 
composition (which, under normal conditions, exerts a partial antitoxic in- 
fluence), rapidly and without passing into the lymph tract at once enters the 
circulation and thus produces an increased toxic action. 

Here, as I have previously indicated, the " hematogenous " and the recent 
" thyrogenous " views of exophthalmic goiter meet, and one appears to be 
the indispensable complement of the other, although the finer threads of 
communication cannot at present be positively recognized. That the glandular 
secretion is not only quantitatively but also qualitatively abnormal owing to 
the altered blood composition is not only shown by the results of experiment 
but there are clinical points of support for this view. I refer to Baumann's 
proof of the decreased amount of iodin in the goiter of Graves' disease. The 
experiments of Chevalier, who in injecting urine into rabbits obtained a 
threefold greater toxic action with that from patients with Graves' disease 
than with normal urine, as well as the demonstration by Boinet, Silbert, and 
Bienfait of several active toxic ptomains in the urine of Graves' disease, may 
be utilized in the same clinical demonstration. 

Finally, the question must be propounded, Upon which constituents of the 
organism, which organs or systems, does the toxic material generated by the 
thyreoid gland exert its pathologic effect? Despite the fatty atrophy of the 
voluntary muscles lately demonstrated in autopsies by Askanazy, there is 
hardly sufficient evidence to enable us to recognize a specific muscle poison, 
as Lemke attempted to do when explaining the cardiac palpitation called 
" tremor cordis " and exophthalmos. This view is opposed by the circum- 
stance that in the development of exophthalmos the exterior muscles of the 
eye are not involved, for, as has been demonstrated in total ophthalmoplegia 
externa, the position of the bulbus in the orbit, as regards the frontal plane 
is entirely irrelevant (A. Fiirst). On the contrary, the organ immediately 
attacked by the poison must be regarded as the nervous system, hence we must 
assign to neurogenous theories the place which they properly occupy in the 
pathogenesis of Graves' disease. In the thyreoid gland which functions nor- 
mally we probably have an effective protective organ for the central nervous 
system. When this antitoxic protective action is absent or diminished it is 
quite proper to assume that the intoxicating effect of the product exported 
by the gland implicates more or less severely all parts of the nervous system 
but chiefly the nerve centers, especially those in the cortical regions which are 
endowed with higher psychical activity, and thus produces the picture of a 
neurasthenic-hysteric neurosis or neuro-psychosis. Xotkin has attempted to 
prove that the previously mentioned " thyreoproteid " has first a stimulating 
and then a paralyzing effect upon the central nervous system. The first cause 
in the pathogenesis of Graves' disease would therefore be the abnormal quan- 



970 PRESENT STATUS OF GRAVES' DISEASE 

titative and qualitative composition of the blood circulating in the thyreoid 
gland; the second, the abnormal secretion by the thyreoid gland of a specific, 
pathogenic, intoxicating, glandular product; the third, the neurosis or neuro- 
psychosis depending upon autointoxication and subsequently upon cachexia 
which is so frequently met with in severe forms of exophthalmic goiter com- 
bined with a more or less advanced local affection of the thyreoid gland. 

TREATMENT 

These theoretic views are merely provisional, but in applying them to 
practice (in this case therapeutic practice), which is the best test of every 
theory, a comprehensive and unprejudiced observation of the natural course 
of the disease and a varied treatment are the necessary prerequisites, but these 
conditions are not often fulfilled. Those who love theory, even in thera- 
peutics, are prone to regard everything from the point of view of this theory, 
and, vice versa, any one inclined to a one-sided and uncritical therapeutic 
method will readily find a theory to adjust to his method. In the treatment 
of Graves' disease we must guard against such an over-estimation of methods 
based upon theoretic conceptions. On the other hand, the comparatively brief 
history of this disease convinces us that each of the previously described 
theories has affected the therapy, and has enriched it with more or less valu- 
able curative measures some of which at least continue to be of use. 

The hematogenous theory, according to which we first of all endeavor to 
improve the pathologically altered blood and the general constitution, sug- 
gests the employment of those drugs known since remote times to be "gen- 
erally roborant " in anemia and chlorosis, such as preparations of iron and 
arsenic, and including mineral waters containing these substances, as well 
as quinin, caffein, ergotin, strychnin, etc.; naturally the "heart tonics," 
such as digitalis and strophanthus, have been less employed because they did 
not, as a rule, have the desired effect of slowing the heart. However, accord- 
ing to the clearness of the indication for these remedies, we will often be 
unable to do without digitalis or strophanthus, given periodically and usually 
with benefit, in the distressing and long-continued attacks of violent cardiac 
palpitation, as well as in advanced cases of severe cardiac insufficiency with 
venous stasis, dropsy, and insufficient diuresis. Lately sodium phosphate and 
sodium salicylate have been especially advised by French authors. 

Much more beneficial than most drugs are the dietetic and physical 
methods of treatment which are employed for the same general reasons — 
to increase the constitutional energy and to change the amount and the com- 
position of the blood. I need only refer to the numerous cases in which 
astonishing results have often been attained by certain dietetic cures (dry 
diet, vegetarian diet; under some circumstances over-nutrition), also by cli- 
matic treatment (prolonged residence even during the winter at high alti- 
tudes although not exceeding 900 meters), as well as by different balneologic 
methods. Winternitz, a past master in this realm, expressed himself a few 
years ago concerning the excellent results of a rational hydrotherapy, strictly 
individualizing. As is well known, applications to the heart or nape of the 
neck of water at a temperature of 50° F., or ablutions of the same tempera- 
ture, often act as a substitute for digitalis. The general effect of these 



TREATMENT 971 

curative methods can be explained according to very different points of view, 
therefore adjusted to different theories, but in many cases, fortunately, there 
can be no doubt of their efficacy or of the cures resulting from them. It is 
well to remember this, because of the too eager and one-sided operative treat- 
ment now so much in vogue. 

Neurogenous theories have particularly enriched the therapy of Basedow's 
disease by the recent addition of electrotherapy and psychotherapy, methods 
whose importance and relative usefulness cannot be denied, although in this 
realm a correct estimation of what has actually been accomplished can be 
arrived at only with great difficulty. Although by some authorities it was 
stated a few years ago that electrotherapy in general did not fulfil the ex- 
pectations that had been created, from my own experience of many years 
I can admit this only with certain limitations. I shall, however, not enter 
into this discussion, nor shall I analyze critically the different methods of 
applying electricity, the question of faradization and galvanization, of the 
peripheral and central, the local and general application of currents, of 
franklinization and arsonvalization, or the various forms of hydro-electric 
baths such as monopolar and dipolar, the alternating current bath, the four 
cell bath, etc., as well as the " electromagnetic systems " which have lately 
been proposed. This is, as the old Colonel in Fontane's " Effie Briest " said 
when difficult and dangerous questions arose, " a wide field," designed for 
individual habits and hobbies, an extensive playground for all time. Under 
some circumstances, however, these methods do not deserve the condemnation 
which has been meted out to them; skilfully and wisely used, and in a pal- 
liative, symptomatic, and even curative respect, they may achieve obvious 
results. Of problematic value, however, is another fruit of the neurogenous 
theory, particularly of the earlier sympathetic view promulgated about a 
decade ago (1895) by Jaboulay, and endorsed by Jonnesco and others, 
" cervical sympathectomy " ; that is, the resection of the cervical sympathetic, 
either of the upper and middle cervical ganglion with the nerves connecting 
them, or also of the lower cervical nodes, therefore the extirpation of the 
entire cervical sympathetic column. The operation which is undoubtedly 
based upon incomplete and absolutely false theoretic deductions has not pro- 
duced the expected results in practice, but has repeatedly resulted in death, 
the cause of which, as of the sudden deaths after removal of goiter, remains 
in doubt (such a case, which occurred a few hours after a " successful " 
operation, has lately been reported by Deshusses x ). Stretching of the cervical 
sympathetic, practiced by Jaboulay, is not free from danger. 

In considering what we owe to the modern application of the thyreogenous 
theory (chemico-toxic or autotoxic) to treatment, two things must be borne 
in mind: First, the different applications in this greatly disputed realm of 
diseases of organotherapy which changes even from day to day; second, local 
operative treatment of the primarily diseased gland by a partial removal 
of the goiter, " exothyreopexy," and by tying the arteries of the gland. An- 
other method of treatment, much employed a number of years ago, and 
which gave such surprising results in myxedema and cachexia strumipriva, 
consisted in the internal administration of older preparations of the thyreoid 

i Journal des sciences medicales de Lille, 1902, Nr. 52. 



972 PRESENT STATUS OF GRAVES' DISEASE 

gland; following the assumption of a mere hypersecretion of the thyreoid 
gland, this appeared irrational or contradictory, while, under the assumption 
of a simultaneous and qualitative change in secretion it appeared to a certain 
extent justified, especially in the form of iodothyrin, and by the proof of a 
decrease of iodin in the thyreoid in Graves' disease was placed upon a firmer 
theoretic foundation. However, most of those who employed " iodothyrin " 
and originally eYpected great things of it, must have desisted from its use, 
at least in Graves' disease, as the expectations which had been raised were in 
no sense realized. The same is no less true of the proposed treatment with 
other glandular preparations, with thymus extract, ovarin, spermin, bile, etc., 
as well as with " protylin" (Roche), a non-toxic, phosphorus and albumin 
preparation artificially produced and much employed of late (phosphorus in 
a completely oxidized form included in the albumin molecule) ; according to 
Kocher this was said to be a specific in hyperthyreosis, the value of which 
in Basedow's disease, according to my experience, is scarcely greater than 
that of any good nutritive preparation. The last few years have given us 
various organotherapeutic specialties which, contrasted with the old thy- 
reoidin, etc., are not employed as a substitute for the absent enzyme of the 
gland, but as a sort of specific antitoxin, antithyreoidin. First to appear, 
and not without the necessary advertising, were preparations under the name 
of Basedowsan, rhodagen, etc., which were very expensive, but of little benefit 
in Basedow's disease. Then, upon the advice of Lantz, the milk of thyreoidec- 
tomized goats, in which the newly formed protective substance was said to be 
excreted, was administered to patients with Graves' disease. Recently, under 
the advice of Moebius, one of the great pharmaceutical firms in Germany 
has produced a thyreoid serum which is called "antithyreoidin Moebius." 
It is the blood serum of thyreoidectomized animals (sheep) from which, about 
six weeks prior to the first venesection, the thyreoid gland had been extir- 
pated. The preparation introduced into commerce is mixed with a 0.5 per 
cent, carbolic acid solution, and shows an apparently perfect stability; it is 
sold in sealed glass tubes containing 10 c.c. ; the price is $1.50. As the most 
common mode of administration is gradually to increase the dose, and conse- 
quently quite large doses are given (4.5 three times daily!), the contents of 
a bottle do not last very long and the method becomes expensive. This would 
be of little importance if the results were certain and the benefit greater 
than with other non-specific curative methods. But in a number of selected 
cases treated in this way for a long time, some mild and early cases, some 
severe ones in later stages, I have not been convinced of any reliable, certain, 
or specific effect from this antithyreoidin. In 3 of 6 cases I saw a remarkable 
decrease in the size of the goiter (in a recent and but slightly developed case 
it almost disappeared), but the other symptoms of the disease, especially the 
cardiovascular disturbances, were influenced but slightly or not at all. In 
one case these temporarily decreased but there was later a decided relapse; 
in another case there was an increase of tachycardia with marked irregularity 
of the cardiac action and aggravated dyspnea. In the other cases there was 
absolutely no result. As to the mode of administration, the remedy should 
be given internally in gradually increasing doses, beginning with 0.5 (there- 
fore 10 drops) three times daily; according to the advice of Schultze's, 1 which 
i Miinchener med. Wochenschr., 1902, Nr. 20. 



TREATMENT 973 

I found valuable, it is best taken in raspberry syrup or sweet wine. As a rule, 
I have increased the dose by 5 drops three times daily every third day, until 
it has reached 2.0 (40 drops) three times daily, after which I decreased it 
in the same way, and after a short or long discontinuance I have for some 
time given medium doses (1.0 three times daily). I have seen no disagree- 
able effects from this method, but, unfortunately, neither have I seen the 
curative results reported by different authors (for example, Eosenfeld 1 ). 
From this point of view, therefore, thyreogenous treatment is not a satis- 
factory therapeutic resource. Frank and impartial consideration of the 
operative treatment of goiter, much resorted to for about fifteen years accord- 
ing to the previously described methods, by no means confirms the brilliant 
expectations which it raised, which were greatly exaggerated by its surgical 
adherents, a few of whom were inclined to regard this local operation as a 
sovereign cure for Graves' disease, and to relegate the affection entirely to 
the domain of surgery. As upon former occasions, I must again express my 
doubts of the correctness of this view which is utterly opposed to the true 
condition of affairs, and depends largely upon a lack of knowledge of the 
nature of the disease as well as upon exaggerated reports of operative results 
in earlier literature. After an experience of forty years with more than 600 
patients suffering from Graves' disease (I now see at least 30 to 40 new 
cases every year), I maintain that in the overwhelming majority of cases 
operative treatment may be dispensed with, that without it and by a well 
chosen and individualized mode of treatment we may secure at least as favor- 
able results, and that its employment should be accordingly limited to the 
few rare cases in which there are extremely severe and threatening local 
symptoms. If we review the surgical cases of the last decade, we find but 
a few isolated ones which will withstand criticism, in which actual cure or 
even marked improvement continued after years of observation, and such 
amelioration frequently related only to the subjective condition, while the 
predominant objective symptoms, such as tachycardia, exophthalmos, Graefe's 
sign, etc., were influenced slightly or not at all. We also meet cases which 
showed no improvement ; with those in which the preferred partial thyreoidec- 
tomy had to be repeated on account of a subsequent growth, and was then 
usually without result, so that the question has arisen whether in such cases 
it is not better to extirpate the entire gland and to compensate for this defect 
by the lifelong administration of thyreoid gland; finally, we meet with cases 
by no means rare in which a fatal result immediately followed the operation, 
the cause often being stated to be " obscure.'' Of the three methods of opera- 
tion, thyreoidectomy and ligation of the arteries of the thyreoid gland still 
show an immediate mortality of 15 to 20 per cent., while Poncefs " ex- 
othyreopexy " (exposing and taking out a lobe of the gland which, drawn 
through the cutaneous wound, is expected to atrophy) has been abandoned 
as being too dangerous. In the practice of eminent surgeons I have observed 
a few such sudden deaths after partial thyreoidectomy in youthful persons 
in whom the affection was by no means severe or hopeless, and I must admit 
that these cases made a very deep impression upon my mind, as the first 
law of our professional art is " ne noceamus." Of course I am far from 

i Allgem. mcd. Centralzeitung, 1903, Nr. 8. — In a case reported by Adam, success- 
fully treated, the cardinal symptom, tachycardia, was absent. 



974 PRESENT STATUS OF GRAVES' DISEASE 

inclined to deduce general conclusions from such unfortunate and isolated 
occurrences. It must, however, be remembered that the patient does not 
succumb to Graves' disease unless there are complications; that even cases 
with apparently most severe cachectic symptoms often improve under hydro- 
therapy, climatic treatment, and the other previously mentioned methods, 
and are kept for years in an endurable condition; and that operations dan- 
gerous to life should be undertaken only when the patients have sufficient 
power of resistance and when the severe local phenomena produced by the 
goiter render the operation imperative. I therefore still consider justified 
the opinion which I expressed a few years ago concerning this question: In- 
stead of seeking in Basedow's disease a present or future field for surgery, 
or even of regarding it as a debatable borderland, we should rest assured 
that for the present we are upon the certain ground of internal medicine, and 
only under most absolute necessity should we swerve to the surgical side. 

An important question and one often difficult to decide is sometimes 
presented to the physician in discussing the relations of Graves' disease (espe- 
cially in women) to marriage, and its physical consequences — therefore, the 
question whether women with Basedow's disease should or may marry. Be- 
fore answering this question we should consider that in Basedow's disease 
we are dealing with a severe, often hereditary, degenerative predisposition 
(neuropsychic constitutional anomaly) as a rule affecting young women, not 
only attended with great debility but simultaneously with serious nervo- 
psychical disturbances which to a limited extent only are susceptible of cure, 
and are therefore often permanent. We should especially bear in mind that 
pregnancy, the puerperal period, and lactation have in many cases a decided 
and generally unfavorable influence upon the course of Basedow's disease, 
either because the outbreak of the disease is directly due to these factors or 
because an already existing affection is decidedly aggravated — especially by 
pregnancy. It is true a few cases have been reported in which, immediately 
after a natural, normal, or artificially terminated pregnancy, the remission 
and even the disappearance of the symptoms was observed. How this influ- 
ence of pregnancy and the puerperal period becomes operative, whether by 
transmission from the thyreoid gland to the sexual organs, these being ap- 
parently in intimate relation, or by direct autotoxic or reflex action (analogous 
to the chorea and the tetany of pregnancy) is not certainly known. That in 
numerous cases there is such an influence is empirically certain, as well as 
the not infrequent direct transmission of Graves' disease to the offspring, the 
prevailing affection to the females, generally from the mother to the female 
and exceptionally to male descendants, also the already alluded to combination 
of Graves' disease with other neuroses and neuropsychoses, as well as with 
severe vasomotor trophic disturbances and autointoxications (myxedema, 
scleroderma, Addison's disease, diabetes mellitus and diabetes insipidus). It 
has been determined by many individual observations that in the families of 
patients with Basedow's disease not only functional mental diseases and 
neuroses but also diseases of the heart and constitutional affections, tuber- 
culosis, cancer, diabetes, etc., prevail, and the repeated occurrence of Base- 
dow's disease in one and the same family (Buschan) has been noted. I 
have under observation a family in which the mother and two daughters 
suffer from Graves' disease; these two daughters, as well as two other daugh- 



TREATMENT 975 

ters, are chloroanemic to a high degree, and show the signs of a conspicuous, 
hereditary, and increased neuropsychic predisposition; in one this amounts 
to a functional psychosis (melancholia). 

If all these experiences are considered, and also the circumstance that the 
individual symptoms of Graves' disease, the goiter, and especially the exoph- 
thalmos when highly developed, awaken a certain physical repugnance, and 
thereby militate against any idea of marriage — we can scarcely escape the 
conviction that if a woman has suffered for some time from pronounced Base- 
dow's disease it is a decided contraindication to marriage, and from a pro- 
fessional standpoint at least this must be accepted as a rule. Naturally, 
when the physician's advice is asked, he should consider the special circum- 
stances of each individual case; on the one hand the degree of severity and 
the duration of the disease, and, on the other, the demonstrable etiologic 
factors, the influence of heredity and predisposition, of accidental and occa- 
sional damage, which may be decisive. When cases arise after marriage, or 
independently of it, or have been aggravated by marriage, by comparing the 
indications of a special case with the general laws herein laid down, we often 
find the treatment a grateful task which is crowned with success. 



63 



SEXUAL NEURASTHENIA 
By A. EULENBURG. Berlin 



DEFINITION AND HISTORY 

Nothing would appear to be more simple than to define and describe the 
pathological entity of " neurasthenia" or, at least, to give a comprehensive 
portrayal, free from uncertainty, of the salient features of its clinical aspect. 
We are constantly using this term ; it is the common property of the physician 
and of the layman who comes to us with the spontaneous announcement that 
he is a " neurasthenic " — not even rarely with the additional statement that 
he is a " sexual neurasthenic/' that he suffers from " sexual neurasthenia." 
Yet, although it is hardly conceivable, and notwithstanding all researches and 
studies in this field, even to-day no neuropathologist would willingly accept 
the task of " defining " neurasthenia as an ontologico-pathologic conception 
in such a way as to be unobjectionable to himself, and both clear and com- 
prehensive. The expression of the New York specialist for nervous diseases, 
George Beard (who died in 1883), is neither more nor less than the Hellen- 
ization of a term used in professional and belletristic literature in Germany 
for 150 years; moreover, the meaning of the word is debatable, or at least 
obscure, as it embraces and brings into prominence in a much too partial 
manner " asthenia? loss of strength, and weakness as the empiric and funda- 
mental symptoms of the " neurasthenic " clinical picture — and this appears 
even more clearly in the synonym employed by Beard, " nervous exhaustion." 
Contrasted with this, but in just as one-sided a manner, the symptom of 
increased irritability was formerly given undue prominence by the use of 
the terms " nervous erethism," " nervosism." To say nothing of other numer- 
ous unsuitable designations, it is much wiser to use the earlier, common, and 
more applicable expression, " irritable nervous debility" which embraces and 
correctly designates both of the component factors which make up the picture 
of neurasthenia, excessive irritability and excessive debility or exhaustion 
(weakness). Under any circumstances, if we desire such comprehensive 
knowledge of individual symptoms as will permit a clinical recognition of 
neurasthenia, we must base our study on these two inseparably connected and, 
to a certain extent, correlated conceptions. Moreover it is evident that in the 
neurasthenic we are dealing not merely with " nerve disturbances " in the 
restricted and ordinary sense of the term, but just as much or even more 
with disturbances of the psychical elementary processes and their effect upon 
the entire psychical life — not merely with " neuropathic " but also with 
" psychopathic " conditions, of all grades of severity and of varying impor- 
tance. In consonance with these preliminaries I should at present like to 
modify our conception of neurasthenia and portray it as a widely distributed, 
976 



DEFINITION AND HISTORY 977 

extremely chronic neuro-psyclwsis which is chiefly characterized clinically 
by pathologic disturbances in the equilibrium of innervation and by changing 
relations between the sensory and motor as well as between the higher cerebral 
(psychic) neuron systems which bring about association, these pathological 
disturbances being nosologically revealed by extreme irritability of the sensory 
and psycho-sensory neuron systems and excessive exhaustion of the motor and 
psycho-motor (intracentral) neuron systems. Like all other attempts at 
definition this naturally offers no actual explanation; still less does it suggest 
a comprehensive and exhaustive " theory " as to the nature of neurasthenia 
■ — it constitutes merely a brief description or is a compilation of its charac- 
teristic and chief symptoms. In studying the condition more closely it 
appears that the wave of irritation from sensory impressions in neurasthenics 
is in a certain sense deepened, and experience teaches that this is true not 
only for the wave of perceptions of the special senses but also for the sub- 
jective feeling chiefly expressed by uneasiness, for the " wave of pain," or, 
more generally speaking, the " wave of uneasy impressions." In fact it is 
characteristic of the neurasthenic that this sensation of uneasiness, the " neg- 
ative impression of sensation " (Ziehen) appears after relatively slight sensory 
irritations and is marked by disproportionately slight resistance; according 
to experience this is usually evoked by irritation from other organs, irrita- 
tions arising from the sensations of their own bodies, which, entering into 
the consciousness as intensified and long-continued sensations of uneasiness, 
lead to manifold sensations of fear and compulsion, to deeply impressed 
conceptions of fear and compulsion, to the numerous and peculiar "phobias" 
of neurasthenics. If the excessive exhaustion of the motor neuron is added 
to the extreme irritability of the sensory neuron, these constitute another 
origin for the development of the pathologic sensation of aversion. For even 
mere physiologic fatigue, and still more so the pathological condition of 
" over-fatigue " or " exhaustion," is accompanied by negative sensory im- 
pressions, by conspicuous sensations of discomfort, the " sensation of ex- 
haustion " of Benedikt — and the sensory reaction of the exhausted cells and 
neuron groups to stimulation, according to the law of exhausted or destroyed 
nerves, is far more extensive and intense, i. e., painful, than the stimulation 
of normal, non-exhausted cells and neuron groups. Such a deviation from 
normal reaction permits us to conclude what causes are necessary to produce 
a pathologic change in the neuron and in its functional and chief constitu- 
ents, the nerve-cells — a somewhat specific "neurasthenic" change of the 
nerre-cells — which is of course by no means so clear as the neuralgic and 
spasmogenic changes (hysterogenic, epileptogenic) gradually explained by a 
technical investigation which has advanced further and further, and has al- 
ready given us such great results in the development and employment of 
staining methods, so that these changes can no longer be regarded as obscure. 
Next the "functional neiwoses" and neuro-psychoses which we at this time 
still regard as neurasthenia, or at least so designate them, will be differen- 
tiated from those organogenetic and histogenetic diseases of the nervous sys- 
tem which present demonstrable material changes in structure, but which are 
not specially related, and which evidently differ qualitatively as well as quan- 
titatively. Until this goal is reached we must be content to explain the 
nature of the presupposed " neurasthenic change " by means of the same un- 



978 SEXUAL NEURASTHENIA 

satisfactory theories which are applied to the elementary processes involved 
in the mechanism of physiologic nerve activity, at least in a hypothetical 
form. Obviously, according to our ordinary explanation of functional devia- 
tions from the normal, certain specific alterations of chemism, of composition, 
of metabolism of the nerve cells, the nature of which is still unknown, are 
decisive. These, reduced to the simplest formula, may be designated as " a 
disproportion between consumption and restitution/' as a disturbance of 
equilibrium between degeneration and regeneration, between mal-assimilation 
and assimilation, in the protoplasmatic, intracellular metabolism of the nerve- 
cells. A habitually deficient, or congenitally defective nutrition, an accumu- 
lation of functionally damaged products of decomposition in consequence of 
their insufficient evacuation, we may regard (according to the "hypothesis 
of exhaustion " proposed by Weigert and Edinger) as the basis of the path- 
ologically changed cell activity, and combined with this is the possibility of 
other general damage by " autointoxication." It is at least a probable, and 
perhaps subjectively a more satisfactory, explanation of the neurasthenic 
changes to hypothecate another origin; namely, from permanent variations 
in equilibrium or an alteration in the force generated {energy production) 
in the nerve-cells. This involves in particular the " energetic 9 \ theory of 
changes in the internal relations of labor which was formulated and published 
by 0. Eosenbach, the energy formation and tension within the smallest labor 
elements, the " energists " of the nerve cells. This abnormal irritability and 
exhaustion which, as we may speedily convince ourselves, form the typical 
symptoms in neurasthenia can then be referred to a too slight " essential " 
and "non-essential" labor activity of the nervous inhibitive apparatus which 
is intended to regulate the protoplasmic processes and other forms of reac- 
tion; therefore, with relatively slight irritation, a disproportionately severe 
metabolism of tension material, of thermic and oxygenic energy, takes place. 
This may be temporarily compensated for by increased action on the part 
of the vascular system, but, if continued, will inevitably produce the symp- 
toms of " fatigue " and " prostration." For a better understanding of this 
theory it must be remarked that the form of activity which, in Eosenbach's 
sense, is to be regarded as " essential " is that depending upon the production 
of tension from energy, and the " non-essential " is that depending upon 
great motor activity, that directed to the displacement of masses, whether 
within or without the organism. The conception of " fatigue " would cor- 
respond to a consumption of movement energy ("kinetic energy") in so 
far as the equilibrium of tension is not yet essentially altered ; " exhaustion " 
represents a consumption of movement energy with marked diminution of 
the supply of tension energy which, however, after sufficient rest may again 
be formed, in which condition a certain amount of energy already formed and 
held in reserve is drawn upon ; finally we have the condition of " asthenia " 
(insufficiency) in which it is impossible to produce fresh movement energy, 
or in which that already existent is insufficient for the essential cell labor 
required for the displacement of masses. We see that these hypotheses of 
exhaustion essentially agree, and, on the basis of a theory developed upon a 
conception of energy, simultaneously result in a finer development and richer 
confirmation of the hypotheses of exhaustion. Those for whom distinctions 
of this kind are too subtle may concur with Eudolf Arndt in regarding 



DEFINITION AND HISTORY 979 

neurasthenia as an expression of nerve activity based on the laws of dying 
or exhausted cells (the so-called Bitter- Valli theory), or he may assume with 
Fere a " decrease of the activity and vibration of the nerve molecule/' or, 
according to the latest theories and tenets, as pathologic " changes of the 
tracts" and "inhibitions" of the neurons and neuron groups, the changes 
in the " tracts " being supposed to be chiefly in the sensor}-, the " inhibitions " 
principally in the motor neuron groups; or we may assume that a consider- 
able number of them are increased. These theories add very little to our 
knowledge; but, like other biologic and pathologic hypotheses, they sub- 
jectively bring to us a sensation of relief in that we feel our ignorance less 
keenly, and, with a few smooth changes, or with symbolic transcriptions and 
theories, we dismiss these conditions. 

No matter what the mysterious nature of the " neurasthenic change " may 
be we have at all events not the slightest difficulty in understanding that in 
the individual case these changes need not be evenly distributed throughout 
all neurons and neuron systems, but that either certain neuron systems are 
especially implicated or that individual neuron systems (either because of a 
lessened predisposition or a more rapid functional employment, or from these 
causes combined) are soonest and most markedly subjected to the "neuras- 
thenic change." From a clinical standpoint it therefore appears reasonable 
to select from an enormous mass the most prominent phenomena which dis- 
tinguish the clinical picture or give it typical color, and to classify or group 
these; this, indeed, has been done for some time. Therefore we speak of a 
cerebral and spinal form of neurasthenia (encephalasthenia and mijcl asthenia) 
or, to specialize still further, of a cardiac, a vasomotor, a gastrointestinal (dys- 
peptic) and, finally, of a genital (sexual) form of neurasthenia. The last 
named, " sexual neurasthenia," which we shall exclusively discuss in the fol- 
lowing article, has received a certain historic justification and sanction from 
the fact that Beard devoted his last years to a special study of the subject, 
and left an unfinished book which was widely read and occasioned much 
comment. The differentiation of this variety has, however, sufficient vindi- 
cation from its uncommon frequency, from its symptomatic and etiologic 
factors, and, above all, from the extraordinary importance of its prophylaxis 
and therapy. 

What we are to understand by " sexual neurasthenia," and how we may 
differentiate it clinically from other localized forms of neurasthenia, is clear 
from the preceding. Here we have a collective designation originating in, 
and corresponding essentially to, practical requirements, and the extensive 
prevalence and severity of this affection demand for it our attention; it is 
a neurasthenia with local genital symptoms (either exclusively predominating 
or only periodically), i. e., with symptoms of "irritable weakness" and of 
increased irritation and exhaustion in the course of the genital nerve appar- 
atus. On further study of this disease we note what is confirmed by universal 
experience, that the pathologic disturbances of the equilibrium of innervation 
are nosologically revealed by excessive irritability of the sensory and psycho- 
sensory systems, by excessive exhaustion of the motor and psycho-motor sys- 
tems belonging thereto, by predominant sensations of disinclination, and, in 
consequence of these, by general reactions upon the entire sensory system and 
the imagination, 



980 SEXUAL NEURASTHENIA 

We shall, therefore, first discuss the general and special symptomatology, 
then its etiological relations, and, finally, the prophylaxis and treatment of 
sexual neurasthenia in so far as this is practicable within the scope of a 
single chapter and from an individual point of view. 

GENERAL SYMPTOMATOLOGY 

The fundamental symptoms of neurasthenia are found in sexual neuras- 
thenia, intimately related to the processes of sexual life; and the correspond- 
ing local color is given in the form of psychical ("psycho-sexual") disturb- 
ances as well as, in a more restricted sense, by nervous (somatic nervous) 
disturbances and changes. The latter are usually noted in the area of the 
genital nervous apparatus with its widely distributed centers and tracts of 
projection, its cerebrospinal, intraspinal, spino-sympathetic, spino-peripheral 
and sympathetico-peripheral neurons. 

Among the chief psychical (psycho-sexual) symptoms we find primarily 
anomalies of sexual sensation which, following the general law of neurasthenic 
disturbances in sensation, are manifested by sensations of aversion, of fatigue, 
and of pain. The " wave of irritation " in this sphere of sexual 1 sensations 
is also depressed, and thus, upon relatively slight irritation, disproportionately 
distributed and long-continued reaction appears, the " negative sensory phe- 
nomena " which dominate the sensations of pain and aversion — therefore 
"psycho-sexual hyperesthesias and paresthesias." Among the symptoms pro- 
duced is a repugnance to natural sexual intercourse, its rare occurrence or 
complete discontinuance, aversion which may increase even to psychical pain, 
and terminate in sensations of fear and the " phobias " peculiar to neuras- 
thenics. If these derangements involve more deeply the play of the imagina- 
tion and become firmly rooted, normal sexual conceptions are weakened, are 
subject to greater inhibitions, with their disappearance sexual coldness and 
dislike develop, and ordinary sexual impulses may be completely lacking; 
on the other hand, many resort to improper associations by means of which 
new or previously excluded fantastic ideas develop in the sexual realm of the 
imagination, and an uncontrolled fancy allows these to fructify into path- 
ologically perverse images and impulses. Here may be found the strongest 
roots and the cause of onanism, which is practised by the neurasthenic not 
only prior to normal sexual intercourse but afterward, even in the married 
state, and at all ages, and is preferred to ordinary sexual intercourse because 
usually not immediately conducive to sensations of aversion and fear, and 
because it presents to the fancy ever changing and desirable imaginary con- 
ditions. 

Onanism is, therefore, not only (as is ordinarily assumed) the most com- 
mon cause of sexual neurasthenia but, perhaps just as often, the symptom 
and sequel of sexual neurasthenia developing from pathologic sensations of 
repugnance experienced in normal sexual intercourse; at all events this cir- 
cumstance favors the return of many neurasthenics and non-neurasthenics 
to these " premiers amours/' To the same extent, and for the same reason, 
there is a tendency to various anomalous forms of sexual intercourse (in mar- 
riage and outside the bond) because these — not yet associated with distressing 
fears — appeal to the imagination as desirable, and lead to the most curious 



GENERAL SYMPTOMATOLOGY 981 

and most severe psycho-sexual abnormalities and perversions. Here are con- 
cealed the deeply embedded roots from which springs the sexual neurasthenia 
of individuals already predisposed to pathological, psycho-sexual, and dan- 
gerous habits, to sadism, flagellation, masochism, fetichism, exhibitionism, 
pederastia, and other perversions of the sexual instinct; finally, to sexual 
inversion in the form of acquired homosexual desires of " uranism " and 
tribadism. 

Those who reject normal sexual intercourse as antipathic, or who find this 
act produces distressing sensations of aversion and fear, and those whose 
psychical and mental predisposition so inclines them, constantly search for 
new and artificial means of satisfaction; they may find these, at least tem- 
porarily, in pederastia and irruniation, in active and passive flagellation, in 
coprophagy and " ideal " coitus, in all modes that a creative fancy can sug- 
gest — and these are numerous. 

It must, however, suffice if we merely indicate the paths leading to the 
mysterious and magic realm of " sexual psychopathy," which from this dis- 
tance is viewed with wholesome awe. We deny ourselves a visit to this 
region, and return at once to the more certain ground of the somatic-nervous, 
pathologic disturbances. 

Here we first consider the various sensory disturbances in the region of 
the genital nervous apparatus which are due to excessive irritability and 
appear as sensations of aversion and pain, hyperesthesia, paresthesia, and 
dysesthesia of the genital projection tracts. On account of the intimate rela- 
tionship of the nerve tracts in the uropoietic and genital apparatus, especially 
in the male, sensations in the lower parts of the urinary and genital passages 
(including their associate organs) cannot be distinguished; hence we find in 
addition to the hyperesthesias and dysesthesias immediately associated with 
sexual intercourse painful sensations also in the urethra and bladder (urethral- 
gia and cystalgia), spontaneous neuralgic pains and pain on micturition 
(dysuria), as well as pathologic reflexes originating from the sensory nerves 
of the bladder and urethra in the form of cystospasms and urethrospasms 
which are usually painful, often a sense of pressure during the discharge of 
urine (strangury, tenesmus vesica?) and spastic ischuria due to spasm of the 
smooth internal sphincter of the bladder and of the transverse striped com- 
pressor urethrse. These conditions may be combined or may alternate with 
one another. The path of these reflexes is through the center of the bladder 
innervation which is supposed to be located in an indefinite area between the 
center for erection, the center for ejaculation, and the center for the rectum, 
and closely adjacent to these in the lowest portion (sacral) of the spinal 
cord and above the terminal part designated the conus medullaris. This 
opinion has lately been combated by L. R. Muller 1 for weighty clinical 
and experimental reasons. The voidance of urine is probably brought about 
by the sympathetic nervous system, by the hypogastric nerves, and the nervus 
erigens. The spinal tracts which conduct it communicate to the centripetal 
spinal fibers of the brain by a reflex action the bladder's condition of fulness. 
While the detrusor is innervated by the nervus erigens which originates from 

1 L. R. Muller (Erlangen), " Klinische und experimentelle Studien iiber die Inner- 
vation der Blase, des Mastdarmes und des Genitalapparates." Deutsche Zeitschr. f. 
Wervenhcilkunde, Bd. XXI, 1901, 



982 SEXUAL NEURASTHENIA 

the three first sacral roots (Rehfisch 1 ) of the hypogastric plexus, the tonus 
of the internal sphincter is maintained and the closure of the bladder is 
effected by the inferior mesenteric ganglion and the hypogastric nerves arising 
therefrom. According to Miiller, the reflexes which are here produced do not 
occur in the spinal cord but below the conus medullaris in the sympathetic 
ganglion nodule of the pelvis, especially in the inferior mesenteric ganglion 
and in the ganglia of the hypogastric plexus where the spinal nerves terminate 
as pre-cellular fibers, and from which the post-cellular nervi of the hypo- 
gastric nerves and of the nervous erigentes pass to the bladder. We observe 
that the process of erection also originates outside the spinal cord, namely, 
in the sympathetic ganglion nodules on the floor of the pelvis which, by 
means of the nervi erigentes, supply the vessels of the corpora cavernosa. 
The impulse for the first act of ejaculation, the opening of the seminal 
vesicles, is also conveyed chiefly through this ganglion, while the second act, 
the discharge of the semen, is produced by the reflex contraction of the trans- 
verse striped ischio-cavernosus and the bulbo-cavernosus muscles in which 
act the spinal cord is also implicated. The ganglion cells for these muscles 
are apparently situated in the lowermost parts of the spinal cord (conus 
medullaris). 

Of course, for our fundamental consideration nothing of importance is 
changed by this transposition of the most important urogenital reflexes from 
the sacral cord to the pelvic ganglia of the sympathetic. But it compels us 
to reject the contradictory and untenable theory of a so-called " lumbar cord 
neurosis " which has for some time been criticised, not only from a clinical 
but also from an anatomico-physiologic standpoint. It would be well if this 
misleading expression were eliminated from our vocabulary ! 

If we adhere to this theory, particularly in studying the motor secretory 
disturbances in the course of the genital nervous apparatus in sexual neuras- 
thenia, we must consider its various divisions — which, however, are also char- 
acterized as " stages." 

The most important of these disturbances are pathologic pollutions and 
erections, and the various forms of spermatorrhea and prostatorrhea ; above 
all, genital debility, the neurasthenic forms of impotence (which in numerous 
cases constitute the chief symptom or, often for a long time, the only one). 
These manifestations, because of their great practical importance — especially 
pathologic pollutions and impotence — call for a somewhat comprehensive and 
special symptomatologic description, as they frequently necessitate special 
treatment and local therapeutic measures. 

SPECIAL SYMPTOMATOLOGY 

(a) PATHOLOGIC POLLUTIONS 

The expression " pollution " means nothing more than a discharge of 
semen apart from the act of cohabitation but, as a rule, accompanied by 
sexual irritation (in the female there is a corresponding discharge from 
Cowper's and Bartholin's glands). The question arises whether and under 

i E. Rehfisch, " Ueber die Innervation Mer Harnblase," Yirchow's Archiv, Bd. 161, 
1900. 



SPECIAL SYMPTOMATOLOGY 983 

what circumstances — particularly in men — these pollutions are to be regarded 
as pathologic, and whether there are normal pollutions which may be regarded 
as physiologic. The great majority of authors undoubtedly regard the noctur- 
nal emissions which occur in otherwise normal and mature males, apparently 
without special cause and at long intervals, as physiologic and normal. Hence 
males in whom, after the age of puberty, such emissions do not from time to 
time occur — and to my knowledge there are not a few of these — would 
resemble mature females who do not menstruate, and who are therefore ab- 
normal. I do not concur in this opinion, but, on the contrary, believe that 
an emission is in a certain sense abnormal and due to anomalous or increased 
irritation, although it is unnecessary to at once regard it as pathologic; for 
instance, it may be like cough or vomiting, which are not " normal," yet, on 
the other hand, are not necessarily symptoms of disease. As is well known 
these depend upon individual conditions, upon the mode of life and the 
nutrition ; for example, upon the use of alcohol at evening, upon the position 
of the genitalia (dorsal decubitus), and upon other accidental and occasional 
circumstances. However, apart from this question and in answer to those 
who in a certain sense regard emissions as normal, I will state that there 
must be a more or less definite limit to this normality, which depends upon 
where we draw the line and what we regard as the criteria of the normal. 
The opinion is almost unanimous that no law for this exists, and that it 
must therefore be based upon a chain of circumstances. The age of the 
individual, the frequency and the time of the emissions, their composition, 
particularly when combined with erection and orgasm, and the local and 
general symptoms which follow the act, are important in the differentiation 
of each individual case. 

The occurrence of emissions before the development of puberty and before 
puberty is established must be regarded as pathologic, especially when these 
pollutions do not occur as isolated instances but are so frequent as to be 
almost regular nocturnal occurrences; this is often the case in boys predis- 
posed to neurasthenia, whose imagination is early excited, by erotic pictures, 
and who are early addicted to masturbation. I was recently consulted on 
account of a boy, aged 15, who suffered almost nightly, at least four or five 
times a week, from repeated seminal emissions, often accompanied by erotic 
dreams ; he was anemic, poorly nourished, and presented the usual symptoms of 
so-called " school nervousness," especially the characteristic headaches (which 
continued after excision of the tonsil), digestive disturbances, etc., and who had 
also long suffered from enuresis nocturna. Cases of this kind, by no means 
rare, might tempt us to propose for the clinical picture, characteristic to a 
certain extent, the term infantile, or better, pre-juvenile sexual neurasthenia. 
Aside from the early age at which these pollutions occur, their frequency 
must be regarded as pathologic, as is also the case in adults if these emissions 
are repeated several times a week, or for a few days in succession, or even 
several times during the same night; the pathologic nature is all the more 
obvious if these patients practise sexual intercourse sufficiently and regularly 
in or out of wedlock. Seminal emissions which occur during the day and 
not at night, with or without obvious cause, or upon slight mechanical or 
psychical irritation, are undoubtedly of morbid character. These " pollu- 
tiones diurnm " are of much more serious import than the nocturnal, for 
64 



984 SEXUAL NEURASTHENIA 

instance, pollutions which occur without erection or orgasm, without a feel- 
ing of lust, in which therefore the emission or the loss of semen takes place 
from a flaccid, non-erect organ. This condition is often associated with 
hyperesthesia and pain in the urethra and bladder, with the previously men- 
tioned reflex spasms of the detrusor and sphincters, the symptoms of 
strangury, of tenesmus (the so-called "irritable bladder") ; or it is combined 
with diminishing sexual power or complete impotence, and is then to be re- 
garded as a sign of exhaustion and by no means as an irritative phenomenon, 
especially if, at the same time, the production of semen is reduced, and only 
a few drops are exuded from the urethra, not actually ejected. The insuffi- 
cient quantity of semen discharged from the seminal vesicles into the posterior 
urethra does not distend the pars prostatica sufficiently to produce an orgasm, 
or the reflex contraction of the ischio-bulbocavernosi muscles by the ejacu- 
latory center in the lowest portions of the spinal cord. In this process, there- 
fore, the center has no function; the semen exudes drop by drop from the 
urethra, but is not ejected, resembling the condition in disease of the conus 
medullaris which functionally deranges or permanently destroys this center. 
Finally we must consider the result of these emissions upon the organism 
and upon the general constitutional condition of the patient. If this retro- 
action is extremely severe and long continued, manifesting itself in general 
shock or lassitude, in debility which is almost complete exhaustion, or in any 
marked and extremely unpleasant local sensations invariably persisting for 
a few days, their pathological dignity should not be underestimated, provided 
that by abnormal frequency, by qualitative deviations, etc., they do not result 
in the previously mentioned exceptional condition. There has been much 
discussion as to the nature of this deleterious retroaction of emission upon 
the general health, and two opinions which are diametrically opposed to each 
other have been brought into prominence. According to the earlier view, 
which has recently again been advanced by Donner, the seminal fluid is sup- 
posed to contain a valuable substance which can be replaced only with the 
greatest difficulty; therefore the loss of more or less of this fluid entails 
serious consequences to the organism. More recent theories attach but slight 
importance to the seminal losses. The destructive factor manifests itself by 
functional hyperirritation which primarily and immediately acts upon the 
affected lumbar cord, also upon near and distant neuron systems, this irrita- 
tion being conveyed to the entire nervous system, transitorily producing a 
kind of nervous shock. It is self-evident that the first view, according to 
which the seminal loss is looked upon as the most serious factor, can scarcely 
be maintained; for, if this conception were true, the amount of the losses 
would be of great significance, while, on the contrary, the resultant excessive 
exhaustion not rarely experienced after very slight loss of seminal fluid and 
also the " shock theory " necessitate a certain limitation or extension. The 
consequences which have been described are also occasionally observed in cases 
where no orgasm has occurred with its attendant effects upon the spinal 
ejaculatory centers. On the contrary, the seminal fluid which has passed 
into the pars prostatica is exuded without any phenomena of a centripetally 
conveyed stimulation. Apparently the implication of the nervous mechanism 
as shown by the appearance or absence of these consequences is of as much 
importance as the preceding general condition of the nervous system, the 



SPECIAL SYMPTOMATOLOGY 985 

degree of neurasthenic change already existent which, in the individual case, 
is the most decisive and important feature of the pathologic process. 

A condition in women analogous to that of pathologic emissions in men 
is observed in the frequent occurrence of pollutions, the so-called vulvo- 
vaginal crises and clitoral crises. The secretion is mainly from Bartholin's 
or Duverney's glands situated at the mouth of the vagina, but it is usually 
mixed with secretion from the vaginal glands, often also with mucus from 
the cervix. A touch upon the mouth of Bartholin's glands at the entrance 
of the vagina, the " erogenous zone," as well as upon the clitoris and other 
erogenous parts of the female, often produces a copious secretion from the 
previously mentioned glands, such as chiefly takes place in masturbators, 
but which also occurs in not a few women during the ordinary act of co- 
habitation although this is of varying intensity. In the female, the discharge 
of a more or less abundant, pale, tough, mucoid secretion after lascivious 
dreams, or even spontaneously, is an unerring sign of sexual neurasthenia, 
and under similar circumstances is analogous to the pathologic pollutions of 
male neurasthenics ; we hear less of these, and they are not sufficiently under- 
stood even by physicians; therefore, when they occur in physical virginity 
from otherwise normal genital organs they are not correctly diagnosticated. 



(&) SPERMATORRHEA AND PROSTATORRHEA 

According to Fiirbringer, " spermatorrhea " is a very suitable designation 
for the emissions independent of seminal discharges, those which occur inde- 
pendently of erection and orgasm as well as without ejaculation, particularly 
in conjunction with the evacuation of feces and urine — so-called defecation 
and micturition spermatorrhea. We are still in doubt as to the origin and 
importance of these processes. Is this an increased production and spasm of 
the seminal vesicles supplied with smooth muscular fibers, therefore an " ir- 
ritative symptom " ? — or, on the contrary, is it a symptom of " weakness" 
due to insufficiency or paresis of the ductus ejaculatorius? — or, as must be 
assumed in many cases, do mechanical factors play a role, especially does 
abdominal pressure either predispose to or directly favor it? In sexual 
neurasthenia, " micturition spermatorrhea," the discharge of semen with and 
after urination or the admixture of semen with the latter (" spermaturia ") 
is comparatively frequent, particularly in those cases in which a local gonor- 
rhea is the cause of the condition, and in which spermatozoa are occasionally 
found embedded in the " Tripperfaden " (see Etiology). Excluding simul- 
taneous micturition the conditions are different in true " defecation sper- 
matorrhea," which in itself is met with much more rarely; here there appears 
to be either a purely mechanical expression of the seminal vesicles — or, as has 
been assumed, by a central radiation process the stimulation produced reflexly 
is said to pass from the center of the rectum in the lowest portion of the 
spinal cord to the neighboring center for seminal discharge. This view in 
its present form can scarcely be adhered to if the results recently reported 
by L. Miiller are proven true, according to which the center for defecation 
as well as that for evacuation of the bladder is not situated in the lowest 
portion of the spinal cord, but, on the contrary, in the sympathetic ganglion 
of the pelvis and the rectum so far as the internal sphincter ani is innervated 



986 SEXUAL NEURASTHENIA 

by fibers of the sympathetic nervous system which originate partly in the 
hemorrhoidal plexus and partly in the inferior mesenteric plexus. The cen- 
tripetal communicating branches of these plexuses, for the rectum as well 
as for the bladder, have merely the function of transmitting the rectum's con- 
dition of fulness and the necessity of evacuation. Medullary spinal fibers 
(through the inferior hemorrhoidal nerve) pass only to the external sphincter 
ani and the skin in the region of the anus, and conduct the contraction of the 
sphincter, designated as the " anal reflex" the center of which is localized 
in the conus medullaris. As the anal reflex in neurasthenics is usually in- 
creased ( Eossolimo 1 ) and combined with generally increased cutaneous 
reflexes — it seems plausible that, not from the reflex of discharge but from 
the anal reflex at the termination of defecation, the seminal glands may dis- 
charge into the urethra ; on the contrary, as we know, the discharge of semen 
as such appears to be independent of the spinal cord, and the ejaculation of 
the semen only is controlled by a center situated in the conus medullaris. 
It is probable, therefore, that the reflex processes combined in micturition 
and defecation spermatorrhea should be regarded as occurring below the 
spinal cord in the sympathetic ganglia of the pelvis. 

The discharge designated as " prostatorrhea'' or prostate secretion (usu- 
ally pathologically changed) without admixture of semen, is decidedly rare 
in sexual neurasthenia, and probably occurs only in cases due to gonorrheal 
infection, and accompanied by gonorrheal local disease. In these cases there 
is either a swelling of the gland from parenchymatous prostatitis or a gonor- 
rheal posterior urethritis, with increased irritability of the prostate, in which 
the glandular secretion either mechanically (by abdominal pressure) or by a 
spasmodic contraction of the smooth musculature of the prostate, is discharged 
into the urethra. The normal prostatic secretion is of thin, fluid, milky, 
turbid consistence, while in inflammatory prostatic disease it is thicker, and 
of muco-purulent character; it has the peculiar, so-called seminal odor; on 
treatment with a one per cent, ammonium phosphate solution it shows the 
well known spermin crystals; besides amyloid granules it usually contains 
cylindrical cells of the prostate which indicate fatty degeneration, also round 
cells and blood-corpuscles in varying quantity; it is frequently observed in 
the urine in the form of threads, resembling gonorrheal threads, which are 
especially profuse toward the end of micturition. Aside from its semeiotic 
importance in differential and local diagnosis, this secretion is not so inter- 
esting as other findings in the urine and the urethral secretions which indicate 
a continuous and local effect from gonorrhea in the course of sexual neuras- 
thenia. 

(c) PATHOLOGIC ERECTIONS. "PRIAPISM" 

The "center for erection," as a rule, has been located in the lowest por- 
tion of the cord near the " ejaculatory center " — a view in opposition to 
which is the circumstance that in transverse disease of the cervical and 
thoracic cords, which are clinically associated with irritative symptoms, path- 
ologically increased erection (priapism) is frequently observed; but this con- 
dition is rare in isolated focal disease of the lumbar and sacral cord and of 

i Rossolimo, " Der Analreflex, seine Physiologie und Pathologic" Neurolog. Cen- 
tralblatt, 1891, Nr. 9. 



SPECIAL SYMPTOMATOLOGY 987 

the conus medullaris. After these regions are destroyed erection is still 
possible. 

L. E. Midler has lately published, in an article repeatedly quoted, the 
experimental proof that the center of erection in animals (dogs) cannot be 
located in the spinal cord since, after the removal of the entire sacral cord 
and the greater portion of the lumbar cord, dogs always exhibited intense 
erection when confronted with bitches in heat, while these erections did not 
occur after section of the spinal cord in the lower cervical region. Therefore, 
the tracts which pass from the brain to the center of erection must be situ- 
ated relatively high, and certainly pass out of the cord in the upper lumbar 
region. Besides from stimulation (psychical) centrifugall}- transmitted from 
the cerebral tracts, erection may be produced reflexly from the periphery in 
many other ways; by mechanical irritation (friction) of the penis — as in 
masturbators — and from the stimulation of the secretion which has accumu- 
lated in large amounts in the sexual glands (seminal vesicles, prostate gland). 
This reflex, like the bladder and rectal reflexes and that for the discharge of 
semen, also occurs below the spinal cord in the sympathetic ganglia on the 
floor of the pelvis. The centripetally conducting tracts of the reflex arc in 
question pass from the dorsal nerve of the penis, the terminal branch of the 
common pudendic nerve, to the conus medullaris, thence by communicating 
branches which have not yet been definitely determined to the previously 
mentioned ganglia which, on their part, bring about the centrifugal innerva- 
tion of the corpora cavernosa by the erigens nerve which passes out of the 
hypogastric plexus. 

If in sexual neurasthenia pathologically increased erections occur during 
the day as well as at night, usually combined with feelings of lust, oftener, 
however, with sensations of disgust, even of actual pain, which last for hours 
(priapism), these symptoms of psychogenous origin are in part the expression 
of abnormal and excessive psychical stimulation which, in the form of erotic 
pictures and conceptions, passes from the brain to the spinal tracts of the 
center of erection — or the} r are due to reflex stimulations which pass from the 
periphery along the paths of centripetal conduction which have been mentioned. 

These symptoms are chiefly found in masturbators in whom central psy- 
chical and the periphero-mechanical irritations are combined in various ways. 
Naturally, increased erections are relieved by masturbation. Erections are 
frequent in those men in whom sexual neurasthenia is due to local genital 
diseases, especially of a gonorrheic nature, or to local irritation developed in 
the posterior portions of the urethra whereby, periodically at least, reflex 
stimulation of the center of erection produces greatly prolonged erections 
which form the most common symptom in the initial stages of gonorrheal 
posterior urethritis. Cases of this kind are usually accompanied by other 
irritative s} T mptoms previously described, by cystalgia and urethralgia, by 
reflex cystospasmus (spasm of the detrusor vesicae.) or urethrospasm (spastic 
ischuria from spasm of the compressor of the urethra), more frequently even 
with simultaneous or alternating spasm of the sphincters of the bladder and 
urethra, in the latter case presenting the well known picture of tenesmus 
vesica, strangury. If prolonged, other symptoms of increased reflex irritation 
as well as pathologic erections decrease or disappear, partly because the 
peripheral sources of irritation gradually pass away, partly and chiefly be- 



988 SEXUAL NEURASTHENIA 

cause the irritability of the center of erection decreases more and more, and 
a condition of neurasthenic impotence is introduced and dominates the later 
stages of the affection. 

(d) NEURASTHENIC IMPOTENCE 

" Impotence " — employed in its widest sense, which is to be immediately 
discussed — if not the most frequent, is practically the most important symp- 
tom of sexual neurasthenia; in the eyes of the patient it is the dominant 
symptom, and the one which, chiefly or exclusively, compels one-third to 
one-half of all sexual neurasthenics to seek professional aid; this, too, is the 
realm probably selected for literary publications — not always above suspicion, 
forming the subject of monographs by specialists, particularly from a thera- 
peutic aspect. 

When patients, among whom neurasthenics are at once detected, complain 
of increasing impotence for which they seek relief, our first object must be 
to ascertain from the history whether they are actually suffering from " im- 
potence " or simply from a " decrease in power " in the scientific sense in 
which we employ this expression. As is well known, we differentiate between 
" potentia cceundi " and " potentia generandi " ; the laity understand the 
expression only in the first sense, therefore sterility due to aspermatism they 
do not designate as impotence — and as physicians we also usually understand 
b}' this only the incapacity to carry out coitus in consequence of 'disturbed 
mechanism of the genital functions, as well as of erection and ejaculation. 
But the laity by no means understand the differences and limitations of these 
conditions because of which deficient or absent libido is satisfied in perverse 
channels, which causes a suspension of the exercise of the sexual functions; 
these conditions, fundamentally so far asunder, are often confounded, but 
by careful questioning we soon learn that the supposed impotence consists 
in a decreased or perverted sexual desire, or more or less pathologic aber- 
rations of this desire, without demonstrable disturbance in power. Only well 
educated persons who are trained to think can be taught to appreciate this 
difference between deficient or absent libido and a loss in power. In the 
same sense a person may refrain from eating because of a want of appetite 
or because the state of his digestive organs does not permit him to ingest 
food and digest it. As a rule, not much can be expected from explanations 
of this kind; nevertheless, we must not fail to make them) for many of these 
patients with supposed impotence and a dread of disease of the spinal cord, 
" tabophobia " — particularly those who have masturbated for some time — are 
brought to us, and because psychical influence is a powerful force in com- 
bating the pathologic symptoms belonging thereto. 

Hence, among sexual neurasthenics we find quite a number who are 
chiefly affected by deficient libido. They are more or less potent — have 
ci power " but do not " desire " ; on the contrary there are some who neither 
desire nor have the power (these are relatively the least miserable) ; finally 
there are great numbers of those who have " desire " but very deficient power, 
and this is finally lost. These suffer from impotence in the restricted clinical 
and practical therapeutic sense — the mechanism- of erection and ejaculation 
being quantitatively affected, or qualitatively changed, or the power appearing 
to be entirely lost. But here, as is evident from our diagram of the anatomico- 



SPECIAL SYMPTOMATOLOGY 989 

physiologic structure of these organs, very different modes of origin and forms 
of impotence are conceivable and possible. 

In studying this diagram briefly from our present standpoint, we must 
differentiate three centers which control the extent and function of the male 
genital organs, namely: 1. The center for erection-, 2. The center for the 
discharge of the seminal fluid; 3. The ejaculatory center. Of these three 
centers the first two have their seat in the sympathetic ganglia upon the floor 
of the pelvis (inferior mesenteric ganglion and hypogastric plexus, especially 
in the plexus seminalis, prostaticus and deferentialis) ; the third, however, is 
situated in the lowest portion of the spinal cord (conus medullaris). Erection 
and discharge from the seminal glands are, therefore, sympathetic reflexes, 
while ejaculation is a spinal reflex. The centrifugal nerve for the purely 
vasomotor process of erection is the erigens nerve which supplies the corpora 
cavernosa (this nerve being a virtual continuation of the first three anterior 
sacral nerve roots which pass to the hypogastric plexus). With sufficient 
stimulation from the center of erection the reflex is conducted to the center 
for seminal discharge, the smooth muscle fibers of the seminal ducts, the 
seminal vesicles, and the prostate contract peristaltically, and the discharge 
of semen takes place into the posterior urethra, the pars prostatica distends, 
this being normally accompanied by an orgasm and with irradiation of the 
reflex to the spinal ejaculatory center. The centripetally conducting tracts 
which produce these reflexes follow, at least in part, the course of the dorsal 
nerve of the penis (terminal branch of the common pudendic nerve) to the 
conus, thence through communicating branches pass to the ganglia on the 
floor of the pelvis and on upward into the tract of the three upper posterior 
sacral roots to the spinal cord. The function of the ascending centripetal 
tracts from the spinal cord to the brain is to convey to the central organ the 
sensory irritation affecting the erogenous zones as well as the filling and 
discharge of the seminal glands and the sensations produced thereby. Their 
cortical terminations and " transcortical " sensory continuations are as little 
known as the " transcortical " motor beginnings and the course of the cen- 
trifugal genital tracts starting from the brain, which apparently increase and 
also inhibit irritative conditions throughout the spinal cord, conveying them 
from the spinal cord to the previously mentioned sympathetic ganglia where, 
like the spinal nerves of the bladder, they terminate in these ganglia as pre- 
cellular fibers. 

According to our diagram, starting from the periphery and passing to 
the center, we may and must theoretically differentiate the following forms 
of impotence ; i. e., the suspension of the male genital functions : 

(1) Peripheral sensory impotence due to functional disturbance of the 
urogenital centripetal tracts which pass to the sympathetic reflex ganglia. 

(2) Peripheral motor {vasomotor) impotence from functional disturb- 
ance in the motor (vasomotor) innervation tracts of the corpora cavernosa — 
i. e., in the erigens nerve — of the smooth musculature of the spermatic duct, 
the seminal vesicles and the prostate gland, also of the bulbi and ischio- 
cavernosi muscles. 

(3) Sympathetic ganglion impotence caused by a disturbance in function 
of the sympathetic ganglia of the floor of the pelvis which control the reflexes 
of erection .and seminal discharge. 



990 



SEXUAL NEURASTHENIA 




(4) Spinal ganglion impotence from disturbance of function of the 
ejaculatory center situated in the lowest portion of the spinal cord (conus). 

(5) Spinal sensory conduction impotence from 
functional disturbance in the spinal portion of 
the ascending genital centripetal tracts. 

(6) Spinal motor conduction impotence from 
functional disturbance of the spinal portion of 
the descending genital centrifugal tracts. 

(7) Cerebral sensory and 

(8) Cerebral motor conduction impotence due 
to analogous functional disturbances in the intra- 
cerebral portions of the corresponding genital 
tracts. 

(9) Cortical sensory and 

(10) Cortical motor impotence from disturb- 
ances of function in the cerebro-cortical termina- 
tions of the affected tracts. 

(11) Inter central impotence caused, by dis- 
turbance in the function of the communicating 
tracts of the sensory and motor cortical termi- 
nations. 

(12) Transcortical x impotence through func- 
tional disturbance in the cortical centers beyond 
those previously mentioned and combined with 
them, these being higher psychosexual centers, 
the centers for memory and for sexual concep- 
tions. 

We are chiefly interested in the forms enu- 
merated in sections 1 to 4 because these are usu- 
ally combined or they occur successively and play 
the leading role in neurasthenic impotence. In 
1, peripheral stimulations are not conveyed with 
sufficient strength to the reflex ganglia. In 2, 
the reflexes produced in the ganglia are not 
sufficiently strong. In 3 and 4, the reflex trans- 
mission to the ganglion itself is markedly inhibited; in 3, in the sympa- 
thetic ganglia for the reflexes of erection and seminal discharge, and in 4, 
in the spinal center for ejaculation. If, therefore, the fourth form exists 
without the third, erections and the discharge of the seminal fluid may take 
place in the pars prostatica, the semen, however, at most is only exuded but 
no longer ejected. If, on the other hand, the third form exists without the 
fourth, it is a question whether the decrease in power exists only in the\ 
sympathetic center for seminal discharge or in the center for erection, or in 
both simultaneously. In the former case erections may occur without seminal 
discharge; in the second, seminal discharge without erection (a discharge of 
the semen from a flaccid, non-erect organ) ; in the third case the erections 




-©- 



Fig. 195. 



1 A designation for which we have analogous terms in 
and motor aphasia, which have lately become common. 



transcortical," sensory, 



SPECIAL SYMPTOMATOLOGY 991 

are feeble, weak, or not sufficiently prolonged and finally cease, the seminal 
discharges being scant and eventually absent entirely. In the fully developed 
cases belonging to this category there is, of course, no question of ejaculation, 
since no orgasm or sufficient erection and extension of the pars prostatica 
takes place. Before reaching these terminal stages which indicate decided ex- 
haustion in the genital reflex tracts and centers, in many cases a stage precedes 
which is characterized by signs of excessive irritability in the sensory {adduc- 
tion) tracts of the genital reflex arcs with a more or less developed exhaustion 
in the motor tracts (abduction). For instance, while the erections are usually 
weak and of insufficient duration, and the quantity of the discharge from the 
gland is decreased, the wave of irritation is still comparatively deep so that 
ordinary peripheral irritation by the accumulation of blood in the corpora 
cavernosa is no longer necessary, and the irritation is centripetally conveyed 
toward the spinal ejaculatory center and upward with abnormally decreased 
resistance, so that the periodic variations in the function of the three genital 
centers which follow one another are decidedly lessened, and the entire 
mechanism functions much more rapidly. 

While emission usually occurs only from the erect penis, the friction of 
which in the vagina produces a sensation of lust, and the irritation of the 
glandular secretion effused into the pars prostatica originates and increases 
the ejaculation which culminates in an orgasm — under circumstances de- 
scribed the irritation which leads to erection is sufficient, after the resulting 
emission, to cause a discharge from the seminal glands; simultaneously with 
this, the ejaculation of the semen takes place through reflex contractions of 
the ischio- and bulbo-cavernosi muscles. Therefore in the preliminary stage 
prior to impotence, the familiar and greatly dreaded early evacuation of semen 
occurs, " ejaculatio pmcox." 

Combined with the forms which have been minutely described in the pre- 
ceding, many others are observed in sexual neurasthenia. " Irritable weak- 
ness " in the region of the nervous genital apparatus makes it obvious that 
in neurasthenia we are not only dealing with a " neurosis " in the ordinary 
sense of the word but with a neuro-psychosis, and that the phenomena of 
functional change and disturbed innervation here observed have a more or 
less potent but invariable and permanent influence upon the psychical factors. 
While the activity of the cortical and "transcortical" (psycho-sensory) sex- 
ual centers from individual portions of the sexual apparatus is variously 
stimulated by centripetal paths — by the cerebrospinal conduction tracts which 
are thus brought into activity, and are indicated in the diagram by 5 and 7 — 
upon the other hand, from these sensory and also psychomotor sexual centers 
associated with them (intercentral), influences of partly exciting, partly 
regulatory, and partly inhibitory nature are conducted through the centrifugal 
conduction tracts 8 and 6 to the peripheral, motor, vasomotor, and secretory 
centers of the genital apparatus. In referring to what has been previously 
stated in regard to this subject, I need only emphasize that, from the una- 
nimity of combined psychical relations, this psychical deficit so constantly 
appears in the neurasthenic that, in the neurasthenic forms of impotence, we 
must often consider chiefly or almost exclusively " impotence of a psychical 
(psycho-genetic) origin." In the main this is due to some inhibiting influ- 
ence, neurasthenic obsessions and constrained conceptions concerning the 



992 SEXUAL NEURASTHENIA 

mechanical occurrence of erection and its associate processes, intraurethral 
discharge from the glands, the orgasm, and the ejaculation. Inasmuch as 
these inhibitive conceptions are usually the result of depression or the 
product of intense feelings of disgust engendered by former sexual acts or 
immediately following these, we have before us phenomena of psycho-motor 
inhibition due to excessive or anomalous psycho-sensory irritation; therefore, 
true symptoms of " irritable weakness." 

These residual conceptions of inhibition, according to their mode of de- 
velopment and the severity and frequency of the preceding sensations of aver- 
sion, may be of general nature or may be aroused by special conditions in the 
individual case, or they may be only slight and transitory individual factors. 
Where the latter is conspicuously the case, we designate these as " relative " and 
" temporary " forms of impotence, which are nevertheless always important 
psychical (psychogenous) forms. By this we mean that impotence is always 
associated with certain preconceptions and exists under definite circumstances 
(for example, in natural sexual intercourse, not, however, in unnatural modes 
of satisfying lust), or of definite persons (for example, of the wife, not, how- 
ever, for the mistress) — "relative impotence "; or it appears only periodically 
while at other times there is but slight or even unabated potentia coeundi — 
" temporary impotence" In relative impotence the sensations of aversion 
and the inhibitions consequent upon these are not generally associated with 
all but, in the main, with definite forms of sexual intercourse, or to sexual 
intercourse with certain individuals who for some reason have become re- 
pulsive and antipathic ; in " temporary impotence " the inhibitive influences 
which prevent the impulse are transitory and periodic, certainly are not always 
active to the same extent. Therefore " relative " like " temporary " impotence 
frequently corresponds to early stages of the affection, which gradually with 
a further increase of the inhibitive influence becomes absolute and permanent 
(total) psychical impotence. This terminal stage is usually reached only 
after a long time, if at all, and this depends greatly upon the individual con- 
ditions, the temperament, the character and mode of life, and especially also 
upon the morale and duration of the treatment which has in the meantime 
been instituted. 

Of course, in the female no condition arises which corresponds to male 
impotence. What is known in women as anaphrodisia, or frigidity, more 
closely resembles deficient sexual libido in the male; but it cannot be denied 
that the absence or insufficient power of erection of the clitoris may and quite 
frequently does exert an influence. This female organ is innervated from 
the vesico-vaginal plexus, the lowest portion of the inferior hypogastric plexus, 
as well as by the sympathetic, and in its development shows, quantitatively 
and qualitatively, i. e., in regard to its erogenous action, extreme variations. 
Naturally we learn very little in regard to this, for to the physician this area 
is in every sense a " noli me tang ere" and to the great majority of married 
men the physiology of the female organism is such a terra incognita that they 
neither acquire knowledge which can be utilized nor are they able to impart it. 
A certain amount of information on this point, as well as on other subjects 
relating to erotic functions, would in some cases do no harm and would 
probably prevent many a marital calamity. 



SPECIAL SYMPTOMATOLOGY 993 



(e) HYPOSPERMIA (OLIGOSPERMIA) AND ASPERMIA 

Among the symptoms of sexual neurasthenia, particularly in advanced 
cases accompanied by impotence, diminished secretion of the sexual glands 
(hypospermia, oligospermia) should probably be included — but concerning 
this we know very little. The secretion of the male genital glands is influ- 
enced by s} r mpathetic nerves which originate in the seminal and prostatic 
plexuses of the inferior hypogastric plexus. These plexuses, as well as the 
plexus deferentialis connected with them, furnish simultaneously the motor 
nerves, which, reflexly stimulated, bring about the discharge of the glandular 
secretion in the posterior portion of the urethra as the first stage of the 
process of ejaculation. It is not inconceivable that the stimulus originating 
from the periphery of the genital apparatus may change these motor and 
vasomotor processes of erection, as well as the secretory processes of the gen- 
ital gland, in a definite or modifying, quantitative, or qualitative manner. 
So far as the male generative gland and the secretion stored in the seminal 
vesicles alone come into consideration, the amount and consistence, the tur- 
bidity and viscosity of the seminal fluid, the number of seminal corpuscles 
contained therein, the appearance and composition and the motility and 
vitality of the latter may present varying conditions. But we have no definite 
knowledge concerning these conditions in sexual neurasthenia, particularly 
if we leave out of consideration those cases which are due to gonorrhea or a 
gonorrheal local infection in the form of posterior urethritis, prostatitis, 
epididymitis, etc., and to the more or less marked changes in the formation 
and composition of the secretion which may appear in the clinical picture. 

We are most likely dealing exclusively with such complications in the 
alterations of form and the changes in vitality of the spermatozoids which, 
under the designations respectively of " asthenospcrmia" (Fiirbringer) and 
" azoospermia " indicate decided diminution and suspension of motility and 
loss of vitality of the spermatozoids, which conditions are recognized and 
dreaded, and play an important part in male sterility. It may be regarded 
as certain that profound azoospermia, in particular, and consequently absolute 
impotentia generandi, in itself is never the result of sexual neurasthenia; on 
the contrary, these cases always prove the existence of another condition, 
particularly a bilateral gonorrheal epididymitis which is readily recognized. 
On the other hand, the possibility of a decrease in number (" oligozoosper- 
mia"), a decrease in size and also in the motility of the spermatozoids, can- 
not be denied in isolated cases of severe sexual neurasthenia; corresponding 
cases may be met with frequently in which pathologic emissions have oc- 
curred for years, probably with the usual simultaneous weakening of the 
potentia coeundi; in fact, a more or less decided decrease of the potentia 
generandi may appear in these cases. But the difficulties and sources of 
error in accepting and utilizing findings of this kind — resulting partly from 
the absence or admixture of the prostatic secretion with the secretion of the 
seminal vesicles, and from the decrease or lack of acidity of the prostatic 
secretion, etc. — are so important as to warn us not to be too hasty in the 
individual case in forming conclusions, especially from uncertain generaliza- 
tions of such individual findings. 

The rare condition known as " aspermia" in which there is complete 



994 SEXUAL NEURASTHENIA 

arrest of the secretion from the sexual glands, is even less likely than azoosper- 
mia to be related to sexual neurasthenia. In the majority of cases desig- 
nated as aspermia (or, more commonly, as " aspermatism") there is less of 
a suspension of glandular production than of excretion, and this is due to 
congenital or acquired mechanical excretory disturbances; for example, to 
strictures. 

ETIOLOGY 

It is difficult to obtain accurate statistics as to the relative and absolute 
frequency of sexual neurasthenia — as well as of neurasthenia in general. In 
the hospital and clinic, of course, the affection is not observed, and in 
polyclinics it is comparatively rare; nerve specialists, on the other hand, 
particularly in private practice, have frequent opportunities of noting the 
condition, the cases forming such a large proportion of their clientele that 
they are inclined to form exaggerated opinions concerning it. To give an 
approximate idea of its prevalence, I will state that among 3,414 private 
patients of whose cases I have made notes in the last four years 593 — there- 
fore, 17.4 per cent. — presented such symptoms that they may be designated 
as " neurasthenics " (excluding others which denoted complications, and also 
traumatic cases) ; among the 593 were 179 — therefore, 30.1 per cent, of the 
neurasthenics, and 5.2 per cent, of the total 3,414 patients — presenting the 
undoubted type of "sexual neurastl tenia." Upon the average, therefore, we 
may assume that of every three neurasthenics one is almost always a sexual 
neurasthenic, which may appear somewhat too high a proportion; it is prob- 
ably accounted for by the fact that sexual neurasthenics, from the nature of 
their affection, especially when there is decreased or total impotence (or when 
they believe this), are impelled to seek professional aid. 

Sexual neurasthenia occurs chiefly in the male; well developed, typical, 
pathologic pictures are not observed in the female, or are so rarely seen that 
we might be inclined to combine the pathologic conception of sexual neuras- 
thenia with other a potiori closely related symptomatic designations. Among 
the above 179 sexual neurasthenics there were but 6 women — only to 6 cases 
could I apply the designation sexual neurasthenia, and then only because of 
the symptom of pathologic pollutions (without other abnormal local finding) 
combined with decided and general neurasthenic symptoms. Two of these 
cases occurred in masturbators, in the anatomical sense both still "virgins," 
and of the psychologic type of the " demi-vierges " ; the others were married 
women, two probably being masturbators, and two addicted to homosexual 
practices. Analogous cases are probably not rare, but only exceptionally 
come under the notice of the physician — these cases are perhaps more familiar 
to gynecologists than to nerve specialists. In regard to the influence of age, 
sexual neurasthenia is, of course, a disease of puberty or of active sexual life, 
therefore prone to appear in the three decades of life between 20 and 50; 
but neither the earlier nor later life, at least in the female, is entirely exempt. 
The " pre-juvenile form " of sexual neurasthenia has been partially described 
under symptomatology, also a "pre-senile" form; but, under sexual neuras- 
thenia another must be considered which actually belongs to senility, and is 
characterized by decreasing power and growing antipathy to the ordinary 
methods of satisfaction, and results in an increasing tendency to sexual per- 



ETIOLOGY 995 

versions, to flagellantism, to sadism, to fetichism and to symbolistic acts, and 
the like. 

Among the "causes" or, more correctly, the conditions for the develop- 
ment of sexual neurasthenia, just as for neurasthenia in general, a peculiar 
neuro-psychic predisposition, a " nervous constitutional weakness/' as we ex- 
plicitly designated this in the introduction to this article, plays a paramount 
role. Nevertheless, not to exaggerate the importance of this constitutional 
factor, the original predisposition, we must not withhold the empiric state- 
ment that not a few persons become sexual neurasthenics who at the beginning 
showed no such predisposition, but in whom the development may be traced 
to purely local genital affections, to a " genital neurosis " which becomes 
widely distributed, or to a general neurasthenia. This is analogous to the 
conditions so frequently found in nervous diseases due to accidents, especially 
accidents due to occupation — in which at first a localized neurosis in connec- 
tion with the accident ("traumatic local neurosis") develops and subse- 
quently the slowly progressive symptom-complex of post-traumatic neuras- 
thenia (or hysteria). In other cases neurasthenic symptoms referable to 
other regions, or to general neurasthenia, long precede, and only when genital 
local affections appear in consequence of succeeding occasional or accidental 
damage does the symptom-complex assume the typical form and color of 
" sexual neurasthenia." 

Such an " occasional " or " accidental " damage, active in the development 
of the disease, is above all caused by local diseases of the male genital ap- 
paratus (especially from gonorrhea) — and onanism. 

Among the so-called infectious " sexual diseases," in a restricted sense, 
the chancroid plays no part, and syphilis only a comparatively subordinate 
role, in the development of sexual neurasthenia ; in many ways they may 
produce general neuroses in the form of neurasthenia and hypochondriasis 
(syphilophobia) — but never or only exceptionally with the local coloring 
which characterizes sexual neurasthenia. Here we do not so much consider 
" primary " local affections as the later and ultimate general consequences of 
syphilitic infection, syphilitic anemia and cachexia — to say nothing of nerv- 
ous syphilis or of suspicious post-syphilitic (" metasyphilitic ") diseases of 
the central nervous system which may still exist. The most weighty influence 
is, therefore, to be attributed to gonorrhea, the extraordinary nosogenetic im- 
portance of which was revealed to us by the pioneer discovery of the gonococ- 
cus by Neisser two decades ago, which gradually enabled us to comprehend 
fully some of the important findings in the pathology of these organs, and 
also in diseases of the nervous system, our study of which is as yet by no 
means concluded, being continuously complemented and broadened by fresh 
communications. 1 Here general neuroses (neurasthenia and hysteria), dif- 
ferent forms of nervous local diseases, neuralgia, muscular atrophy, gonor- 
rheal neuritis and myelitis, etc., are most important — in the latter conditions 
the toxic products ("toxins") which have developed in the course or re- 
mained after the cessation of chronic gonorrhea are decisive factors. The 
conditions which here interest us especially and which have been described, 

1 Compare my article, " Gonorrheal Nervous Diseases." Deutsche med. Wochen- 
schr., 1900, Nr. 43. 



996 SEXUAL NEURASTHENIA 

gonorrheal local affections of the posterior portions of the urethra, chronic 
and " latent " gonorrheal forms, neglected forms or those incorrectly treated, 
especially by too powerful or too protracted local treatment which has in- 
tensified the local condition, lead to the development of sexual, and subse- 
quently to general, neurasthenia. Perhaps one-half of all the cases of 
" neurasthenic " impotence are of gonorrheal origin, and in these cases we 
will almost invariably find that there was a preceding and prolonged inflam- 
matory condition of the posterior urethra, or even of the bladder or still 
higher portions of the urinary apparatus, which may still be present — in 
which condition we do not necessarily always find gonococci in the urine 
and in the urethral secretion, for gonococci encapsulated in the follicles of 
the urethra and prostate gland are impossible to demonstrate; besides the 
examination is often made much more difficult by secondary invasions and 
mixed infection (bacterium coli, etc.). This is not the place to discuss 
minutely this condition; in the individual case due stress must be placed 
upon the finding of gonorrheal local infection, of gonorrheal urethritis, 
balanoposthitis, cavernitis, epididymitis, prostatitis, spermatocystitis, etc., and 
upon the proof of prostatic urethral disease; the possible implication of the 
bladder, ureters, and pelvis of the kidney must also be seriously considered. 
An experienced specialist should be consulted, for a urethroscopic and 
cystoscopic examination for this purpose is often indispensable, and is simul- 
taneously of therapeutic interest. 

Local affections of the male urogenital organs, other than gonorrheal, are 
comparatively seldom the cause of sexual neurasthenia; but even these — 
phimosis, balanitis, varicocele, non-gonorrheal epididymitis, posterior urethri- 
tis, prostatitis — if there is no suspicion of infection, must always be consid- 
ered of pathogenetic importance. 

In all of these chronic, local affections it is not so much the nature and 
severity of the pathologic local changes as the intensity and long continuance 
of the irritation which acts upon the urogenital nerve terminations — for 
instance, in the " area sensitiva " of the prostatic portion of the urethra — 
and subsequently its general influence upon the nervous system; not rarely 
the psychical effect is of decisive importance. The latter becomes still more 
prominent in the second predisposing condition, onanism — which sometimes 
produces general and sexual neurasthenia not by itself alone, but by " ideal 
concurrence " with other deleterious agents, such as alcoholism, nicotinism, 
etc. It is erroneous to regard the influence of onanism as brought about, in 
the main, by consecutive local pathologic changes, by swelling and inflam- 
mation in the prostatic urethra, etc., or to regard such changes as specially 
significant. These opinions were formerly held, and are to some extent 
to-day, chiefly owing to the weight attached to the well-known views of 
Lallemand. In many cases the absence of findings in urethroscopic local 
investigations proved the fallacy of this opinion, which has been questioned 
for some time. On the other hand, in the immediate consequences we must 
differentiate sharply between those due to masturbation and to the psychical 
nature of onanism. In the former we seek evidence of the effect upon the 
sensitive nerves of the external genitalia, the glans, the prepuce, the skin 
of the penis, inevitable manual and mechanical irritation from friction or 
other acts of masturbation whereby erection, orgasm, and seminal discharge 



ETIOLOGY 997 

are produced reflexly through the sympathetic ganglia, and if the act be 
frequently repeated, or, without this, from slight exhaustion in the affected 
tracts and centers, it may cause neurasthenic changes in these areas of the 
nervous apparatus which may finally become permanent. These irritations 
from the nerves of the external genitalia (see Impotence Diagram, under 
~Ko. 1) are increased by irritation centripetally conveyed from the nervous 
apparatus of the urethra, also from the sympathetic centers, even when an 
orgasm has followed, and there is a consciousness of satisfaction, perceived 
as an increased sensation of lust, which may almost cause syncope. Hence 
the cortical, transcortical, and psychical centers may gradually be implicated. 
This occurs to the greatest extent in the cases in which from the onset there 
is uncontrolled, perverted action of the fancy, or onanism of the most ex- 
haustive kind. Xot rarely they are highly educated persons, people of in- 
tellect, who succumb to these refined but undoubtedly dangerous " psychical " 
forms of onanism. While the human idiot, like the ape — " simia quam 
similis turpissima bestia nobis" — in masturbation merely obeys a dull im- 
pulse without " thinking " of, or experiencing, anything more than momentary 
" animal " satisfaction, the normal human being, endowed with a higher 
intelligence and strong sensibilities, rich in ideas but weak in will power, 
may in a remote by-way lose himself and drift aimlessly into the dream-land 
of an erotic paradise, there to lose not only time and strength but the best 
of his personality and — only too frequently — lose it beyond redemption. If 
to this be added bitter self-reproaches, mortification and pain at the remem- 
brance of his own conduct which, nevertheless, he feels he cannot alter, above 
all tormenting fears aroused by baneful literature which irresistibly points 
to bodily and mental invalidism, not only the sexual neurasthenic develops 
but, as we have seen in countless instances, at the same time the neurasthenic 
hypochondriac, the tabophobe, and the paralysophobe. This psychical onan- 
ism also directly impairs the health by bringing about " neurasthenic im- 
potence " ; the subject is always dissatisfied, constantly seeking new experi- 
ences, and in choosing and basing his conceptions upon objects independent 
of time and space he finds sensations such as actual life can never furnish, 
or only in the poorest and most artificial form. All lawful pleasures and 
enjoyments which these realities can offer, or which are readily furnished, 
he accepts with feelings of unspeakable loathing, esteeming them lightly or 
hating them. A constantly increasing repugnance to legitimate marital and 
normal sexual intercourse, in fact to any kind, and a hungry seeking after 
sensations and perversities which he has not yet enjoyed or pleasures which 
have not yet been his ensues. The physical practice of masturbation, no 
matter in what form, does not as a rule lead to neurasthenic impotence (on 
the contrary, its injurious effect in this as well as in other directions is still 
greatly exaggerated), but the inseparable psychical reaction, the influence 
upon the character and the temperament, the consequent relaxation and de- 
pression which may be increased to hypochondriasis; in other cases the orig- 
inal psychical coaction combined with the exaltation of the imagination 
laboring in a pathologic direction, the fulfilment of the conception of a dream 
world filled with erotic pictures and scenes as remote from those of ordinary life 
as Heaven is from Hell, and the sovereign preponderance of dream creations 
compared with the impossibilities of dust-born reality demonstrates this fully- 



998 SEXUAL NEURASTHENIA 

Without an intermediate stage of sexual neurasthenia, onanism in its 
various forms and methods of practice is naturally the origin or immediate 
cause of many sexual anomalies and perversions (for example, mutual mas- 
turbation in patients, as well as also in girls, may favor the development of 
homosexual perversions). The minute consideration of this subject is remote 
from the purpose of this article. 

On the other hand, I may reiterate that onanism is in many eases not 
only the cause but in itself the symptom or sequel of sexual neurasthenia 
which has developed to an extreme degree. 

The effects of onanism are not markedly different in the female. If 
comparatively few sexual neurasthenics develop among the many female 
masturbators, this is due in the first place to the nature and manner of 
masturbation in women, the physical and, generally, the psychical reaction 
also being decidedly less than in men (this cannot here be explained more 
minutely) ; also by the circumstance that other neuropathic and abnormal 
conditions designated by different names, as dyspareunia, vaginismus, sexual 
hysteria, nymphomania, female sadism, tribadism, etc., not infrequently are 
produced by this cause. 

What is true of onanism is also true of sexual excesses and aberrations in 
general; they may be the causes, or the symptoms and sequels of sexual 
neurasthenia. Where they are active as etiologic factors there is, as a rule, 
already a more or less marked neuro-psychopathic basis ; nevertheless I have 
known the typical symptoms of sexual neurasthenia to develop acutely in 
previously healthy men immediately after very exhausting orgies. A gradual 
development of sexual neurasthenic symptoms is much more frequently ob- 
served in such men than in those who for years practise the congressus in- 
terrupts with their wives or mistresses. The explanation is obvious; the 
inhibitive exercise of the will is probably early interrupted, the orgasm never 
occurring normally, and the damaging effect of this reaction is to be sought 
in the genital centers of innervation. Cases of this kind in which, by desire 
of the wife, the congressus interruptus is practised year in and year out are 
becoming more and more frequent among that class of people denominated 
as of " education and property." Other practices in the realm of this so 
anxiously desired " facultative sterility " are less deleterious to the male, 
although these also, for example, the employment of condoms and, to some 
extent, exclusive pessaries, prevent satisfaction or make it difficult. Certain 
forms of anomalous intercourse, namely, fellation and irrumation, are ex- 
citing and therefore exhausting. The latter is little practised in Germany, 
and hence less dangerous than fellation which may be practised immoderately 
by experts in the art of love, and conceals a " snake among the Toses." All 
of these habits may sometimes produce merely impotence, at other times 
general neurasthenia with a sexual coloration, at other times pathologic fear 
or a suggestion of impotence is firmly rooted in the consciousness — a condition 
which must be distinguished from sexual neurasthenia in a restricted sense 
as " sexual hypochondriasis" although the distinction in individual cases is 
often quite difficult. 

Early acquired (or congenital?) homosexual tendencies and habits may 
only lead to sexual neurasthenia — but then very speedily — when such indi- 
viduals in opposition to their natures, for conventional reasons, at the urging 



PROPHYLAXIS 999 

of relatives, etc., consent to marriage — either thoughtlessly or to be obliging, 
with the hackneyed self -comfort that all will be right. But all is not right; 
on the contrary, everything is wrong; and then may follow bitter words of 
scorn such as the old c} T nic Martial 1 hurled at a candidate for marriage of 
homosexual tendencies who was dragged to the altar: 

" Heu quantos aestus, quantos patiere labores, 
Si fuerit cunnus res peregrina tibi." 

In these cases extremely stubborn forms of psychogenous impotence are 
prone to develop and are exceedingly difficult to cure, also general psychical 
depression and neurasthenic hypochondriasis. Almost as bad are those cases 
of young men in whom an ultra ascetic or too pietistic education has sup- 
pressed or completely arrested every impulse belonging to sexual life, and 
who are then without the slightest instruction or experience launched care- 
lessly into marriage. These cases in which there is a repugnance to the 
sexual act originating in exaggerated ethical and religious ideas, yet the 
performance of which in the nature of things is felt to be a duty, may by 
the combination of severest pangs of conscience and strongest copulative 
forces seem serio-comic to outsiders. But they may also have an unexpectedly 
tragic termination. I saw an instructive and lamentable case of this kind a 
few years ago in a count, aged 30, who, after being married for two years, 
committed suicide. 

On the other hand it would be considered a fairy tale — a fairy tale gladly 
heard and repeated — to state that sexual abstinence in itself is capable of 
producing sexual neurasthenia ; in such cases we can only consider the influ- 
ence of sexual abstinence when there are other complicating factors, neuro- 
psychopathic predisposition, faulty education and mode of life, a tendency to 
psychical onanism, etc., and when actual sexual life is refrained from or 
feared for other motives. In these exceptional cases the conditions may be 
similar to those previously mentioned. Finally, we can hardly assume that 
the excessive use of alcohol and tobacco is the sole cause of the development 
of sexual neurasthenia. Complicating factors must be added, although we 
cannot deny that these deleterious agents have a highly predisposing effect. 
In some cases of presumable impotence which I observed in men of middle 
age who drank little but were much addicted to cigarette smoking, it ap- 
peared on the whole to be due to a diminution of libido or even its complete 
disappearance rather than to impotentia coeundi in the usual sense. 



PROPHYLAXIS 

The prophylaxis of sexual neurasthenia of course is to a great extent 
the prevention of neurasthenia in general, but in part it belongs to the 
realm of sexual hygiene, a branch of public and individual well-being which, 
for many reasons, is still neglected and has not been generally recognized as 
important. Many prejudices confront us in this realm which necessitate 
delicate consideration and handling, and the free and unrestricted discussion 
of these conditions is almost prohibited in the public forum, and, in fact, 

iBook XL 78, 



1000 SEXUAL NEURASTHENIA 

by all classes of people. Prudery, which is not really indigenous to the Ger- 
man blood, has been artificially inoculated to the detriment of naturalness of 
existence, especially in regard to the processes of sexual life, and the censor- 
ship of the police has been established also in the realms of literary and 
artistic productions, even in that of the public press itself; in journals re- 
puted to be of interest to modest readers of both sexes this strict censorship 
presents insurmountable obstructions to any explanations or attempts at 
improvement in this direction. To mention one example, typical in its silli- 
ness, in an article of mine on " School Nervousness,' 5 published by one of the 
most prominent German belletristic journals, I found to my great astonish- 
ment that the word " onanism " had been stricken out. Therefore onanism 
is not to be mentioned to ears polite, and prostitution and sexual diseases 
still less so ! Under such circumstances how can there be a discussion of 
sexual hygienic questions and problems? May we not soon perceive that we 
are practising a thoughtless " head-of-the-ostrich-in-the-sand " policy in thus 
screening the shame of the people which proliferates fearfully in secret rather 
than uncover it to the full, healing light of publicity ? Perhaps the " German 
Society for the Repression of Sexual Diseases/' just formed, may gradually 
bring about an improvement in this matter. 

This is not the place in which to discuss the general corporeal and 
psychical tonic measures necessary to prevent neurasthenia, particularly in 
those congenitally predisposed and neurophathically tainted. The special 
prophylaxis of sexual neurasthenia necessitates, above all, educational influ- 
ences so that, on the one hand, sexual excitement in the growing youth shall 
be controlled and kept within bounds, and, on the other hand, that he shall 
receive at the right time and in a proper way the instruction which the 
young need. As to the exact mode in which this knowledge shall be imparted, 
general rules cannot be formulated; this must be left to the tact of the 
parents or other members of the family, to the teacher, or to the professional 
adviser who should attempt in each individual case to acquire an influence 
over the youth in question, and thus gain control of his psychical life. The 
worst breeding places for onanism are schools, pensions, and similar institu- 
tions (military schools had formerly a particularly unenviable reputation in 
this respect, perhaps not without justice). What the school may do for the 
prevention of onanism, Hermann Cohn x has stated in a small book which 
at the same time reveals the disastrous frequency of school onanism. He 
demands, first of all, that the pupils be watched by the teacher during the 
hours of instruction as well as during the intermissions for the prevention 
of mutual onanism — a requirement the fulfilment of which may unquestion- 
ably be greatly facilitated by an improved arrangement of class-rooms, par- 
ticularly by a more suitable position of the school desks, as well as by the 
prevention of the present over-crowding of most school-rooms. Cohn insists, 
too, that the teacher should warn the pupils of the injurious effects of auto- 
onanism as well as of mutual onanism, and that by means of lectures and 
pamphlets parents and guardians should have their attention called to the 
duty of teaching children the dangers of masturbation. As is obvious from 

i Hermann Cohn, " Was kann die Schule gegen die Masturbation der Schulkinder 
thun?" Berlin, Richard Schoetz, 1894. 






PROPHYLAXIS 1001 

the last statement, the school should not he expected to do all; on the con- 
trary, parents and family, to a much greater extent than has heretofore been 
the case, should remember their duties in this respect. Children who are 
inclined to onanism — the tendency often betrays itself very early, usually at 
the fifth or sixth year — must be carefully guarded both while awake and 
asleep, and, above all, exciting agents and evil companionship must be 
avoided; in boarding-schools, etc., common sleeping rooms should be strictly 
and most carefully watched. The most severe punishment should be inflicted 
on the one who starts mutual onanism; if necessary, expulsion from school. 
In auto-onanists — male and female — it should be carefully noted whether 
local irritation is present (among other factors in both sexes, oxyuris) ; this, 
however, is rarely a cause. A hygienic mode of life as to clothing, sleep and 
nutrition (the blandest diet, avoiding everything stimulating, especially alco- 
hol) ; systematic exercise even to extreme fatigue most effectively combats the 
morbid desire for masturbation. 

Another factor in prophylaxis consists in the prevention of venereal dis- 
eases, above all, in the prevention of gonorrheal infection, which we have 
learned to recognize as one of the most important and most widely distributed 
etiologic factors of sexual and subsequently general neurasthenia, especially 
of its most severe and most dangerous forms and sequels. What may be here 
accomplished is evidently not to be found alone in useful advice concerning 
personal protective measures (employment of the drop apparatus after 
Blokusewski and similar ones) and thereafter the earliest possible (abortive) 
treatment of acquired gonorrhea by antiseptic injections which are readily 
employed. Far more vital and important is the instruction, particularly of 
boys, as to the dangers of illegitimate sexual intercourse in general, above 
all of intercourse with prostitutes; this has, for example, been attempted by 
a society of physicians in St. Petersburg, 1 and also recently in an article 
from the pen of Prof. A. Herzen - addressed by German teachers to the 
students in the high schools. Much can still be done in this direction ; the 
difficulties mentioned at the beginning of this chapter must be gradually 
overcome by gravity and patience. The belief still prevalent among the 
young as to the dangers of sexual abstinence must be combated by scientific 
reasoning. Alcohol, which stimulates sexually, particularly in the ridiculous 
forms in which it is used by students in their drinking bouts, must be strenu- 
ously tabooed. Whether an early marriage or marriage at all is advisable 
depends upon many and varied circumstances ; very generally as it is done, 
I by no means advise marriage as a " prophylactic measure " for neuras- 
thenically predisposed individuals. Experience proves that marriage does not 
protect from onanism, does not prevent either temporarily or permanently the 
overpowering and long-continued desire for self-satisfaction by onanism, par- 
ticularly for " psychical onanism/' because for the predisposed individual 
marital pleasures are uniform and soon exhausted, while the pleasures of 

i " Zur Frage der sexuellen Hygiene." Yortriige, gehalten in der Sitzung des 
deutsehen arztlichen Yereins am 3. Mai, 1899, von Dr. Ernst Horschelmann, Ernst 
Blessig, Ernst Masing, Petcrsburger med. Woclienschi-ift, 1839-41, 1899. 

- A. Herzen, " Wissenschaft und Sittlichkeit, ein Wort an die mannliche Jugend." 
Yortrag, gehalten in Lausanne und Genf. Autorisirte Uebersetzung. Yerlag des " Ve- 
reines Jugendschutz," Berlin C 2, 1901, (Yorsvort von Adolf Harnack.) 



1002 SEXUAL NEURASTHENIA 

imagination present alluring forms and unceasing variety; of course these 
individuals are not restrained either by Church or State from practising new 
refinements and seeking illegitimate intercourse. Moreover, the neuropath- 
ically predisposed person lacks to a great extent the psychical quality of 
adaptation, the faculty of resigning his own to another individuality, even the 
degree of forbearance and abnegation so necessary for a happy or even toler- 
able existence in the married state. Only after carefully considering the 
conditions upon both sides and the character of the parties should a neuro- 
pathically predisposed person be permitted to marry, but marriage should 
never be lauded and extolled as, quand meme, an active preventive. 

TREATMENT 

Sexual neurasthenia admits of a uniform and specific treatment as little 
as it admits of a uniform prognosis ; on the contrary, we must be content in 
the individual case with the apparent causes, with the relation to general 
neurasthenia, with the special symptomatic indications. 

The treatment should be based upon the etiologic factors embraced in the 
preceding description, among the most important and most frequent of which 
are local diseases of the genital apparatus (particularly gonorrhea in men), 
onanism, the abuse of coitus, and sexual perversions. 

In regard to the first factor, existing local diseases, we must beware of 
over-estimating the value of local therapeutic remedies and agents at the 
onset. The statement, usually cautiously made during the treatment, 
" cessante causa, cessat effectus," may here be generally regarded as even 
less applicable, since no immediate causal relation between the local genital 
affection and sexual (and general) neurasthenia need by any means exist. 
On the contrary, as we have seen, the local affection is often merely an 
" accidental cause " which, acting upon a predisposed nervous system, pro- 
duces the neurasthenic disturbances, giving them special form and color, but 
does not actually produce them. The condition of irritable weakness in the 
nervous tracts and centers, nourished and maintained by local effect, may, 
therefore, after the local effects have disappeared or been removed, continue 
substantively and undiminished. These considerations should lead us to esti- 
mate with cool criticism the importance of local therapeutic treatment in 
sexual neurasthenia (also in the hysterical form) ; we should refrain from 
all superfluous or doubtful procedures, but proper local measures with a 
definite purpose should not be neglected or held in abeyance. It is sometimes 
very difficult to draw the line between " too much " and " too little," and 
in doubtful cases we should rather incline toward the side of the harmless 
" too little." If examination reveals the existence of gross structural changes 
in the male urogenital apparatus — especially chronic gonorrhea and residual 
changes in the urethra, bladder and prostate gland, which form by far the 
most frequent local affections leading to sexual neurasthenia, appropriate 
treatment is necessary. What can be done here — aside from the symptomatic 
treatment to be later described — belongs chiefly to the realm of the specialist, 
such as the treatment of gonorrheal and non-gonorrheal posterior urethritis, 
cystitis, prostatitis, urethral strictures, and hypertrophies of the prostate 
gland. In rarer cases diseases of the penis, phimoses, prepucial concretions, 



TREATMENT 1003 

balanitis and the like are the cause, and local measures (operation for 
phimosis, circumcision for a moderately long and hypertrophied prepuce, etc.) 
are indicated. The same views as to treatment are applicable in the sexual 
neurasthenia of the female, in so far as local genital diseases (caused by 
irritation of the vulva in masturbation, vaginal and cervical catarrhs, dis- 
placements, etc.) are, in the individual case, causes of disease or factors which 
increase irritation. On the whole, the greatest care in, sometimes even the 
avoidance or neglect of, active local treatment is advisable, since it is difficult 
to foresee to what extent this excessive irritability with its reflex and resultant 
psychical effects may be due to such treatment, or what undesirable changes 
in the general condition or the individual symptoms may be produced thereby. 
At all events it is wiser in cases of this kind to try to relieve the neurasthenia 
by general treatment, and to combat by palliatives the local effects which 
are not so urgent. 

Instead of the preventive measures which have been described under 
prophylaxis, an attempt has been made to treat onanism partly by mechanical 
and partly by physical and suggestive means; both, as is well known, being 
productive of but slight results. Some of the innumerable, mechanical, pro- 
tective appliances which have been so greatly lauded by their inventors may 
be regarded as useless toys, sometimes very uncomfortable, and only excep- 
tionally beneficial or protective. The relief of local, irritative conditions by 
suitable measures, if necessary even by slight operations (circumcision, cauter- 
ization of the male urethra and of the vulva), is permanent only in isolated 
cases. " Suggestive therapy," here as well as in many other diseases, has not 
fully met our expectations; the results are usually slight or temporary, often 
wholly lacking; nevertheless in severe cases this method may at least be tried 
— also for the sexual anomalies and perversions which are rarely etiologic 
factors. In other cases, particularly in impressionable persons and those 
whose intellects are of high order, hypnotism is often unnecessary; here 
occasionally the counsel of the physician, the appeal to reason and conscience, 
the awakening of the dormant powers of the will, the stimulation of the 
moral energy, while at the same time allaying the fears as to the presumable 
consequences of onanism which are often painted as dark as possible and 
declared to be insurmountable, will slowly but surely produce the desired 
result. 

The abuse of coitus, particularly the congressus interruptus which aims 
to prevent conception and of course necessitates its avoidance, perhaps the 
substitution of other anticonceptional processes which have lately been advo- 
cated in increasing numbers, and no single one of which actually prevents 
the condition, should be discountenanced. 

For the general treatment of neurasthenia, fundamental laws cannot be 
established nor minute directions be given in this article. One point only 
must be emphasized. It is well known that in many neurasthenics who come 
under treatment we attach great importance to the benefits to be derived from 
prolonged rest in bed with simultaneous hypernutrition and the methodical 
stimulation of muscular activity by massage and electricity. In the majority 
of sexual neurasthenics this mode of treatment is rarely indicated; on the 
contrary, from the beginning active muscular exercise to the point of fatigue 
by means of suitable gymnastics or sport, or, in fact, any bodily or mental 



1004 SEXUAL NEURASTHENIA 

exercise that they prefer, is more beneficial. Here we note simultaneously 
the salutary effect of psychical influence. It is necessary to remove the 
thoughts as far as possible from the sexual sphere, and concentrate them 
elsewhere; we must impress upon the minds of these patients, and steadily 
maintain, that man has higher and more important functions in life than 
those connected with the genital system ; we must endeavor to awaken interest 
in other things, and to provide them with work adapted to their powers and 
inclinations. Change of residence and climate, prolonged journeys upon land 
and sea, the new impressions which they receive and the new train of thought 
which follows the changed mode of life often assist greatly. Under all cir- 
cumstances the patient must be made to understand that he not only will 
recover, but also will most rapidly recover by a mode of life which conforms 
to all the requirements of rational hygiene, and which at the same time 
necessitates a strict and energetic self-denial — for example, in many cases 
sexual abstinence as well as the avoidance of smoking and drinking and other 
injurious habits of life. This psychical pedagogic influence, with the simul- 
taneous strengthening of the body and mind, and the most careful avoidance 
of all threatening and baneful factors, is the only one which, in cases at all 
curable, is sure to yield satisfactory results, not merely transitory or apparent 
results — while in these cases local and symptomatic treatment is often merely 
an auxiliary measure, rarely a necessity, the exaggerated use of which prevents 
or warps the cure. 

Nevertheless, some of the symptoms of sexual neurasthenia are in them- 
selves of such great importance, and may produce such an intense reaction 
upon the entire psychical life of the patient that, in many cases, we cannot 
refrain from considering these and attempting to combat them with all the 
remedies at our command. These are certain, so-called, "irritative symp- 
toms/' frequent emissions, spermatorrhea and prostatorrhea, frequent erec- 
tions (priapism) on the one hand, and " debilitative symptoms/' the neuras- 
thenic forms of impotence, on the other hand — although such a contrast as 
we have seen does not exist pathogenetically, and according to experience we 
are often in a position to treat simultaneously the symptoms of both forms; 
for example, frequent emissions and a decrease in sexual power, and our 
therapeutic indications must be upon this basis. 

In the symptomatic treatment of these conditions — as well as in the gen- 
eral treatment of neurasthenia — physical curative measures, hydrotherapy, 
balneotherapy, electrotherapy, and kinesitherapy, and regulation of the diet 
and the mode of life are of paramount importance, while drug treatment, 
except for a few remedies, is less valuable. 

The exceedingly numerous ways in which hydrotherapy may be utilized 
for thermic irritation also permit its varied employment in the conditions 
we are here discussing. Given a rational indication for hydrotherapeutic 
measures, we must proceed on the assumption that the fundamental activities 
are generally lessened by cold and increased by heat; this "law" does not, 
of course, embrace the very brief application of cold by which sensory as 
well as motor functions are frequently increased, and which acts favorably 
on exhaustion ; on the other hand, moderately warm temperature has a quiet- 
ing and anodyne effect. As a rule, considering these fundamental views, in 
cases of painful irritation, in dysesthesia and neuralgia, in dysuria and 



TREATMENT 1005 

strangury, in irritative secretory anomalies, or when emissions and priapism 
are frequent, we preferably employ warm water (35° to 32° C.) and rarely 
long continued applications of cold water; while, with symptoms of debility, 
especially in the neurasthenic forms of impotence, stimulating procedures 
such as alternate cold and hot applications are especially to be recommended. 
This indication is met by the very common use of sitz baths. When we are 
seeking to relieve pain and irritative phenomena, protracted, moderately warm 
sitz baths (once or twice daily) are most suitable, and may be continued from 
a quarter to one-half or even three-quarters of an hour; in very sensitive 
patients we gradually include irritation by heat. The same is also true of 
irrigations, of compresses, and of applications of dry heat. Where, on the 
contrary, we wish a stimulating effect, we may employ once a day either 
very quick cold or hot sitz baths, at a temperature of 40° C, from fifteen 
to thirty minutes. Flowing sitz baths, as well as sitz baths with an ascending 
douche and massage baths, may also be here considered. Two forms of local 
employment of hydrotherapy are by means of Winternitz's psych rophore and 
Chapman's back coil (as well as similar apparatus) which are much used. 
I employ the " psyehrophore," as a rule, in the irritative conditions previously 
mentioned, beginning with a temperature of from 30° to 25° C, gradually 
reducing it to 20° G. or lower, and simultaneously decreasing the time of 
application from 15 to 10 or even 5 minutes; this application is generally 
made every other day; but with very sensitive persons only twice a week. 
In cases of impotence hot water, 40° to 35° C, is applied with the psy- 
ehrophore for fifteen minutes. As Chapman's coils are kept on the body a 
longer time and gradually become warm, they also may be used in irritative 
conditions at a reduced temperature (20° to 15° C). After from one-half 
to three-quarters of an hour the water must be changed. These coils may 
be used while the patient is walking about, but when he is in the recumbent 
posture the pliable metal coils of aluminum, which are manufactured by 
different firms as coolers for the back, for the abdomen, for the penis, and 
for the scrotum, are preferable. For long continued application of heat the 
well known thermophore compresses, as well as electro thermic compresses, etc., 
may be used. 

Among the curative agents of balneotherapy in a restricted sense, the 
acratotherms of moderately warm temperature (Schlangenbad, Wildbad, etc.) 
as anodyne and quieting baths, and those which increase heat (Bormio, 
Gastein, Teplitz-Schonau, Kagaz-Pfafers, Plombieres, etc.) may also be em- 
ployed as stimulating remedies in sexual weakness. In the latter case the 
ultimate effect may be decidedly increased by the altitude of the spa in ques- 
tion (Bormio, Gastein) as well as by combining the baths with mechanical 
irritation, douches or massage. Sea battis, thermal salt baths containing car- 
bonic acid (Oeynhausen, Xauheim), and iron baths containing carbonic acid 
(Franzenbad, Marienbad, Driburg, Pyrmont, Cudowa, etc.) have the same 
effect. According to reliable observers an immediately stimulating sexual 
effect follows carbonic acid baths and gas douches, a fact which should receive 
greater consideration than has been accorded it by physicians or even, as a 
rule, by the management of spas (Franzenbad and Marienbad furnish excel- 
lent examples of facilities of this kind). 

Electro-therapy in the usual forms (hydro-electric baths, general frank- 



1006 SEXUAL NEURASTHENIA 

Ionization and arsonvalization, electro-magnetic therapy) may be resorted to 
for its anodyne effect on sensory conditions — as a local application, however, 
it shonld be employed with the greatest care and only by specialists. In this 
realm of disease the thoughtless, haphazard application of the galvanic or 
faradic current, often without reliable apparatus or with one which perhaps 
does not record the exact force of the current, by physicians and of course 
still more often by quacks, is an actual crime, and not only is electro-therapy 
thereby greatly discredited, but, what is worse, the patient is often seriously 
injured. For reasons previously stated a local treatment which does too much 
is, in the majority of cases such as we are considering, generally detrimental. 
I do not favor the frequent attempts at intraurethral as well as intrarectal 
galvanization and faradization. All of these processes — and also local massage 
which will soon be discussed — are well calculated to generate or to maintain 
a local functional irritation and, at all events, they direct the attention of 
the patient too much to the local affection, and prevent a soothing psychical 
influence or, at least, render it very difficult. From the standpoint which 
should be maintained in the neurasthenic forms of impotence, all local reme- 
dies, especially those which to the laity seem to be quieting, electric, local 
procedures, prove to be unpleasant and often painful. Naturally there are 
exceptional cases — individuals for whom a somewhat energetic local treatment 
is indispensable for its suggestive effect — but even in such cases I would urge 
the greatest care in the employment of intraurethral faradization and gal- 
vanization, and that galvanization be limited to the percutaneous (the cathode 
in the form of large pliable lead-plate electrodes placed upon the vertebral 
column, the anode as Hirschmann's scrotal electrode, or as a pliable plate of 
about 50 ccm., in the perineal region; where there are symptoms of irritation 
the stabile current, inclusion and exclusion; in debility these should be care- 
fully and alternately introduced at the conclusion of a sitting which should 
last from five to seven minutes). Where there is a large static machine, the 
employment of spark currents (currents from the external cover of the Leyden 
bottle or condensor plates) with a large flat condensor electrode along the 
vertebral column and a foot plate, this may be used for several minutes daily. 
If occasionally for special reasons we desire to employ intraurethral electriza- 
tion with the constant current the greatest care is necessary. The urethral 
electrode (the best is in the form of a Nelaton's bougie, well sterilized) is 
to be introduced within the prostatic urethra and, to lessen the local irritation, 
should always be combined with a positive pole while the cathode as a large, 
flexible, lead plate is placed upon the perineum, in the gluteal fold, or upon 
the sacrum. Inclusion and exclusion is with the rheostat, the current to be 
feeble (not more than 1 to 1.5 milliamperes), producing merely a faint local 
sensation; the current is to be continued from one or two to four or five 
minutes, and the duration gradually increased; the application should not 
be oftener than two or three times a week. Faradization through the urethra 
and rectum — the latter for the production of reflex stimulation, analogous 
to the treatment for enuresis — is less difficult to carry out, but, as may be 
assumed, is always of questionable value, or certainly has but temporary effect. 
Kinesitherapy (or, as some designate it, " mechano-therapy " ) — gymnastic 
exercises and massage — for general strengthening is exceedingly valuable in 
neurasthenics; its local employment, particularly massage, is subject to the 



TREATMENT 1007 

same criticism as electric local treatment. That not only the abdominal 
muscles but also the muscles of the floor of the pelvis — especially the levator 
ani — may be affected by systematic gymnastics is not a new principle although 
it has lately been again promulgated by Thure Brandt (for massage treatment 
in gynecology). Tapping the lumbar region, pressure over and shock to the 
hypogastric plexus and the pudendic plexus, are old and well tested Swedish 
movements — they were employed and taught in my father's Gymnastic Insti- 
tute fifty years ago — although modern masseurs are continually rediscovering 
and claiming them as the progeny of their own fertile brains. Instead of 
manual resistance, gymnastics, apparatus gymnastics (Zander) have lately 
been employed but without benefit, and the manual practice of shock massage 
has been substituted for and often supplanted by stationary and portable 
apparatus, vibrators with hand or foot motor, electro-motor, or carbonic acid 
motor, etc. 1 0. Preiss, in Elgersburg, has introduced "massage baths" 2 in 
which the effect of baths and massage is combined; the bath itself gives the 
massage, the water being set in motion by a sufficiently strong power (pressure 
of at least three atmospheres) after the patient is so fastened by a suitable 
apparatus that the water may act upon a desired part of the body. Preiss 
states that with massage baths given on this principle he has seen favorable 
results in sexual weakness with emissions. During the bath the patient was 
in the recumbent posture; the lumbar portion of the spinal cord and the 
region of the perineum were treated while he stood in the tub; the tempera- 
ture was either cool or lukewarm (18° to 24° C). In youthful masturbators 
of both sexes these baths have proven of decided value. 

In the treatment of emissions, spermatorrhea, and prostatorrhea, as well 
as in impotentia coeundi, Zabludowski has lately employed a system of mas- 
sage of the genital region combined with massage of other parts of the body, 
therefore more general massage. 3 According to Zabludowski these manipula- 
tions affect the genital apparatus and its adnexa, as well as the spinal cord, 
by way of the reflexes and radiation, the skin of the body, the muscles, the 
blood and lymph vessels of the entire body, finally, by stimulating conceptions 
in the cerebral centers. A definite series of effects is produced; the patient 
is manipulated in the recumbent posture, the right and left lateral positions, 
and the knee-elbow position, the patient lying upon the belly, and, if neces- 
sary, resuming the recumbent posture. This massage, with one or two treat- 
ments daily, should be continued, upon the average, for six to eight weeks. 
In sexual neurasthenia I do not regard this method as perfectly harmless, 
and great care should be exercised in the choice of cases, since, as a rule, for 
reasons previously mentioned, its use in masturbators and 3 r outhful patients 
with emissions, in fact, where irritative symptoms are present, is not advis- 
able. In some cases of neurasthenic impotence no benefit has followed its 
prolonged use, while marked improvement was promptly brought about by 
other remedies (for example, by injections of yohimbin). 

In the diet of sexual neurasthenics, just as with neurasthenics in general, 

i Vergl. J. Zabludowski, " Technik der Massage." Leipzig, Georg Thieme, 1901. 
2 0. Preiss, " Massagebade." Zeitschrift fur diatetische und physikalische Thera- 
pie, 1900, 1901, Bd, Bd. IV, Heft 3. 

s Zabludowski, " Zur Therapie der Impotentia virilis." Zeitschrift fur diatetische 
und physikalische Therapie, 1899, Bd. Ill, Heft 4, 
65 



1008 SEXUAL NEURASTHENIA 

all alcoholic drinks must be absolutely prohibited and foods containing 
alkaloids (coffee, tea, cocoa), as well as tobacco, must be decidedly limited. 
In the majority of cases total abstinence from stimulants, alcoholic as well 
as non-alcoholic, cannot be enforced nor is it always desirable, because the 
beneficial stimulation of the central nervous system, particularly the heart 
and vasomotors, by its careful use, cannot be entirely dispensed with. Much 
is accomplished if we induce the patient to give up alcoholic drinks, to limit 
the use of tobacco and, instead of strong coffee, to drink weak tea, cocoa, or 
substitutes for coffee (malt coffee). Spices also must be limited; in young 
neurasthenics especially, as well as adolescents in general, spiced food fre- 
quently does harm and produces severe nervous disturbances during the period 
of the body's development. What is true of most vegetable spices is also true 
of the essential constituents of meat broth, the extractive products of meat 
(the so-called meat stock) ; therefore " strong bouillon/' which is recom- 
mended over and over again as a presumably strengthening remedy, is for 
these cases decidedly objectionable. The same is also true of beef tea and 
the like prepared at home, for although they have a high nutritive value on 
account of dissolved proteids which they contain, they also have the same 
stimulating effect as ordinary meat broth. Since in nervous persons in gen- 
eral, the ingestion of meat is to be limited, and although strict vegetarianism 
is not to be advised, the albumin requirement of the organism should chiefly 
be satisfied by vegetables combined with animal products (milk, butter, 
cheese, eggs). For supper, at least, no meat should be taken under any cir- 
cumstances; the amount of food eaten should be small, and the food should 
be easily digestible. It should be eaten early, and afterward little or no fluid 
should be drunk (least of all beer, but preferably small quantities of car- 
bonated table water, or grape and other fruit juices free from alcohol). 

It may appear to be a contradiction of the foregoing when I say that, 
from most remote times, some foods, especially delicacies and spices, have 
been lauded as presumable aphrodisiacs, the belief being that they increased 
sexual desire. This was believed — partly because of the odor which char- 
acterized them — of animal and vegetable foods such as game, fish, caviar, 
oysters, truffles, celery, asparagus, chestnuts and the like, and still more so 
of strong spices (pepper, cloves, nutmeg, vanilla, etc.) and the more powerful 
alcoholic liquors. But the presumable aphrodisiac effect of these substances 
is just as chimerical as that of most aphrodisiac drugs; and, if they have any 
effect, it consists in stimulation and congestion, chiefly of the urogenital 
organs, and is therefore not harmless. 

In patients with emissions, the " hygiene of sleep " must be carefully 
observed. In addition to the restriction of solid and fluid food in the evening, 
we must endeavor to bring about free and regular bowel evacuations before 
going to bed ; habit has a great influence, but this must be assisted by suitable 
laxatives taken during the day. Patients must not go to bed earlier than 
two and a half to three hours after supper, they should amuse themselves 
by play or light reading, not too exciting, or by a short walk; the bed should 
be hard and cool, the night clothing and bed-covering light; therefore, no 
feather beds nor heavy blankets, and no large, soft, and heating pillows. 
Lying on the back during sleep should be avoided ; the legs should be propped 
high; if emissions have frequently occurred in the early morning hours the 



TREATMENT 1009 

patient should rise at proper times during the night (either by auto-sug- 
gestion or the aid of an alarm clock) to relieve the bladder. 

In drug treatment we consider the so-called anaphrodisiacs, i. e., those 
remedies which diminish sexual desire, also those which increase it, the 
aphrodisiacs, although the benefit to be derived from either is very doubtful. 
The anaphrodisiacs are especially recommended to combat the tendency to 
masturbation as well as to correct the frequent pollutions and erections, 
priapism, etc. The preparations of bromin and camphor are usually employed 
for this purpose; and monobromate of camphor, which is a combination of or 
substitute for camphor, is often used internally, 0.1-0.5 in capsule or pill 
at evening or several times during the day. Exceptionally sedatives and tonics 
(chloral hydrate and digitalis) are useful. Lupulin (glandulae lupuli), 
which contains an ethereal oil and the bitter product of hops, probably has 
no anaphrodisiac action, certainly it is very unstable and uncertain (dose 
0.3-0.6 in powder or pill). 

Among the aphrodisiacs, cantharides or Spanish flies (lytta vesicatoria) 
is first mentioned, the action depending upon the cantharidin (up to .5 per 
cent.) which they contain; but, on account of the extremely severe and, in 
many cases, dangerous irritation of the urinary passages which they cause, 
preparations of cantharides are rarely employed by physicians for this pur- 
pose; they are much used, however, in various love potions, bonbons, and 
pastilles (Diavolini, pastilles galantes) which are supposed to have an erotic 
effect, and which are still employed in Southern countries as they were in 
the time of Casanova and de Sade. Various kinds of beetle act similarly to 
cantharides, especially the melee varieties, among which the May worm 
(melee majalis) probably has this action because of the amount of cantharides 
it contains. The various resins and balsams, myrrh, galbanum, balsam of 
Peru, vanilla and crocus are illusions as far as their aphrodisiac effects are 
concerned, as is also probably true of phosphorus, including the nerve tonic, 
of the glycerin phosphoric acid preparation (lecithin) recently so much 
praised, and of arsenic. 

The effect of strychnin and of drugs containing strychnin is better, but 
they are undoubtedly dangerous, and must be given in comparatively small 
doses. Lately we have found a substitute in an alkaloid of the same group, 
and it appears to be the only effective drug aphrodisiac; namety, yohimbin 
(Spiegel). This is prepared from the rind of the West African yohimbin 
— also from the similar but weaker yohimbenin — and was isolated by Spiegel 
in the form of white crystalline needles; the alkaloid injected experimentally 
in rabbits and mice, and especially in dogs, produces enlargement and 
hyperemia of the sexual organs (the testicles and epididymis) with very 
marked erections. Given in larger doses it produces general spasms, and 
death from respiratory paralysis. For therapeutic purposes yohimbin tablets 
(each containing 0.005) are given internally two to four times daily, or a 
solution is prepared by dissolving the drug in 100 parts of distilled boiling 
water (10 drops of this solution correspond to one tablet; therefore 1.0 to 2.0 
in solution should be given in the course of a day). 

I have used these yohimbin preparations in numerous cases of neuras- 
thenic impotence in the last two }^ears, and in general am well satisfied with 
the results. The principal effect is that they cause erection, generally after 



1010 SEXUAL NEURASTHENIA 

using the remedy for one or several days, and thus make coitus again possible 
after it had been impossible for years; there are no secondary symptoms. In 
the first case in which I used it, a man about fifty years of age, owing to the 
absence of erections, had been unable for twelve years to practise coitus, but 
he reported in a few days that erections had reappeared and he was now 
able to perform this function. We cannot always count upon such brilliant 
results. In severe cases in which the remedy has failed to have any effect 
after long internal use, I have lately resorted to subcutaneous injections, 
and have, at least in cases of neurasthenic impotence, seen much benefit; for 
this purpose I use a two per cent, solution. The solution must be kept in a 
dark bottle as it is slowly decomposed by light and changed to a yellow color. 
A drop of chloroform assists in preserving the fluid. It is best to order 
only small quantities at a time, sufficient for from ten to twenty injections. 
The prescription is about as follows : 

^ Yohimbini Spiegel 0.2 

solve in 

Aq. dest. sterilisat 10.0 

D. ad vitr. fuse. 
M. D. S. : %f c yohimbin solution for subcutaneous injection (dose 
0.5-1.0). 

I begin with an injection of one-half the contents of a one gram syringe 
(= 0.01 yohimbin) ; this solution is injected upon the inner surface of the 
thigh, and if no effect is produced or if it is insufficient I increase the dose 
to 1.0; I have never had occasion to go beyond this. At first the injections 
are given daily, but after they have taken effect only every second or third 
day or even at longer intervals, and after about twenty injections have been 
given the drug should he discontinued for a considerable time. I have never 
seen any disagreeable secondary effects; in isolated cases (3, so far) a tem- 
porary chilliness and outbreak of sweat with weakness appeared after the 
first injection, but not subsequently. The effect, which is to stimulate 
erection, slowly appears; as a rule, in the morning after an injection on the 
preceding afternoon or evening. 

Although yohimbin can by no means be regarded as a specific for im- 
potence I believe that, at least in the treatment of neurasthenic impotence, 
it renders all other drug treatment superfluous. This is true not only of the 
previously mentioned " aphrodisiacs " but also of the various forms of or- 
ganotlierapy, of treatment with Brown-Sequard's testicular fluid (" se- 
quardine," liqueur testiculaire) which is usually diluted with an equal quan- 
tity of sterilized water, 2.0-6.0 and subcutaneously injected, also of its 
substitutes for internal administration, the " tabloids " of didymin (one or 
two three times daily) and similar preparations. The sperminum Poehl, 
which also belongs to this category, is an organic product of metabolism, 
undoubtedly valuable, which may be employed in neurasthenia as a general 
tonic, but is not a specific for impotence. It is generally used subcutaneously 
(a one or two per cent, solution kept in a sterilized bottle), but is also 
administered internally (as "essentia spermini," a 4 per cent, solution, from 
25 to 30 drops to be given in warm, alkaline, mineral water). 



TREATMENT 1011 

In conclusion we must refer to attempts which have been made to cause 
filling of the corpora cavernosa mechanically by the local use of instruments 
on the glans penis, thereby causing an erection and orgasm. The number 
of " Yenus-rings " employed for this purpose, also instruments bearing sim- 
ilar names which are often advertised in the newspapers as harmless (some- 
times as "rubber articles"), is quite large. Physicians will not advise the 
use of any such instruments. 

If the preceding description seems incomplete, if some points have not 
been discussed, it must be remembered that we are not dealing with the treat- 
ment of impotence as such, but only as one of the symptoms of sexual neuras- 
thenia, and that here and there we have digressed somewhat beyond this limit. 
In conclusion I must reiterate : All local therapeutic and symptomatic en- 
deavors, important as they may appear to be in the individual case, must 
be subordinated to a psychical, pedagogic influence — or effect this as auxil- 
iaries — the cure depending chiefly upon a quieting effect, upon the strength- 
ening of the will power, upon diverting the mind from the sexual sphere, 
upon stimulating the will to combat individual tendencies, and upon the\ 
stimulation of the mental and bodily energies by compensating exercise and 
activity. Only when this treatment is strictly adhered to will it be possible, 
with all our professional knowledge and experience, for us to bring about 
actual and permanent cures, not apparent successes such as are achieved by 
professional and non-professional charlatans, wonder-workers, quacks, hyp- 
notists, magnetizers and Christian Scientists. 



EPILEPSY 
By W. VORKASTNER, Berlin 

HISTORY 

Epilepsy, or better so as not to prejudice the case, the epileptic attack 
was known to the ancient world ; it has always been the subject of professional 
interest and constantly stimulated medical investigation to a high degree. 
The epileptic attack produces one of the most striking clinical pictures. This 
impression is reflected in a characteristic manner by the phantastic relations 
with which the underlying condition producing the epileptic attack has been 
surrounded. The best known designation perhaps is morbus sacer. This 
probably originated from the unsophisticated observation of the frequent 
delirium with ecstatic or religious coloring or even from the fact that persons 
of high mental development and of great prominence quite often are subjects 
of epilepsy. Celebrated historical epileptics are mentioned in all text-books 
and it is unnecessary to refer to them here.^ The belief in similar mysterious 
relations is betrayed by designations such as morbus dcemonicus, or the term 
which indicates a connection with the constellations, morbus astralis or morbus 
lunaticus. 

At the present time we have emancipated ourselves from views which 
referred the origin of epilepsy to the ominous play of supernatural powers. 
Nevertheless, and in spite of eager investigation for centuries, the final and 
intimate nature of this remarkable disease has not been completely unveiled. 
As we shall see, later on, it has been possible experimentally to produce this 
or that isolated component of the epileptic attack in animals; it is true the 
microscope has revealed minute changes in the epileptic brain; more than 
once we have thought to have neared the goal, but prosaic criticism pointed 
out that a phase of the attack was not the attack itself and that the mi- 
croscopic alterations were probably not of a primary, etiologic nature, but 
secondary and a consequence of the prolonged series of attacks. Thus we 
were finally forced to accept the humbled position, of bestowing upon the 
great X of the epileptic question a name, and, for this purpose, the term 
epileptic change was coined. That this is nothing more than a paraphrase 
and a tacit admission of our ignorance need hardly be emphasized. There- 
fore anyone who attempts, at the present time, to give a description of 
epilepsy stands by no means upon the firm foundation of positive facts. This 
existing uncertainty, however, goes still further; it includes the limitation 
and extent of the conception of epilepsy, and this may in itself be designated 
as exceedingly indefinite. 

Fere holds that epilepsy is only a symptom-complex, that the realm of 
1012 






GENERAL SYMPTOMS 1013 

so-called genuine or primary epilepsy will become appreciably more restricted 
the further we advance in our investigations. This prevalent symptomatic 
conception is associated with an extremely wide limit of the conception of 
epilepsy, for, Fere includes quite a number of pathologic conditions which, 
according to our present views, have nothing in common with epilepsy, such 
as angina pectoris, Meniere's disease, gastric vertigo, infantile convulsions, 
and above all Jacksonian epilepsy. Binswanger denies this predominant 
symptomatic character which would degrade epilepsy to a mere collective 
term. On the contrary, he adheres to the opinion that epilepsy from its 
clinical symptoms as well as from its course is a well characterized chronic 
disease of the nervous system. We shall follow this latter view in our 
description, for to gain a general survey this is necessary, and besides it is 
the opinion of the greatest majority of authors and probably deserves the 
greatest consideration. By this it is not intended, however, that we entirely 
reject the views expressed by Fere. 

A special description is probably required by the adjective genuine, by 
which the great majority of epileptic attacks are characterized, in contrast 
to the so-called symptomatic epilepsy. These 2 terms have been applied in a 
different sense and may, therefore, readily give rise to errors and misunder- 
standings. In using these terms the anatomical and etiologic condition has 
not always been kept in mind but only the clinical. Originally the designa- 
tion genuine epilepsy was intended to characterize the affection as a func- 
tional neurosis, as a neurosis without an anatomical lesion, without a known, 
definite cause. Symptomatic epilepsy, i. e., epileptic or epileptiform attacks, 
in which gross anatomical lesions were found which might be regarded as 
the direct causes of the disease, was contrasted to this. Among the cases 
that are due to a focal disease of the brain, such as encephalitis, some are 
characterized clinically and symptomatologically by the fact that during the 
attack, at least in the beginning of the malady, there are marked admixtures 
of partial (Jacksonian) epilepsy. In some of the other cases the character 
of the spasmodic attack and the course throughout correspond to epilepsy 
but there is no anatomical lesion. Therefore if we consider genuine epilepsy 
from a purely clinical standpoint, we may say that, in such cases, upon the 
foundation of a focal disease, genuine epilepsy has developed; perhaps to 
some a paradoxical manner of expression. The term symptomatic epilepsy 
has, now and then, been applied to convulsions; these may deserve the term 
symptomatic, but not that of epilepsy, according to the previous definition 
(uremic convulsions, convulsions from cerebral pressure, etc.). 

GENERAL SYMPTOMS 

Following these introductory remarks we shall first describe the symptoms 
of the individual attack and its varieties, then we shall enter somewhat more 
minutely into the genesis of the spasm and its accompanying conditions, as 
well as upon the nature of the hypothetical epileptic change, and, finally, the 
course of the malady which is characterized by the attacks of spasm, the 
etiologic factors, the differential diagnosis, and the treatment. (The mental 
disturbances have been discussed in the Volume treating of Disease of the 
Mind.) 



1014 EPILEPSY 

In many cases the epileptic attack is preceded by certain premonitory 
symptoms; these are partly of a subjective, partly of an objective, nature. 
We must further distinguish: prodromes, which may precede the attack for 
a longer or shorter time — hours or days ; then Binswanger's " remote pro- 
dromes " and symptoms which immediately precede the attack, while the 
patient is still fully conscious, these signalizing the approach of an attack. 
They have been designated " aura?" the term given them by Galen. Some 
authors confound these two varieties and include the " remote prodrome " 
with the aura. Clinically, it is true, there is identity between many of the 
prodromes and the symptoms of aura. We sometimes have the impression 
that among the prodromes there is, to a certain extent, a protracted or an 
anticipated aura. 

The " remote prodromes " relate principally to the psychic sphere. The 
underlying feature in the epileptic change in temperament, the abnormal 
irritability, is often very distinct for some time before the appearance of the 
attack. The patient resembles an explosive mixture; a spark, an insignificant 
cause, is sufficient to provoke a severe outbreak of rage; this may be accom- 
panied with an attempt at violence upon those about the patient. Occasion- 
ally there is more of mental depression; a heavy load appears to rest upon 
the patient, a gloomy apprehension of coming evil, of something productive 
of harm; he is uneasy, restless, and — like an animal suffering from hydro- 
phobia in the stadium melancholicum — cringes before his fellows. The de- 
scription " of being charged " has been used, something like the oppressive 
sultriness of the atmosphere before a storm; in fact the attack in such cases 
acts like a lysis, like a clearing thunder storm; the patient in contrast to 
other patients is relieved, as if a heavy load had been taken from him. Some- 
times a cheerful, exhilarated condition has been noted. In a few instances 
a conspicuous increase in the sexual desire has been reported; this was asso- 
ciated with erection and emission; it may appear also without preceding 
libido as a prodrome and is then included among the somatic symptoms. 
The somatic prodromes consist of a number of complaints which might be 
readily designated as neurasthenic : lassitude, headache, paresthesia, hyper- 
sensitiveness to light and sound, restless sleep interrupted by dreams. One 
of my patients always noted the approach of an attack by dreaming the night 
previously of corpses and coffins. Binswanger calls attention to the similarity 
of some of these prodromes with the symptoms of the stage of incubation in 
the acute febrile diseases. If we add to these the occurrence of heaviness in 
the limbs, nausea, vomiting, rushes of heat to the head, and chilliness, the 
resemblance is even greater. Often there are digestive disturbances (diar- 
rhea). Fere refers the continuous yawning which is sometimes noted in the 
prodromal stage to the insufficient respiration which was determined by him 
prior to an epileptic attack. Singultus and sneezing have also been noted. 
Vasomotor prodromes, such as erythema, urticaria, circumscribed swelling of 
the skin, cyanosis of the hands, as well as transitory cutaneous and mucous 
membrane symptoms, have been observed. (Case of Fere: a patient had a 
tickling sensation in the uvula followed by a guttural cough, 24 hours after- 
ward an epileptic attack.) Particularly in childhood short circumscribed 
or isolated muscular contractions have been seen to precede the attack for 
some time (Charcot, Ballet). 



GENERAL SYMPTOMS 1015 

The aura is very much more common than the remote prodromes. For- 
merly it was regarded as an integral constituent of every attack, but recently 
the regularity and frequency of its occurrence has been much questioned. 
Lasegue has been bold enough to state that : " no individual who has an aura 
is epileptic." In how far this is justified our later considerations will de- 
termine. It is a matter of fact that a large number of persons who present 
clinically the picture of genuine epilepsy have an aura. Delasiauve was the 
first to classify the various kinds of aurae; he mentions the sensory, those of 
the special senses, the motor, and the intellectual aurse; a classification which 
has maintained itself, at least in principle, up to the present time. 

The patient during the aura, as already mentioned, is in possession of 
his mental faculties which the aura reaches and there, by a process of memory, 
produces the conception of a coming attack. This conception is often ac- 
companied by intense fear, which — although not often — gives rise to a motor 
reaction, in the form of an attempt to run away. This forward running 
associated with fear is psychologic and should not be confounded with the 
aura cursativa which will be mentioned later on. Other patients behave in 
a different manner, and, with the signal of the aura, they may prepare for 
the attack by assuming a suitable position. The circumstance is worthy of 
mention that some patients, even after the aura has appeared, with all of 
their power attempt to maintain consciousness and thus abort the attack 
by an act of the will (Oppenheim). An attack need not necessarily follow 
the aura; corresponding to the condition of consciousness the memory of 
the aura is well retained by the epileptic ; only in cases of retrograde amnesia 
is it absent. 

In proceeding historically in the description of the individual forms of 
aura we must begin with the sensory or sensitive aura ; for the remarkable 
feeling of being blown upon, which some epileptics have at the beginning 
of the attack, has given the entire group the name aura (Galen). More 
common than this form of sensory aura is paresthesia, or even pain, some- 
times of a general nature, often localized. " A peculiar sensation rises from 
the feet, when it reaches the head, consciousness is lost." Local paresthesia 
often begins in the distal parts of an extremity advancing proximally. Often 
in an extremity there is a sensation of numbness which constitutes the aura. 
The mucous membranes may be the seat of local paresthesia; a patient of 
Fere suffered from burning in the urethra. It is of importance to state that 
a true globus may be present as an epileptic aura. The very interesting 
kinesthetic aura must also be included among the sensory ones : the patient 
has a sensation as if there were a change in position in one of his extremities. 
Vestibular hallucinations (a feeling of swaying) have also been reported. 

An aura of the special senses is more common than the sensory sort. 
Every special sense may be the seat of an aura, even that of smell and taste ; 
those of sight and hearing are, however, most common. There is sudden 
darkness, the patient loses sight, or he sees sparks, lights and lightning, a 
red sea of flame. Eed plays a great role in epileptic hallucinations, as well 
as in the hallucinations of sight, which are occasionally very complicated. 
The figures usually have something red about them, a red cap, a red shawl, 
or the like. I noted a peculiar visual illusion in a patient : his aura con- 
sisted in noting that the faces of those about him suddenly became pale and 
66 



1016 EPILEPSY 

corpse-like ; soon afterward he lost consciousness. The " increase and de- 
crease in size of objects " as an aura, sensu strictiori, belongs to motor auraa, 
as this is evidently due to a change in tonus of the muscles of accommodation. 
Oppenheim states that this condition is often associated with vertigo. In 
regard to hearing, the elementary hallucinations consist of groaning, whistling, 
rushing sounds, which form the aura. The attack is not infrequently ushered 
in by tinnitus aurium, or a sudden sensation of deafness, in one or both ears. 
Hearing melodies or akoasmia is rarer. One of my patients after a prelim- 
inary attack of tinnitus had a sensation " like the sound of well known voices 
heard in a dream/' but without being able to understand words or appreciate 
them. 

A motor aura is much rarer. This consists of isolated clonic contractions 
or of a tonic spasm of an extremity. This resemblance to Jackson's cortical 
epilepsy may give rise to errors in diagnosis; we shall return to this in the 
discussion of the differential diagnosis. Symptoms of absence of function 
in the form of a paresis or paralysis of an extremity have been reported 
(Nothnagel). The motor aurae in the form of coordinate voluntary move- 
ments (wiping the nose, etc.) or of locomotor movements are very inter- 
esting. The aura cursativa belongs to this category, consisting in propulsion, 
with the retention of consciousness and without a psychologic motive. Fur- 
ther among the motor aurae are: singultus, respiratory spasm (inspiratory 
spasm of Seeligmiiller), yawning, coughing, sneezing, and deglutition spasms; 
the 3 last named often associated with a corresponding sensory aura (tickling 
in the throat). The occurrence of aphasic speech disturbances must also be 
mentioned. 

Delasiauve's intellectual aura had better be called a psychic aura for, not 
only intellectual but mental disturbances appear as aurae. The latter appear 
as sudden sensations of fear or as an outbreak of anger; more rarely as 
sensations of happiness or liveliness. The first are shown by absent-minded- 
ness, by sudden inability to follow the train of thought, or the sense of 
what has been said, or as a constrained conception. A lightning-like review 
of a large number of memory pictures from the patient's life; or a so-called 
memory identification counterfeit (a feeling as if the patient had already 
encountered the same situation) is noted. 

The vasomotor auro3 consist of cardiac palpitation, congestion, outbreaks 
of sweat, numbness, or cyanosis of the fingers and toes, erythema of the skin. 
It must be remembered that a number of these vasomotor phenomena are 
secondary, due to fear of a coming attack. This is especially the case if, 
as is not uncommon, these vasomotor symptoms are associated with some 
other form of aura. In fact it may be stated at once that combinations of 
various kinds of aurae occur. The visceral aurce may be divided into the 
sensory, and into that form which passes to the special senses. The visceral 
sensory aurae consist of nausea, vomiting, a sensation rising from the stomach ; 
the motor visceral aura, of rumbling in the intestines, acts of vomiting, etc. ; 
strangury and tenesmus may be a visceral sensory aura as well as the con- 
sequence of motor phenomena. 

The Attack. — The first phase of the actual attach is the general tonic 
spasm. If the patient is standing he falls to the floor unconscious; often 
while falling there is a piercing cry. Whether the fall is the consequence of 



GENERAL SYMPTOMS 1017 

the loss of consciousness or independent of it cannot be answered; but we 
shall recur to this point later on. The fall is abrupt and occurs with great 
force so that serious injuries may result. The patient often falls upon his 
face, striking the forehead. Epileptics may often be recognized at the first 
glance by a deep cicatrix upon the forehead. But it is not rare for the 
patient to fall backward. The proximity of dangerous things, such as a 
heated stove, becomes disastrous to him. Especially if there be no aura severe 
injuries may result. 

Simultaneously with or immediately after the fall the tonic spasm begins. 
It is to be regarded as a rule that it involves both sides of the body in a 
uniform manner. The head is either somewhat retracted or the so-called 
"conjugate deviation" of the head and eyes appears (Binswanger does not 
mention this among the symptoms of the typical attack). Sometimes the 
head is inclined to one side and rotated slightly upward; if there is at the 
same time conjugate deviation of the eves, postures result that are very diffi- 
cult to define. In other cases the eyes are rigid, or rotated upward, the eyelids 
either wide open or spasmodically closed, the jaws tightly locked. If the 
reaction of the pupils to light is tested, it is most often found that it has 
disappeared; the corneal reflexes are also absent; the tendon reflexes cannot 
be tested on account of the general tonic tension (see below) ; the trunk 
either shows opisthotonos or emprosthotonos, rarely is there curvature to 
one side. The muscles of the neck stand out tensely and even those of the 
abdomen show tonic spasm; respiration is arrested. The extremities are 
either in a position of flexion or extension and are rigid; an attempt to alter 
the position of these spasmodic, tense members meets with the greatest re- 
sistance. Often at the onset of the attack, simultaneously with the lateral 
rotation of the head and eyes, we note the legs, which are separated, and the 
arms which are abducted and raised, assume positions of flexion. The fingers 
are mostly flexed; enclosing the thumb in the hollow of the hand has been 
regarded as an important criterium of the epileptic attack since ancient times. 
The face, which was at first ashy pale, gradually takes on a dark blue, 
cyanotic appearance. This tonic stage lasts but a few seconds. While the 
body is still tense (muscles of the trunk) the second, the clonic stage begins: 
in the large joints of the extremities there are brief spasmodic flexion and 
extension movements, the trunk also shows marked clonic contractions, the 
eyes move to and fro, the muscles of the face are in constant motion, the 
jaws are brought together exposing the tongue, which is also in clonic spasm, 
to severe injuries; bloody foam appears at the mouth, the patient gives 
expression to peculiar gurgling sounds, urine and feces are frequently voided 
involuntarily, provided this has not already taken place during the tonic 
stage. An emission of semen is rarer; vomiting is extremely infrequent. 
Gradually these clonic convulsions lessen in severity, there are longer intervals 
between them, which may sometimes be occupied by isolated tonic spasms. 
Finally they cease and there is a feeble tremor of the entire body. 

In addition to the convulsions just described there may be other move- 
ments in this stage which have a more coordinate character (kicking, etc.). 
In fact the convulsions of this stage are not uniform but are a mixtum com- 
positum and can only be called clonic cum grano sails. If there has been 
conjugate deviation in this stage there is commonly the " fatigue deviation " 



1018 EPILEPSY 

of the head and eyes to the opposite side. A deep sigh is often the termination 
of the attack in typical cases. 

Binswanger divides these clonic conditions into : 

(1) Locomotor movements (hitting, throwing, pushing and stepping 
motions) ; 

(2) Jerk-like contractions of the head, trunk, and extremities (especially 
of the proximal portions of the latter) ; 

(3) Tremor; 

(4) Isolated clonic convulsions of individual muscle groups, especially of 
the distal areas. 

Fere calls attention to the fact that the convulsions of the clonic stage 
frequently show a preference for one side of the body. The duration of this 
stage is from 1-5 minutes. 

In connection with the attack there is usually a deep sleep which lasts 
several hours. It is much less common for the patient to regain conscious- 
ness at once and to resume his work without noting any of the effects of the 
attack. The patient often has himself awakened from this post-paroxysmal 
sleep, but this is not advisable, as he is then tired, worn-out, " dizzy in the 
head," irritable, and out of humor. After being awakened in this manner 
it may happen that he is in a condition of " dream-like confusion " which 
corresponds to the post-epileptic dazed condition of epileptic mental disturb- 
ance. These post-paroxysmal phenomena are suggestive of restless sleep and 
apparently associated with dreams; the patient throws himself about, occa- 
sionally opens the eyes, mutters a few incoherent words, or performs some 
complicated movement. The more quiet and deeper the sleep the less diffi- 
culty does the patient experience after a spontaneous awakening. In rare 
cases the post-epileptic sleep is uncommonly profound, so that the patient 
does not react to any stimulus, the condition corresponding to coma. I 
recently saw a case in which there was persistent coma lasting 24 hours after 
an attack; the attending physician having made a diagnosis of meningitis. 
In these paroxysmal sleep and comatose conditions, it must not be forgotten 
that Babinski's toe reflex is commonly present upon both sides (as is the 
usual condition immediately after an epileptic attack) ; this is quite an im- 
portant diagnostic factor. Oppenheim's leg phenomenon has been frequently 
demonstrated. Furnrohr has demonstrated the presence of the deglutition 
reflex. 

After the Attack. — This leads us to the objective and subjective symptoms 
which remain after the epileptic attack. Fere has devoted special attention 
to these and by exact and persistent researches has enriched our knowledge in 
this realm of motor and sensory phenomena. Many patients suffer for hours 
and days. These symptoms relate to general discomfort, lassitude, debility, 
and difficulty in applying their thoughts. A slowing of associated activities 
may be recognized by determining the reaction time. There are often diges- 
tive disturbances (diarrhea). The temperature after the attack rises a frac- 
tion of a degree as may be seen from accurate temperature investigations. A 
great change in metabolism is indicated by the loss in weight which is fre- 
quently observed. Often there is albumin, more rarely sugar, in the first 
urine that is voided; polyuria is infrequent. Mendel and others were the 
first to call attention to an increased excretion of phosphoric acid. It was 



GENERAL SYMPTOMS 1019 

determined, by Lepine and Jacquin, that this increase concerns principally 
the earthy phosphates and not the alkaline ones, and Fere was led to the 
belief that the excretion of the total phosphates was not only due to an 
increase of metabolism, but also to a special nervous process. The entire 
question as to the phosphates, however, is a disputed point. The increased 
toxicity of the urine, discovered by Yoisin, must be mentioned. In the blood 
after the attack Henocque found a decrease in the reducing property of the 
hemoglobin ; decreased amount of hemoglobin with hyperglobulia ; Fere found 
an instability of the corpuscles (after being removed from the circulation 
they soon took on a spherical shape). Donne reported an increase of leuko- 
cytes; Hayem hematoblasts. We shall return, later on, to these investigations 
in metabolism, as they are the foundation of a special etiologic theory. Fere 
proved experimentally that the physiologic action of pilocarpin upon the 
secretion of sweat, after an attack, was decidedly decreased and slower than 
during the apparently healthy interval. On the other hand salivation has 
been frequently reported after an attack. Edema and erythema has occa- 
sionally been seen. In the cerebrospinal fluid, removed by lumbar puncture, 
I have been able to demonstrate a slight lymphocytosis after an attack. If 
this observation proves to be correct upon subsequent investigation, the in- 
ference will be obvious that we are concerned with a secondary condition due 
to a hvperemic process of the meninges. Donat found cholin in the cerebro- 
spinal fluid. 

Symptoms referable to the motor functions are regularly found in the 
form of muscular debility, but this is never so great that it appears clinically 
as paralysis ; it shows itself as a decrease of power. This may be easily and 
distinctly demonstrated if, as was done by Fere, dynamo-metric curves of 
the patient are taken during the period free from attacks. A slight paresis 
of the ocular muscles is shown by slight nystagmus or nystagmiform move- 
ments. The tendon reflexes, after an attack, are decreased, as a rule, after 
severe ones they are absent, so that in the degree of the decrease there is 
some measure regarding the severity of the last attack. Fere calls attention 
to the increase of the idiomuseular irritability. 

Severe general muscular debility is noted when quite a series of 'attacks 
follow one another in rapid succession. Then the clinical impression of 
paralysis is present. The patients, especially children, cannot stand, the 
movements of the arms are uncertain and ataxic. If, by way of a test, the 
child is stood upon its feet, it collapses ; the head falls forward or to the 
side. Speech is severely impaired, it becomes lalling or indistinct. This last 
named condition coincides with the fact that even after a single attack some- 
times slight speech disturbances are noted. Another sign noted by Fere, with 
the glossodynamometer invented by him, is that the tongue is always involved 
to an extraordinary degree in this post-paroxysmal weakness. Babinski's 
toe-reflex can usually be demonstrated. Thus a picture results which closely 
resembles infantile pseudobulbar paralysis. Only spasms are not present; 
on the contrary there is rather hypotonia; the tendon reflexes are not in- 
creased. The paretic symptoms disappear when the attacks cease; on the 
other hand the condition may become stationary, provided the attack continue 
in undiminished frequency. In a case that I saw recently there was a 
peculiarly distributed choreiform athetoid unrest over the entire body. Op- 



1020 EPILEPSY 

penheim reports the case of an epileptic, aged 11, in whom there was high- 
grade incoordination of all movements and dysarthria following a period of 
cumulated attacks. 

We cannot as yet discuss the question whether localized motor symptoms 
of paralysis may transitorily or continuously follow an attack — provided there 
are many. The same is true of transitory conditions of motor aphasia. 
Binswanger regards the existence of post-paroxysmal circumscribed symptoms 
of absence of function as a proof of the existence of a focus and hence an 
indication for operation. In favor of the fact that we are concerned with 
symptomatic epilepsy, in cases of a non-traumatic genesis in which there are 
localized post-paroxysmal paralyses, is this, that in such cases the attack 
differs from the genuine epileptic one (the attack beginning in one member, 
with distribution according to the Jacksonian type). The transitory post- 
paroxysmal monoplegias and hemiplegias then would indicate the clinical 
manifestations of a latent etiologic factor. Naturally only the most minute 
microscopic examination, in such cases, would reveal to us whether or not 
a focal disease was present. On the other hand it must be remembered that 
not infrequently atypical attacks of genuine epilepsy present distinct features 
of partial (Jacksonian) epilepsy (Binswanger). There are even cases which 
most closely resemble a cortical disease and Jacksonian epilepsy, in which 
no anatomical lesion has been found and these have in part (whether cor- 
rectly or not) been included with genuine epilepsy (Leo Miiller's idiopathic 
hemiepilepsy, pseudo-Jacksonian epilepsy). 

Oppenheim assumes that the more developed the post-paroxysmal paralyses 
and the longer they continue, the more probable is the existence of sympto- 
matic epilepsy. Binswanger reports cases in which there were originally no 
paretic symptoms pointing to the presence of a localized disease of the brain, 
the attacks throughout having the character of true epilepsy, which were, 
however, succeeded by more or less extensive transitory pareses, until finally 
well marked symptoms of paralysis dominate the inter-paroxysmal clinical 
picture. He believes that these are transitional cases between functional and 
organic epilepsy in that there are minute pathologic foci forming the starting 
point of the affection which cannot be recognized upon a macroscopic exam- 
ination of the brain. These cases are, perhaps, identical with Voisin's epilep- 
sia spasmodico-paralytica. 

A case that I saw recently illustrates the difficulties of the question under 
discussion. 

A boy, aged 13, presented nothing in his history of note, except that he was 
asphyxiated at birth and had a mild attack of measles at 4 years of age. When 7, 
peculiar attacks first appeared: the patient became pale, and with a rigid facial 
expression there was a semi-rotation about the longitudinal axis of his body. (The 
parents did not know to which side the rotation occurred.) The attacks then ceased 
up to his tenth year; upon their recurrence there was, in addition to the rotation 
of the body, a fall with loss of consciousness and involuntary evacuation of urine. 
The attacks then changed their entire character, for convulsions appeared which, 
according to the report of the parents, began in the left half of the face, principally 
implicating the left half of the body and these were succeeded by transitory weakness 
of the left half of the body. In these attacks there was involuntary passage of urine. 
The attacks that we observed corresponded to those of ordinary epilepsy, only the 
tonic tension of the left half of the body was distinctly stronger, there was conjugate 
deviation of the eyes to the right, consciousness was lost at once at the onset 



GENERAL SYMPTOMS 1021 

of the attack, when there was at the same time involuntary passage of urine ; neither 
did the clonic stage present peculiarities, at most a slight predominance of the con- 
vulsions upon the left side of the face; but the cessation of the attack was quite 
peculiar, corresponding entirely to the Jacksonian type. The convulsions first ceased 
upon the right side, then in the left leg, and, finally, there was only convulsion in 
the muscles supplied by the left facial nerve. After the attack there was transitory 
paresis of the left hnlf of the body, with an increase of the reflexes, and also Babinski's 
sign. After an accumulation of attacks the paresis became continuous. After these 
attacks were arrested by the administration of chloral, there was a distinct tendency 
to improvement of the paresis. 

Any of the organs of special sense, just as they may be the seat of an 
aura, may present residual symptoms of absence of function. A limitation 
of the field of vision was noted by Fere in all patients in whom an examina- 
tion had been made during the first 30 minutes after the attack. Thomsen 
states that he has noted these symptoms of absence of function only after 
the psychic equivalent of the epileptic attack. Bilateral or unilateral deaf- 
ness, decrease of the acuity of hearing, have been reported as post-paroxysmal 
phenomena. A very interesting observation has been recorded several times 
(Charpentier), namely, simultaneously, anesthesia of the pinna and of the 
mastoid process upon the affected side. In post-paroxysmal amblyopia, there 
is said to be a similar condition in the region of the eye. The most common 
disturbance in sensation is a diminution of the pain sense which continues 
for a long time. By some authors this is associated with the slowing of the 
associated activities which exists after the attack. In its most severe grades 
(epileptic stupor) it is particularly well developed. Localized disturbances 
of tactile sensation are often found in combination with local motor paralyses. 
Finally, post-paroxysmal ageusia and anosmia must be mentioned. 

Varieties of Attack. — The epileptic attack does not always present a 
typical character. There are deviations and varieties which are just as 
necessary for the physician to understand as the appreciation of the attacks 
that run a typical course. These atypical attacks appear either between the 
typical ones or exist alone forming the exclusive clinical picture. Binswanger 
differentiates the following varieties: 1. The fully developed atypical attach, 
2. the rudimentary attach, 3. abortive attacks. The plainest case is when 
all phases of the typical attack appear and only present an irregularity in 
their course. Thus it may occur that the clonic stage precedes the tonic, 
or that clonic and tonic spasms alternate, or are present in different members 
at the same time, further that the tonic spasm appears before consciousness 
has been lost so that the patient notes the " stiffness " of his limbs. Bins- 
wanger points out that in these latter cases the tonic convulsion does not set 
in as a generalized spasm but that it is frequently unilateral, or in the form 
of paraspasms. These fully developed atypical cases are contrasted with the 
rudimentary attacks. Here, besides the loss of consciousness, there is only 
a single component of the convulsion, clonic or tonic spasm, or they are sub- 
stituted by coordinate or locomotor movements, in part or entirely. Loss 
of consciousness and partial tonic spasm is quite common in the apoplecti- 
form variety of epilepsy; this is often confounded with conditions of syncope. 
In these cases the tonic spasm, if present, affects the muscles of respiration, 
so that breathing is arrested and cyanosis appears. The patient is entirely 
immobile as if in the deepest syncope, but even in the absence of tonic spasm 



1022 EPILEPSY 

of the muscles of respiration, there is often involuntary evacuation of urine. 
The pupillary light reflex is absent. Loss of consciousness and clonic con- 
vulsions are also noted in other cases that closely simulate syncope, and in 
which the flaccid and motionless patient presents momentary spasms. Fur- 
ther, those cases which resemble the infantile tic de salaam or hysterical 
shaking spasm belong to the category of Binswanger. There must be in- 
cluded here also those cases in which there is tonic spasm, in the members 
of one lateral half of the body, perhaps in an arm, while the other side of 
the body shows coordinate movements — most frequently there are brushing 
and grasping motions. I recently saw a case in a boy, in whom, besides the 
lose of consciousness, there was tonic spasm only. During the time of the 
generalized tonic tension of the body rhythmic masticatory motions were 
noted. This observation leads us to the discussion of cases in which the 
tonic or clonic spasm is replaced by coordinate or locomotor movements. 
Among these is the so-called " manege epilepsy " and epilepsia rotatoria, in 
which there are circular movements upon the axis of the body performed 
while the patient is unconscious, and epilepsia procursiva, in which there is 
unconscious propulsion and then the patient falls or in which these move- 
ments cease upon regaining consciousness. Epilepsie retropulsive has been 
described, and epilepsie marmottante must be mentioned, in which there is 
the repetition of words without sense, the patient being unconscious. 

The final group of atypical cases of epilepsy includes those in which either 
the motor spasm or unconsciousness is lacking or in which one of these 
components is merely indicated. Here we must mention first : the lightning- 
like, transitory, abortive attacks or petit mal, so-called since antiquity. Dur- 
ing these attacks the patient suddenly with visible pallor (or blushing) ceases 
his activity, permits an object to fall out of his hand, or suddenly stops in 
the street while walking, looks rigidly into space, immediately resuming his 
walk or going on with his previous occupation. The knowledge of these 
forms of the attack, although quite ancient, has unfortunately not yet become 
the common property of physicians, so that many cases of this kind remain 
unrecognized. I cannot refrain, at this point, from paying a tribute to the 
excellent faculty of observation of the Berlin school teachers, who send many 
patients of this kind to the Clinic, often with a complete diagnosis. These 
patients frequently do not know of their attacks, provided there is no aura; 
not rarely the loss of consciousness is not so profound, the memory of the 
attack, therefore, being retained, it is regarded by the patient as an attack 
of vertigo or 'syncope (vertigo epileptica). Often there are isolated motor 
symptoms mixed with these abortive attacks; slight contractions in an ex- 
tremity or still more frequently coordinated movements (snuffling, smacking 
the lips). Involuntary evacuation of urine is not rare. I have at present 
under observation a child, who during an attack of petit mal while eating 
does not cease chewing, becomes pale, and gazes fixedly into space, not re- 
sponding to questions. 1 In other cases of petit mal the motor symptoms, in 
proportion to the loss of consciousness, are more prominent. These may 
consist in isolated clonic contractions or in a brief tonic spasm of an ex- 

1 It must be remembered that in some attacks only inhibitive processes affect the 
cortex, while the subcortical centers that bring about the activity of the principal 
movements (perhaps also mastication) continue with undisturbed function* 



INDIVIDUAL SYMPTOMS 1023 

tremity. If there be no loss of consciousness these cases are very difficult 
to differentiate from motor aura?, especially if no attack follows. Vasomotor 
symptoms (pallor) are present as a rule. Some few, isolated, muscular 
twitchings may appear inter-paroxysmally and we must not fail to include 
Unverricht's cases in which there is a combination of myoclonia and epilepsy. 
Then there are also cases in which the patient, without loss of consciousness, 
suddenly collapses in the knees or falls and immediately rises; attacks which 
Binswanger characterizes as isolated discharges of inhibition in the motor 
regions, corresponding to his genetic theory, which will be discussed later on. 

Another variety of the attack must be described in which vasomotor 
symptoms are very prominent, these cases having been given the name 
vasomotor epilepsy. These attacks run their course with profuse outbreaks 
of perspiration and a rise in temperature; the amount of urine is greatly 
decreased. 

When we refer to the epileptic equivalent we mean the psychical paroxysms 
of epilepsy. The term equivalent is also employed for a number of neuro- 
pathological phenomena. The so-called epileptic vertigo, in the majority of 
cases, is not true vertigo, but an attack of petit mal,.with but slight disturb- 
ance in consciousness. But true attacks of vertigo may also occur as epileptic 
equivalents (Fere). There must also be mentioned outbreaks of perspiration 
and attacks of tremor (Fere), as well as attacks of narcolepsy, from which 
the patient cannot be roused. Oppenheim mentions, as an equivalent of this 
kind, a comatose condition lasting several days. 

INDIVIDUAL SYMPTOMS 

In proceeding to discuss the individual symptoms of the epileptic attack, 
quite a number require but a brief consideration, for they are only the sec- 
ondary results of some of the important manifestations of the attack. For 
a considerable number an explanation, or more correctly an attempt at an 
explanation, will be found, when we have learned to understand the mechan- 
ism of the attack. The initial cry belongs to the first group and is due to 
the air being forced through the spasmodically narrowed glottis, the muscles 
of the abdomen and of respiration being in tonic spasm. The old view of 
Marshall Hall must be mentioned, in which the tonic spasm of the muscles 
of the larynx was regarded as the primary condition and all of the succeeding 
conditions as the result of a carbonic acid saturation of the blood (trachelis- 
mus), a view which was very easily disproved for, after producing a fistula 
in the trachea, the attacks did not cease. Spasm of the muscles of respiration 
and the dependent dyspnea certainly cause the increasing cyanosis, which 
succeeds the initial pallor. Small cutaneous hemorrhages (or hemorrhages 
from the mucous membrane: conjunctiva), are probably due to venous stasis; 
these are often seen as the residues of the attack. Binswanger points out 
that the region about the mastoid process is the area of predilection of these 
hemorrhages. Others refer these small bleedings to the greatly increased 
blood pressure which is said to be present at the beginning of the attack; 
this has, however, not been definitely proven. Other investigators maintain, 
on the contrary, a fall in blood pressure at the onset of the paroxysm. Thus, 
it was noted that a bleeding wound of the forehead ceased to ooze blood at 



1024 EPILEPSY 

the beginning of the attack; Binswanger, however, points out that this may 
have been due to a tonic contraction of the muscles, thus causing the hemor- 
rhage to cease. Investigations of the blood pressure by the sphygmometer 
during the attack encounter great technical difficulties so that a definite con- 
clusion cannot be obtained. The reports in regard to the vessels of the eye- 
ground, from which it was expected that an idea might be obtained as to 
the condition of those of the body and especially of the cerebral arteries, 
differ to such an extent that they are valueless. Sometimes dilatation is 
found, other observers report contraction of the retinal arteries. Bechterew, 
from investigations in dogs and cats, arrived at the conclusion that during 
the entire course of the attack there was raised arterial and venous pressure. 
Examinations of the cerebrospinal fluid by the same author render it likely 
that there is an increased arterial flow to the brain. My finding of a post- 
paroxysmal lymphocytosis is in favor of the same view. 

Among the sequels of the clonic stage there must be mentioned the in- 
juries to the tongue; this organ is tossed to and fro between the jaws during 
the clonic convulsion. These to and fro movements of the tongue probably 
also produce an increase in the salivary secretion, and, partly from the 
effects of the spasm of the muscles of mastication the saliva becomes foamy 
and intimately admixed with the blood which originates from the bitten 
tongue; then it appears as bloody foam at the lips. The clonic contractions 
of the diaphragm explain the peculiar sounds which the patient emits. Evac- 
uations of urine, feces, and semen are most likely due to an involvement of 
the smooth muscles in the convulsion during the tonic and clonic stages, or 
they are the consequence of the effect of spasm of the transverse striped 
muscles of the abdomen, anus, perineum and sphincter of the bladder. The 
same considerations may be invoked to explain the vomiting, in which prob- 
ably also an accumulation of swallowed air, due to spasm of respiration and 
deglutition, plays a role. 

THEORIES OF EPILEPSY 

Passing to the origin of the tonic and clonic spasms, as wen as the other 
symptoms of the attack, we must first investigate the theories of the causation 
of the epileptic insult and the seat of the epileptic lesions and in this connec- 
tion inform ourselves in regard to the results of animal experiments. The 
historical development of these theories gives us at the same time an inter- 
esting picture of the evolution of our views in regard to the functional 
activity of individual portions of the brain. So long as it was maintained 
that the cortex of the cerebrum was not concerned with the motor functions, 
these being associated with the mid-brain, the " medulla theory " of epilepsy 
was in bloom. When Fritsch and Hitzig published their celebrated discovery 
in 1871 upon the electric excitability of the cerebral cortex and thereby de- 
prived the old Flourens theory, which stood for the functional equality of 
all portions of the brain, of all foundation, it was obvious to assume that all 
the symptoms of the epileptic attack were due exclusively to processes that 
affected the cortex of the cerebrum. We will see that here also we have 
taken too much for granted and that the true solution is probably between 
these views. 



THEORIES OF EPILEPSY 1025 

The medulla theory was favored by Kussmaul and Tenner. It was based 
upon the observation that if the arterial supply in animals was cut off 
(hemorrhage, bilateral ligature of the carotids) this was capable of producing 
epileptiform convulsions. These convulsive movements, consisting in striking, 
pushing and treading, also took place when the cortex of the brain and the 
basal ganglia were removed. In the human epileptic attack this cutting off 
of the arterial blood supply was supposed to result from a primary stimulation 
of the vasomotor centers in the medulla oblongata. The resulting anemia 
of the cerebrum gave rise to loss of consciousness; that of the mid-brain 
causing convulsions. 

Binswanger raises the following objections to this view: 

(1) Kussmaul and Tenner did not succeed in demonstrating that the 
medulla was actually the region causing the convulsion, for later experiments 
(Frensberg, Luchsinger, Schroff) proved that the spinal cord in itself, after 
being separated from the medulla, was capable of producing similar spasms; 

(2) It could not be proven that an irritation of the vasomotor center 
in the medulla oblongata gave rise to an anemia of the cerebral cortex. On 
the contrary, the investigations of Eiegel and Jolly demonstrated that neither 
section of the cervical sympathetic nor irritation of the central stump had 
any influence upon the filling of the vessels of the pia. 

The medulla theory appeared to gain some confirmation in the path- 
ological findings of Schroder van der Hoik's, who found small vascular 
dilatations in the fourth ventricle, and in the experiments of Nbthnagel, who 
succeeded in producing spasms in animals by mechanical irritation of some 
points in the medulla oblongata. Xothnagel modified the medulla theory 
to the extent that simultaneous and coordinate with the irritation of the 
vasomotor center which produces anemia of the cerebral cortex, there is an 
irritation of the " hypothetical spasm center " in the pons. That this irrita- 
tion of the " spasm center " in animal experiment may be merely reflex is 
emphasized by Xothnagel as well as by later investigators. The transmission 
was supposed to occur by way of the sensory trigeminus root. NothnagePs 
experiments on rabbits were later repeated, extended, and supplemented by 
Binswanger and Ziehen. If we study the results of these experiments, which 
refer in part to the region of the pons (Binswanger), in part to the corpora 
quadrigemina and the optic thalamus (Ziehen), 3 series of associated phe- 
nomena appear: 1, tonic or tetanic tension of all of the muscles of the body; 

2, associated movements, such as kicking, stepping, running and tramping; 

3, locomotor movements. As these symptoms appear in the picture of spasm 
even after the influence of the cerebral cortex is cut off, the assumption is 
justified that in the cerebral areas under discussion there are special centers 
for these previously mentioned movements. Pathologic irritation causes them 
to become spasm centers; their physiologic significance being similar, par- 
ticularly the last 2 named varieties, in which we have but an abnormal in- 
crease of the physiologic form; occasionally this may be quite different and 
is true especially of the tonic spasm. Binswanger was unable to produce 
associated movements from these areas of the brain in dogs nor has it been 
possible to produce them from higher portions of the canine- brain. Bins- 
wanger deduces, from this fact discovered by him, that even in the dog, the 
view of a pure medulla origin of the epileptic seizure is not justified. 



1026 EPILEPSY 

The medulla theory received a severe blow by the Fritsch-Hitzig discov- 
eries and above all by the further fact reported by Hitzig, that in the dog, 
after extirpation of the motor cortical centers, there may be spontaneous 
recurring attacks which very closely resemble the epileptic seizure in man. 

This at once altered the entire position of affairs and the cortex of the 
cerebrum was looked upon as the point of origin of the epileptic attack and 
very soon this region was regarded as the only area from which the symptoms 
of the seizure could be produced. Unconsciousness, as a clinical symptom, 
was readily understood from this new conception. The clonic contractions 
originating from irritation of the cortex, which first appeared isolated in 
circumscribed muscle areas then being distributed in conformity with the 
anatomic law of position in relation of the cortical centers, did not resemble 
the epileptic seizure in the human being very closely; nevertheless, there 
could be but little objection to the above mentioned view, for, not only could 
the clonic component of the epileptic attack be produced by cortical stimu- 
lation but also the second component, the tonic convulsion, was brought about 
by the application of stronger currents. 

Even to-day numerous exponents of the pure cortical theory, as described 
above, regard the tonic spasm as the effect of an extraordinarily strong sum- 
mation of irritation; a single one being capable of producing a clonic con- 
traction. Gowers is of the opinion that it is the venous stasis which causes 
the tonic convulsion in the second stage of the epileptic seizure to dissolve 
itself into clonic convulsions. But we have already seen that tonic spasms 
and other symptoms (motions of running and walking) which very closely 
resemble some of the phenomena that were described in the symptomatology 
of the epileptic attack, may be produced in animal experiment by irritation 
of infracortical regions. 

The experiments of Ziehen have rendered it very likely that even the 
contractions obtained upon cortical stimulation may be subdivided into cor- 
tical and infracortical components. Ziehen extirpated a certain cortical center 
and stimulated the neighboring area by faradism. It was noted that with 
medium strong currents the extremity corresponding to the extirpated center 
revealed tonus, while clonic cortical spasms were predominant elsewhere. 
Upon stimulation of the exposed medullary layer only tonic convulsions ap- 
peared in the extremity. These researches gave birth to another view than 
the one mentioned above, which considered, not only the cortex of the 
cerebrum but also the infracortical centers, as responsible for the epileptic 
seizure. The intimate functional relationship of the different parts of the 
brain to some extent justifies this view. The irritation first affects the cortex 
— this is indicated primarily by unconsciousness. It is most probable that 
at the same time an irritation of the infracortical centers also takes place. 

But all of these animal experiments have not produced an epileptic attack 
which in all points resembles the seizure in man, and, therefore, what we 
endeavored and hoped to attain (namely, an explanation of the seat and the 
lesion of epilepsy) and especially an insight into the nature of epilepsy has 
as yet eluded us. The entire subject remains entirely hypothetical. For a 
long time the comparison of a " discharge " or, something more concrete, 
the comparison with the process in a Leyden jar (Schroder van der Kolk) 
was employed. The nerve cells, it was believed, in consequence of their dis- 



THEORIES OF EPILEPSY 1027 

turbance in chemism have lost the faculty to eliminate and free energy in a 
normal manner. This leads to an accumulation of energy which is then 
explosively discharged— in the form of an epileptic attack. Hughlings Jack- 
son referred to the " discharging lesion " ; he believed, that in consequence 
of an arterial occlusion, particularly in a certain group of cortical cells, 
especially at the periphery of the region which was deprived of function by 
this arterial occlusion, the faculty mentioned above was lost. This group 
of cells was regarded by him, to a certain extent, as a " hyperphysiologic 
parasite," the epileptic being the host, and when these cells became active 
they not only functioned abnormally themselves but also compelled the other 
areas of the brain to assume the same extravagant activity. Binswanger 
accepts this picture of a discharge. According to this author such a dis- 
charge may become operative in 2 different directions, firstly in the sense of 
a stimulation and secondly in that of inhibition. In every nerve cell besides 
stimulating there are also inhibitive processes. The reaction of a cell to 
irritation does not depend upon the force of the stimulation and upon the 
degree of the irritability of the cell (this also has been invoked to explain 
the epileptic change), but upon other stimuli which reach the cell, i. e., in 
other words, whether the cell is in condition of rest or activity. Binswanger 
regards the nature of the epileptic alteration as a disproportion between 
stimulating and inhibiting processes, in an unequal relation between capacity 
for action and resistance to action in the cortical cells. If the process of 
irritation is increased in the active cell, there is, finally, a discharge of stimu- 
lation; in the quiescent cell the process of inhibition is increased to a dis- 
charge of inhibition. Here it is necessary to discuss whether the expression 
" discharge of inhibition " is happily chosen. It can only be understood if 
we regard the irritative and inhibitive processes in the cell as physiologico- 
chemically equal functions. In general an absence or inhibition of function 
rather raises the thought of a minus energy becoming liberated than of a 
discharge. Therefore, in explanation of the occurrence of the inhibitive symp- 
toms of the epileptic seizure, other possibilities come into question. It is 
possible that the irritative discharge, in certain cell groups, may have an 
inhibitive action in other regions of the brain, this might explain the coin- 
cidence of the 4 mic spasm and the loss of consciousness (Gowers). Or in 
the districts of inhibition there may have previously been a latent stimulation 
so that there may be exhaustion and not inhibition. This latter view is 
favored by Fere. Binswanger admits that it is very difficult to discriminate, 
clinically, between inhibition and exhaustion but he holds that Fere con- 
founds these two series of symptoms. 

Binswanger's theory unquestionably has great didactic value, for it refers 
the complicated symptoms of the seizure to the simple formula of a discharge 
of stimulation and inhibition. If we regard the various symptoms of the 
attack in the light of this theory, the various aurae appear as — frequently 
circumscribed — discharges of stimulation and inhibition of the cortex. On 
account of their localization it was thought that they should be regarded as 
focal symptoms which indicate the region of the original irritation, and cases 
of epilepsy especially after focal diseases of the brain were pointed out in 
which the aura actually corresponded to the functional nature of the cortex 
at the lesion. Hence the doubt expressed by Lasegue as to the value of the 



1028 EPILEPSY 

aura as a genuine epileptic symptom. Authors even went so far as to refer 
all cases of epilepsy to a latent focal disease, so that the conception of genuine 
epilepsy would be entirely repudiated. These conclusions have not, however, 
up to this time been confirmed by pathologico-anatomical findings, so that a 
justifiable doubt of their correctness is certainly reasonable. It is true we 
do not know the causes for the limitations of many aurae and, as I must add, 
of many paroxysmal and post-paroxysmal symptoms. Binswanger in addition 
to his view, cited above, calls attention to the fact that the degree of activity 
of the individual cortical area may be decisive for the effect of an irritation. 
Perhaps individual conditions (varying degrees of stimulation and exhaustion 
of different cortical areas) play a role. The attack begins with a diffuse 
discharge of inhibition in the cortex, which is shown clinically as loss of 
consciousness and a sudden fall (inhibition discharge in the motor region). 
Both symptoms, according to Binswanger, are not dependent upon each 
other, but are coordinate, for on the one hand, there are epileptic conditions 
in which, in spite of profound unconsciousness, the patients do not fall, and 
on the other hand, there are instances in which the discharge of inhibition 
is limited to the motor region, discharges of inhibition in a restricted sense. 
During this period of the seizure the discharge of stimulation is supposed 
to occur only in the infracortical areas. The expression of this infracortical 
stimulation discharge is the tonic convulsion. Only after inhibition has 
ceased in the cortex do stimulative discharges appear in the form of clonic 
contractions. The infracortical component (striking and stepping motions) 
often even now may preponderate. Other authors maintain that the fall 
is the result of loss of consciousness (Oppenheim). This view is logical if 
the point of origin of the tonic spasm is referred to the cortex. 1 

In regard to the other classical symptoms of the epileptic insult (saliva- 
tion, circulatory and respiratory disturbances), according to Binswanger, 
there is no objection to the opinion that they originate from stimulation of 
cortical and infracortical centers. Clinically this is favored by the frequent, 
isolated appearance of these symptoms in abortive attacks. The initial pallor 
deserves special mention for this was regarded formerly as the clinical ex- 
pression and proof of a primary alteration of the vasomotor center in the 
medulla oblongata. Binswanger refers it to an infracortical discharge of 
stimulation equal to the cortical discharge of inhibition; Jackson and Gowers 
believe that it is secondary. Jackson thought that it was directly dependent 
upon cortical discharge (stimulative discharge), for, drawing his conclusions 
from peripheral vascular spasm, he assumed an arterial spasm in the affected 
cortical region. Gowers considers the arterial spasm as reflex, the conse- 
quence of cortical discharge. 

INTERVALS BETWEEN ATTACKS 

Up to this time we have devoted our attention exclusively to the individual 
epileptic attack. A seizure, no matter how much it may correspond to the 

i That the original point of attack of the irritation which gives rise to the spasms 
is only the motor region, is disputed by Unverricht, who upon the basis of some ani- 
mal experiments (which are, however, disputed) also regards the posterior cortical 
areas (occipital lobes) as epileptogenous. 



INTERVALS BETWEEN ATTACKS 1029 

epileptic type, is not yet epilepsy. The characteristic of the affection known 
as epilepsy consists in the repetition of attacks from time to time, either 
spontaneously or as a reaction to some deleterious cause. 

The intervals between the various attacks vary in different epileptics 
according to the severity of the malady. There are epileptics who have an 
attack only every year or every month, others in whom there are but weeks 
or days between the seizures. Sometimes there is a variation in the fre- 
quency of the attack, in the sense that there are periods of infrequent seizures 
which alternate with those in which the attacks are numerous. Especially 
the rudimentary and abortive attacks (Binswanger) are the ones which show 
a cumulative effect. Seizures with long intervening periods mostly correspond 
to the fully developed type. Under the last named circumstances it may 
happen occasionally that a fully developed attack is replaced by a number of 
abortive or rudimentary ones; being to a certain extent divided or broken 
up (Binswanger). As a matter of fact the greatest variation in the course 
is possible and this may be still further modified by the intervention of 
psychical epileptic equivalents. Frequently only slight vertigo, during long 
intervening periods, indicate the continuance of the malady. If an accumu- 
lation of seizures takes place we speak of a series of attacks. These series 
may present a mixture of various types and may be followed by severe con- 
ditions of bodily and psychical exhaustion (epileptic stupor). If, during a 
prolonged series of attacks, there is no return of consciousness in the interval 
between the convulsions, we speak of the status epilepticus. These condi- 
tions, endangering life, are characterized by the addition of some other symp- 
toms, above all by a decided rise in temperature. Often there is an early and 
serious paresis of the muscles of deglutition. Binswanger differentiates be- 
tween a convulsive and a comatose stage of the status epilepticus. The lat- 
ter either corresponds to profound coma or to a condition of unconsciousness 
with low muttering delirium. Death is often due to pulmonary edema or 
acute cardiac insufficiency. 

The time of day at which the attacks appear varies greatly. Fere noted a 
preference for the early morning hours. There are, however, cases in which the 
attacks occur particularly or exclusively at night (nocturnal epilepsy). These 
attacks are especially important because they often escape recognition. The 
cases of enuresis nocturna, arising in late childhood, are particularly sus- 
picious in this respect. In these instances the enuresis may be a symptom of 
the attack. But true enuresis nocturna may introduce epilepsy; this is also 
without question in the case of pavor nocturnus and of somnambulistic con- 
ditions, which, however, belong to the realm of epileptic mental alteration. 
In these instances we refer to larval epilepsy. If, as is not rare, there are 
at first only nocturnal attacks, and then late in the course of the malady 
these appear during the day, we may easily be misled as to the period of 
the onset of the seizures. In regard to the affection in general it may be 
stated that it appears particularly during youth, during the first 2 decades. 
In childhood there is very often a connection with acute infectious diseases, 
a subject which we shall investigate more thoroughly in the description of 
the etiology. 

The views are far asunder in regard to infantile convulsion being of 
epileptic origin. These infantile convulsions (eclampsia) differ from reflex 



1030 EPILEPSY 

epilepsy in that the convulsions are due to a transitory peripheral irritation, 
disappearing after this has ceased; in reflex epilepsy there is a permanent 
peripheral irritation of which, finally, the convulsions may be independent, 
so that after removal of the irritation they do not cease. Binswanger, in 
contrast to Fere, separates eclampsia from epilepsy. These convulsions, which 
result from the most varied kinds of irritation, especially those resulting 
from the intestinal tract, are due (Binswanger) either to an increased tend- 
ency to convulsions, which exists at this period of life, or they they are due 
to a hereditary neuropathic predisposition — and this he regards as an eti- 
ologic point of contact with epilepsy. He differentiates from eclampsia, the 
convulsions of the first days of life, regarding them only as a " signal of 
incapacity to live." That persons who have suffered from infantile convul- 
sions may develop epilepsy as well as other neuroses, such as hysteria, later in 
life is in favor of Binswanger's view of the neuropathic genesis of eclampsia ; 
but the fact of the occurrence of hysteroepilepsy cannot be used in favor 
of a distinctive position of eclampsia. This question is therefore still sub 
judice. 

Puberty shows a special preference for the development of epilepsy, espe- 
cially in the female. In this decade, as may be gathered from statistics, 
there is a preponderance of feminine epileptics which is decreased later on. 
With the third decade there is a decrease in the frequency of the malady, but 
cases have been reported which originated in the fourth decade and even 
later; however, these cases arising late in life occupy a special position 
(epilepsia tarda) in regard to etiology in so far as they are often due to 
cerebral arteriosclerosis and hence usually must be included with symptomatic 
epilepsy. 

PROGNOSIS 

The prognosis of epilepsy is always serious, and quoad restitution em at 
all events dubious. There are some cases in which apparently a cure takes 
place, but this number is small, although formerly it was probably under- 
estimated (about 5$). Various dangers threaten the epileptic, above all, 
progressive mental deterioration and dementia. This is directly proportional 
to the frequency of the attacks, so that the prognosis is the more serious the 
greater the number of attacks. Cumulative attacks of petit mat are quite 
justly feared, especially as they are very apt to be exceedingly resistant to 
bromin treatment. But direct danger to life also threatens the epileptic in 
the form of severe injuries during the attack (I recently saw a fracture at 
the base of the skull the consequence of an attack), severe post-paroxysmal 
conditions of exhaustion with dyspeptic symptoms, serious psychoses with 
marked motor irritation, and above all the status epilepticus. Fatal cases 
during the attack have also been reported, the cause in such cases being 
embolism, softening, hemorrhage, rupture of the heart, asphyxia. Epileptics 
are said to have a shorter period of life than normal persons. Among inter- 
current diseases that cause death, tuberculosis is particularly mentioned. A 
variety that is especially unfavorable, rapidly leading to dementia and death, 
has already been mentioned, Voisin's epilepsia spasmodico-paralytica. I 
should like to call attention to a fact, at this point, in regard to prognosis, 
namely, that in hereditarily predisposed and psychically degenerated indi- 



ETIOLOGY 1031 

viduals (degcncres superieurs, hereditary, psychopathic constitution) epileptic 
or epileptiform attacks occur occasionally as a reaction to some deleterious 
factor. In these cases the prognosis is usually unfavorable although only 
one attack may appear. But psychical degeneration, just as in the acute 
degenerative psychoses, sometimes has rather a favorable prognosis. West- 
phaPs cases probably belong to this category, in which an isolated epileptic 
seizure introduced a very complicated psychical pathologic condition. 

ETIOLOGY 

In turning to the etiology we may refer to much that has already been 
stated, for it was necessary to consider many of these points in the preceding 
subject matter. The immediate causes of the epileptic change are shrouded 
in deepest mystery, provided we do not consider as such the anatomical 
brain lesions which are sometimes present. Among the mediate causes we 
must differentiate between the predisposing and the exciting ones. Bins- 
wanger requires a further division, into preparatory and productive causes 
of the epileptic alteration, and of the individual attack. In practice this 
division cannot be carried out in the majority of cases ; predisposing and 
exciting causes frequently appear to be the same, the exciting cause of the 
epileptic change is often the exciting cause of the first attack; the predis- 
posing causes of the first attack, as a rule, escape our knowledge. On the 
other hand, we often find the exciting cause of the individual attacks in the 
existing epilepsy to be the accidental cause. We have learned already that 
epilepsy may be due to focal disease of the brain. 1 This corresponds to Hit- 
zig's observation, that, after extirpation of motor cortical centers in animals, 
epileptiform convulsions develop. It also explains the not infrequent com- 
bination of epilepsy and infantile cerebral convulsions. This genuine epilepsy, 
developing upon the floor of a focal disease, must not be identified, at least 
not clinically, with Jacksonian epilepsy, which is the expression of a circum- 
scribed cortical irritation. If the focus has an infracortical location, partial 
tonic convulsions (or tremor) may occur, according to Binswanger; these 
are neither genuine epilepsy nor Jacksonian epilepsy. There are cases of 
focal disease in which there are, at first, convulsions of the Jacksonian type, 
these being replaced later by true epileptic seizures. This may give rise to 
an intermediate stage of convulsions which may present features of Jack- 
sonian epilepsy as well as of the ordinary attack (hence the proper doubt of 
the primary nature of all epilepsy in which the attack reveals a decided ad- 
mixture of incomplete convulsions). It may then be said that the irritation, 
which was at first local, has distributed itself gradually and in a diffuse 
manner over the entire cortex of the brain; it is, therefore, usually inde- 
pendent of the focus which gave rise to the original circumscribed irritation, 
for it continues to exist after the (operative) removal of the focus. 

We have already referred to the enormous influence of hereditary factors. 
At the present time this is hardly questioned except by Delasiauve who does 
not recognize it as of importance. In the overwhelming majority of cases 
it is a hereditary, constitutional, neuropathic predisposition upon the founda- 

i Cysticercus epilepsy belongs to this category. 



1032 EPILEPSY 

tion of which epilepsy develops. Any damage in the individual's life is 
sufficient to set the stone rolling, to produce the epileptic alteration and 
simultaneously the first epileptic attack. This heredity depends either upon 
the fact that the germ plasma of one of the two progenitors was deficient 
from the start or, that during life it suffered a damage of some kind which 
deprived it of its complete function. In the former case we note traces of 
the neuropathic predisposition also in the ascendants. Epilepsy is very rarely 
transmitted from the parents to the child; the figures, however, increase to 
a decided extent if we include in the statistics children of epileptics perishing 
from infantile convulsions. Usually there is not a direct, similar heredity, 
but a direct or indirect dissimilar heredity, i. e., in the direct ancestors or 
in a collateral line of the ascendants there are other affections, which also 
develop upon the foundation of a neuropsychopathic predisposition, namely, 
neuroses and psychoses of the most varied kind. An especially intimate rela- 
tion has recently been recognized between migraine and epilepsy, not only 
because migraine is often found in the ascendants of epileptics, but because 
migraine and epilepsy are often associated in the same individual. In the 
second instance it is due to the action of chronic intoxications and chronic 
diseases of the germ plasma. Here I must first mention, and particularly 
emphasize, chronic alcoholism; in the direct ascendants of epileptics it is 
found with extraordinary frequency. Formerly to acute alcoholic intoxication 
was ascribed a deleterious influence upon the germ plasma and etiologic 
importance was attached to procreation under the influence of liquor. Ee- 
cently emphasis is laid upon the point that neurotic and psychopathic indi- 
viduals readily tend toward alcoholic excesses, also that they are often intol- 
erant of alcohol and in the condition of alcoholic excess show a special 
tendency to sexual activity. Among other chronic intoxications there are to 
be mentioned: chronic lead poisoning and morphinism; among chronic dis- 
eases, syphilis, tuberculosis, arthritis deformans, malaria, diseases of the 
blood, gout, and diabetes. Here it must be remembered that the two last 
named affections are also regarded by some authors as the expression of a 
neuropathic predisposition. Among the cases of acquired inferiority of the 
germ plasma those may be included in which the only etiologic factor of a 
neuropathic predisposition consists in the advanced age of the parents, espe- 
cially of the mother. A convergent predisposition, i. e., the coincidence of 
a congenital and acquired deficiency of the germinal products of both parents 
is particularly serious to the offspring. Fere sees great danger in the tend- 
ency of neurotics and psychopathies of all kinds entering into wedlock. In 
so far as epilepsy is concerned, in these and other cases of grave degenerative 
heredity, there is not infrequently a quickening of epilepsy with a congenital 
inhibition of development, forms of feeble mindedness up to complete idiocy, 
or with the varied neuroses or psychopathic conditions. In milder cases there 
are frequently only a number of somatic signs of degeneration (asymmetry 
of the skull, anomalies of the ear, and of the teeth, etc.) in addition to those 
of a psychical sort. Not only is there a hereditary predisposition, but there 
is also an acquired one, developing early in the life of the individual, intra- 
uterine or extra-uterine, which may furnish the predisposition for the devel- 
opment of epilepsy. It is probable that harmful agencies which act upon 
the fetus involve the organism, the more seriously the earlier they act (Bins- 



ETIOLOGY 1033 

wanger) ; then either severe anatomical disturbances in development appear, 
or, in milder cases, a general constitutional weakness of the organism. The 
later this effect occurs, after the individual organs have begun to develop, 
the more the individual organ appears to be affected and especially the nervous 
system; in milder cases only in the sense of a constitutional inferiority. In 
regard to injuries of the fetus, bodily and psychical trauma (fright) in the 
mother are especially important. The latter probably acts as a circulatory 
disturbance. But trauma at birth (forceps, narrow pelvis, asphyxia, fall of 
the child) and also that immediately following birth is not without im- 
portance in the etiology, apart from the fact that by causing an injury to 
the brain, it prepares the ground for the development of epilepsy. In regard 
to the factors which are active in later life there are especially two points 
to consider: first, we will find among them a number which we have already 
recognized as damaging the germinal plasma, and second, a number of these 
agents may produce the epileptic change or they may play the role of the 
causative factor. 

That physiologic processes may assume this part has already been stated; 
the best examples of this kind are the physiologic processes at puberty. Quite 
frequently the first menstruation is the cause of an outbreak of epilepsy; 
if the later menstrual periods are the invariable, accidental causes for the 
appearance of the attack, periodicity appears which gives rise to the menstrual 
or the pre-menstrual type of epilepsy. Here pregnancy and lactation (epilepsy 
of pregnancy and lactation) must be mentioned. During the climacterium 
sometimes an old quiescent epilepsy reappears, just as other neuroses show 
an exacerbation at this period. Coitus is said to be an exciting, etiologic 
factor; it is positive that this is much more frequently the accidental cause 
of an attack when the affection already exists. 

The acute infectious diseases are of great etiologic importance, especially 
in childhood. It is a well known fact that epilepsy may develop after 
measles, scarlatina, pertussis, diphtheria, enteric fever. The effect of the 
infection may vary; either it is the exciting factor, provided the predis- 
position is already present, or, in consequence of toxic influences or other- 
wise as by an accompanying encephalitis (which may be without symptoms), 
it gives rise to the epileptic alteration. The conditions are still more compli- 
cated in the case of the chronic infectious diseases, especially syphilis, and 
here hereditary as well as acquired lues must be taken into account. We 
have already learned to recognize syphilis in the parents as a factor capable 
of damaging the germinal plasma. Here syphilis is not transmitted; only 
the neuropathic predisposition which may give rise to epilepsy is produced. 
Should the virus be transmitted we must again differentiate two conditions : 
first, epilepsy may develop from specific meningitic or circumscribed gum- 
matous processes (symptomatic epilepsy), or it appears as a pure dynamic 
disturbance probably due to the action of toxins. The same differentiation 
is true of cases of epilepsy after acquired syphilis. " Functional " syphilitic 
epilepsy, according to Fournier, is said to appear especially during the sec- 
ondary stage and to disappear with this stage (Fournier's para-syphilitic 
epilepsy). This disappearance is denied by other authors. Occasionally 
syphilis may act as the exciting factor, provided the predisposition is present. 
It must always be remembered that epileptiform convulsions may for a long 



1034 EPILEPSY 

time be the isolated prodromes of a metasyphilitic disease (dementia para- 
lytica). Fere mentions the frequency of the simultaneous occurrence of tabes 
with epilepsy; this is probably a combination of tabes with arteriosclerotic 
epilepsy (analogous to the combination of tabes with cerebral thrombosis, 
etc.) or even with syphilitic epilepsy. Among the other chronic infectious 
diseases malaria must be mentioned. Other chronic affections that have been 
associated with epilepsy are : rickets, scrophulosis, anemia, chlorosis, leukemia, 
gout, arthritis deformans, and diabetes. In regard to rachitis and scrophu- 
losis, these diseases, as emphasized by Binswanger, are mostly the signs of 
a general constitutional debility. As this is frequently associated with a 
neuropathic predisposition, in so far as the latter is merely a partial con- 
dition of the former, epilepsy and the previously mentioned affections should 
not be placed in etiologic dependence, but are rather to be regarded as con- 
ditions parallel to one another. Nevertheless, the severe nutritive disturb- 
ances in rickets and scrophulosis may be concerned to a considerable extent 
in the development of epilepsy. The etiologic association of epilepsy with 
gout and diabetes insisted upon by French authors is rejected by Binswanger. 
That these two affections may be regarded as stigmata of a neuropathic pre- 
disposition has already been stated. When they occur early and are associ- 
ated with epilepsy we must at the same time consider a parallelism in the 
symptoms. As a rule, however, the convulsions appearing at the onset of 
an attack of gout or diabetes must be included with the group of toxic con- 
vulsions and these, according to Binswanger, have nothing in common with 
true epilepsy. 

The theory of the etiologic connection of epilepsy with profound disorders 
of metabolism and the phenomena of auto-intoxication compels me to incor- 
porate a discussion of this genetic association which attempts to refer epilepsy 
in general to processes of auto-intoxication. Zeni is the latest author to 
draw conclusions of this kind from his theory of the auto-cytotoxins and anti- 
auto-cytotoxins. The foundation of all of these views' is the previously 
mentioned investigations of metabolism. In regard to the toxicity of the 
urine, Fere and Voisin reached contradictory results, one finding a hyper- 
toxicity and the other a hypotoxicity of the urine after the attack. I should 
like to point out that the toxicity of a urine, according to the latest researches 
of Schumacher, depends largely upon its concentration, and its specific grav- 
ity. Oliviero and Voisin isolated from the urine of an epileptic an exceed- 
ingly toxic product having an odor of ammonia and nutmeg, which, when 
injected into the circulation of animals, produced violent convulsions. 
Krainski, to whom we owe comprehensive studies of metabolism in epileptics, 
found that defibrinated blood, taken during a seizure, when injected into 
animals produced convulsions, but that taken during the period free from 
attacks did not have this result. The same conditions were found by Cololian. 
Krainski demonstrated a constant relation between the excretion of uric 
acid and the epileptic seizure, in the sense that a change in the excretion of 
uric acid precedes the attacks; this is the more marked the more severe 
the succeeding attack. Haig considered the retained uric acid as the toxic 
agent which produced the convulsion. Krainski holds ammonium carbamate, 
which originates from uric acid, responsible; this substance being normally 
used for the synthetic formation of uric acid. As a matter of fact, according 






ETIOLOGY 1035 

to the researches of Krainski, disproportionate quantities of carbamic acid, 
as well as of ammonia, were found in the blood of epileptics, the quantities 
being the greater the severer the attacks. On the other hand ammonium 
carbamate when injected into animals produced serious nervous symptoms 
(somnolence, epilepsy, tetanus). It may be stated, in regard to these in- 
vestigations of metabolism, that the proof has not yet been furnished that 
they actually provoke the epileptic seizure and that we are not concerned 
merely with the resulting phenomena of the epileptic charge and discharge. 
Binswanger believes that this is due directly to a disturbed chemism of the 
nerve cells, but the effect of the motor discharge must also be considered. 
But even in the first instance, that altered metabolism is the direct " agent 
'provocateur" of the epileptic discharge, the dependence upon periodic nervous 
disturbance (change in the activity of secretion of the kidneys) must be 
considered. But even this would not relegate the conception of the epileptic 
alteration out of existence; besides these disturbances of metabolism are 
present only in some epileptics (Hebold-Bratz, Binswanger). Binswanger 
holds that it is not impossible to separate this group pathogenetically as tox- 
emic epilepsy from the other varieties. In this view he approaches the opinion 
of Fere to which in the main he is seriously opposed, namely, that what we 
regard to-day clinically as genuine epilepsy is still further divisible eti- 
ologically. 

In addition to the chronic diseases mentioned above a number of special 
affections of the organs of the body must be included which are in etiologic 
connection with epilepsy. Among these are: the lungs, the heart, the liver, 
the pleura, the ear, the genitalia, the stomach and the intestines. Here 
reflex epilepsy (which will be considered later on) comes into play. Pro- 
vided there is not an accidental coincidence of these maladies with epilepsy 
they are probably only the exciting factors, the predisposition being already 
present. In patients with cardiac disease, apart from reflex causes, the effect 
of circulatory disturbances must be considered. Whether all epileptiform 
conditions (Stokes- Adams disease) are to be included with epilepsy appears 
very doubtful. In gastro-intestinal diseases toxic influences are present. 
Early and prolonged masturbation is probably only a predisposing cause and 
besides must be looked upon as a neuropathic stigma. Among the chronic 
intoxications there are to be mentioned: alcoholism and chronic lead poison- 
ing. Both may be considered not only indirect but direct factors giving rise 
to epilepsy by damaging the germinal plasma. Alcoholic epilepsy and lead 
epilepsy are usually considered special forms. Alcohol may occasionally act 
as a predisposing cause. Excessive use of tobacco is sometimes the cause of 
an attack, but it has been stated that chronic nicotin poisoning may directly 
institute a seizure. Among other poisons there are to be mentioned : carbonic 
oxid, chloroform, ether, cocain, antipyrin, camphor. Their etiologic relation 
to epilepsy is, however, very doubtful. 

Trauma plays a much more important role in the etiology, especially that 
form which affects the head; but other varieties than those associated with 
severe bodily shock may result in epilepsy. When trauma to the head pro- 
duces grave anatomical lesions, such as hemorrhage, meningeal hemorrhage, 
depression of bone, the picture of ordinary epilepsy may be clinically present. 
Under these circumstances it occasionally happens that a constant localized 



1036 EPILEPSY 

aura or a post-paroxysmal sign of absence of function indicates the lesion 
and its seat. As a rule there are uniform, partial convulsions. In other 
cases of traumatic epilepsy there is no sign, at least macroscopically, of an 
anatomical lesion (whether or not there are minute miliary hemorrhages in 
the cortical substance cannot be answered). Again the fact must be stated 
that trauma may only be the predisposing cause. Among the cases due to 
trauma of the head the unique case of Breitung's must be included: epilepsy 
developing after the application of douches to the head. Psychical trauma 
must be included with bodily trauma; fright, and mental overexertion, as 
trauma with protracted action. These are probably not exciting but pre- 
disposing causes. That with bodily trauma, psychical trauma (fright), 
which accompanies it, may also be in etiologic relation, must be considered. 

Reflex epilepsy belongs to the category of traumatic epilepsy. In regard 
to this variety we must mention the animal experiments by which epileptic 
convulsions were produced by various procedures acting upon the peripheral 
nervous system. These experiments are intimatety connected with the name 
of Brown-Sequard who produced epileptic convulsions in guinea pigs after 
dividing the sciatic nerve; similar convulsions were produced by injuries to 
the spinal cord and later Westphal succeeded in causing them by blows upon 
the skull. These convulsions have certain characteristics, firstly, their ap- 
pearance is associated with the irritation of definite zones, the so-called 
epileptogenous zones, which develop in the animal that is operated upon 
within the first few days and which may be observed externally (falling out 
of the hair, anesthesia). If a zone of this kind is irritated there arise, during 
the first period, reflex movements in the animal in the form of scratching 
and resistance in the limbs of the same side. Later these reflex actions are 
increased to general convulsions, of which it is questionable, whether they 
are associated with loss of consciousness. The convulsions in man, which 
are regarded as reflex epilepsy in the restricted sense, present certain analogies 
to those produced in the animal. A lesion of a peripheral nerve trunk is 
common to both (after injuries to, and diseases of, the spinal cord in man 
convulsions have been noted which may be included under the conception 
of reflex epilepsy). Often in man there is the formation of traumatic neuro- 
mata, embedding of nerve trunks in cicatricial tissue, etc. (perhaps also 
cicatrices in the skull). An epileptogenous zone is also present in these 
cases, for pressure upon the cicatrix often produces an attack. These attacks 
are still further characterized by the fact that after a sensory aura, which 
involves the injured part, an isolated tonic tension appears during which 
consciousness remains normal; later this tonic contraction also involves the 
other extremity upon the same side and finally the opposite extremities; loss 
of consciousness and the other criteria of the genuine epileptic attack are 
then added. Binswanger does not discuss the question whether or not in 
these convulsions there is a step-like progression from the level of the centers 
in the spinal cord to the cumulate centers in the medulla and from these 
to the cortex of the brain, or whether the old medulla theory is to be invoked 
to explain this group of epileptic attacks, according to which loss of con- 
sciousness is a consequence of irritation of the vasomotor center in the 
medulla oblongata and that the cortical anemia consequent upon this occurs 
simultaneously with the stimulation of the motor medullary centers. The 



DIAGNOSIS 1037 

conception of reflex epilepsy has been extended te a series of cases which 
probably have in common only the existence of a peripheral irritation. This 
irritation, in the cases in question, probably has but a predisposing influence. 
In addition to the affections enumerated above I must add: dental caries, 
anomalies of refraction ( ? ) , polypi of the ear and larynx, adenoid vegeta- 
tions, phimosis, intestinal parasites. The last named are said to give rise 
to epilepsy by the production of a toxin. The formation of spontaneous 
epileptogenous zones in man is exceedingly doubtful. 

There are many accidental causes for the appearance of an attack when 
the affection exists. In great part they have been already mentioned; bodily 
as well as psychical overexertion is an important factor. In cases of nocturnal 
epilepsy physiologic sleep is regarded as an accidental cause. 

PATHOLOGY 

I may be very brief in regard to the pathological anatomy. All lesions 
in focal diseases or severe cortical changes with simultaneous idiocy naturally 
need not be considered. Sclerosis of the hippocampus major, for a time, was 
considered of great importance; it was shown, however, that this sclerosis 
is not present constantly in the brain of epileptics and, again, that it is 
present in persons who have never suffered from epilepsy. Oppenheim is 
of the opinion that it is an unimportant stigma of degeneration. Eecently 
attention has been directed to minute lesion in the cortex of the brain of 
epileptics (destruction of nerve cells, proliferation of glia fibers in the cerebral 
cortex, Chaslin, Alzheimer, and others). It is very doubtful whether these 
histologic changes can be regarded as the cause of the epileptic attack and 
that they are, therefore, merely sequels of the existence of the affection; just 
as this is self evident of many pathologic lesions found in the brain of persons 
perishing during the attack or in the status epileptic us. The lesion discov- 
ered by Schroder van der Kolk also belongs to this category. Binswanger 
regards it as a step backward in pathophysiologic knowledge if all disturb- 
ances of nerve function, even the exceedingly minute expression of activity 
of the central nervous system, is constantly and exclusively referred to gross 
lesions of the nervous substance. He also maintains that at the present time 
a causal pathological lesion has not been demonstrated. Liepmann is of the 
opinion that there is no pathologico-anatomical lesion in epilepsy, a view that 
every unprejudiced investigator must accept, in spite of the many lesions 
that have been reported in the brains of epileptics. 

DIAGNOSIS 

The diagnosis, in the greatest majority of cases, is very easy, provided we 
adhere to the characteristics of the attack described previously and consider 
that epilepsy is a chronic disease. 

According to Binswanger, infantile convulsions may be differentiated 
from epilepsy by a preliminary stage of motor unrest or of somnolence associ- 
ated at first with isolated contractions in the muscles of the face, the clonic 
stage, a tonic stage appearing later. In addition eclamptic convulsions show 
a tendency to accumulative appearance. Ziehen regards the preponderance 



1038 EPILEPSY 

of the tonic spasm as characteristic. There is usually the demonstration of 
some etiologic factor (dentition, gastro-intestinal affection, fever) which 
serves as a differential criterium. 

When there are convulsions resembling epilepsy, especially if they occur 
in later life, the possibility of another underlying affection must be consid- 
ered. Oppenheim calls attention to cases in which a brain tumor revealed 
its presence by the symptoms of epilepsy which were present for years. In 
such instances the brain tumor (like any other focal disease) became the 
exciting cause of the epileptic attacks. In other instances it is the intra- 
cranial pressure accompanying the brain tumor which is the cause of the 
epileptic attacks. Here the epileptiform attacks accompanying cerebral 
syphilis must be included. The toxemic convulsions in gout and diabetes 
must be remembered. Dementia paralytica not rarely runs its course with 
epileptiform spasms; usually these are only incomplete or of the Jacksonian 
type; but we have already mentioned some cases in which the attacks closely 
resembled genuine epilepsy. These may precede the appearance of the 
paralysis for years. Epileptiform attacks also occur in multiple sclerosis. 
These references are sufficient to indicate the importance of making a careful 
examination of every patient with epileptic attacks. It may always be re- 
garded an incomplete examination if an ophthalmoscopic investigation of these 
patients is neglected. 

Jacksonian Epilepsy. — Nor is the recognition of the Jacksonian form 
difficult. Here the convulsions, clonic contractions, occur in a circumscribed 
muscular region and are gradually distributed, in analogy with the anatomical 
situation of the motor cortical centers. There is either entire absence of 
loss of consciousness or this occurs only after the convulsions have attacked 
the other half of the body. Difficulties can only arise if the convulsions are 
unilateral or if a motor aura precedes them; and even here the early loss of 
consciousness will indicate the correct diagnosis. There are intermediate 
forms which correspond neither to the epileptic attack nor to the Jacksonian 
variety but present a complicated picture. These cases are always suspicious 
of an organic foundation and here we find the post-paroxysmal circumscribed 
symptoms of absence of function which were detailed in the description of 
the symtomatology. 

The apoplectiform attacks of epileptics may cause confusion with syncopal 
conditions. Here we must accurately note the isolated spasm of the respir- 
atory muscles, the cyanosis which results and which is so common in this 
condition. The person in syncope is pale, there is pronounced cardiac weak- 
ness, the pulse is feeble and rapid; in the epileptic attack the pulse is full 
and tense. We must never omit an inquiry as to the prodromes of the loss 
of consciousness. In epilepsy there is often the characteristic aura; in syn- 
cope there is a sensation of weakness and nausea. Quite characteristic and 
of some value in differential diagnosis is the common appearance of an out- 
break of cold sweat prior to an attack of syncope. The sweating at the 
beginning of an epileptic attack is usually associated with intense heat. In 
doubtful cases the involuntary evacuation of urine favors epilepsy. Never- 
theless, Oppenheim hesitated in a case in making a diagnosis of epilepsy: A 
woman with mild vasomotor disturbance suffered from attacks of uncon- 
sciousness prior to her menses, accompanied with involuntary evacuation of 



DIAGNOSIS 1039 

urine and feces. He believed cerebral anemia, due to vasomotor disturbance, 
to be the cause and this opinion seemed to be correct for no other attacks 
took place. At this point we must mention the so-called " laughter-stroke " 
of Oppenheim (Lachschlag) ; a condition in which there is a brief loss of 
consciousness after excessive laughter. We must bear in mind that excessive 
laughing may occasionally be a predisposing cause of an epileptic attack. 
Meniere's disease, included by Fere with epilepsy, may readily be differen- 
tiated from an epileptic attack on account of its very characteristic symptom- 
complex, even when associated with unconsciousness. There is often difficulty 
in the differential diagnosis between arteriosclerotic epilepsy and of the 
vasomotor climacteric neurosis; such difficulties arise when arteriosclerotic 
epilepsy occurs as an attack of vertigo combined with slight mental dullness 
and introduced by sensations of ascending flashes of heat. The vasomotor 
attacks of climacteric neurosis are not associated with loss of consciousness; 
an examination of the mental condition is very important in these cases for 
in climacteric neuroses intelligence is normal, while in arteriosclerotic epilepsy 
there are commonly the signs of beginning arteriosclerotic mental feebleness. 
Binswanger states that in the clinical picture of neurasthenia there are 
psychical attacks of inhibition with symptoms of motor irritation and those 
of absence of function ; these are liable to cause confusion with abortive 
epileptic seizures. In one case there were vertigo, difficulty in speech, spasm 
of the jaws, dimness of the field of vision, pallor of the face, but without 
loss of consciousness. The original diagnosis of epilepsy proved to be in- 
correct. 

The differentiation of epileptic and hysterical attacks is of the greatest 
importance; this, in very many instances, is by no means easy. Even the 
prodromes (increased irritability) and the aura of the hysterical attack 
(globus) occasionally present great similarity to epilepsy. Fere believes the 
diagnosis of hysteria to be assured when the globus rises from the ovarian 
region; the fall, in the hysterical attack, is more gradual so that severe 
injuries do not result, so that the presence of an injury in a doubtful case 
is in favor of epilepsy. Many hysterical attacks present a so-called epileptoid 
phase, a phase of tonic tension of all of the muscles of the entire body, in 
which the respiratory muscles may be implicated and in consequence cyanosis 
may result. If this epileptoid phase is succeeded by cloivnism, arc de cercle. 
attitudes passionelles, dream-like delirium, or if any of these phenomena 
appear alone, the character of the attack becomes clear. Even if the con- 
vulsions of this clonic stage are not excessive they nevertheless frequently 
bear the character of voluntary movements. Ehythmically these patients 
protrude the tongue, throw the head backward, ball the hand into a fist, 
throw their arms about, etc. The physician must be cautious not to regard 
the automatic coordinated movements which sometimes appear in atypical 
or rudimentary epileptic attacks as hysterical. These clonic convulsions, in 
hysteria, are often associated with crying, scolding, etc. A rhythmical pro- 
trusion of the abdomen (coitus movement) is very characteristic. According 
to Fere this may occur during the interval of a series of epileptic attacks. 
The greatest difficulty arises in those cases in which the epileptoid phase 
presents all the characters of the hysterical attack: all the symptoms which 
have been described as appearing in the epileptic seizure may also be found 
67 



1040 EPILEPSY 

in hysteria : complete loss of consciousness, rigidity of the pupils, biting the 
tongue, involuntary evacuation of urine. On the other hand pupillary rigidity 
may be absent in epilepsy. It may, however, be stated in general that 
pupillary rigidity, biting the tongue and involuntary evacuation of urine are 
exceedingly rare in hysteria and in a doubtful case are in favor of epilepsy. 
On the other hand it is not rare for hysterical patients to bite and gnaw 
their lips during an attack. Even when the tongue is bitten, it may be noted 
that this is due less to a decided bite upon the margin of the tongue than 
to surface injury from rubbing and friction of the tongue against the teeth. 
In doubtful cases an attempt should be made to produce an attack by pressure 
upon a hysterogenic zone or to abort a paroxysm by this means. Among the 
post-paroxysmal signs small hemorrhages behind the ear and in the con- 
junctiva are in favor of epilepsy. The condition of the phosphates in the 
urine, described previously, may be utilized in the differential diagnosis. 
Attacks of vertigo with momentary loss of consciousness, which resemble 
epileptic petit mal, appear also in hysteria, according to Fere. There are 
also attacks which present distinct features of the epileptic as well as of the 
hysterical convulsion; these are known as Jiystero epilepsy. Fere points out 
that commonly one of the components, either the epileptic or the hysteric, 
is prominent in the attack. Those cases are also designated as hysteroepilepsy 
in which, in addition to undoubted epileptic seizures, conclusive hysterical 
paroxysms appear; in regard to the first mentioned category as well as to 
these cases it may be taken as a rule that they are subjects with the signs 
of severe neuropathic predisposition. The occurrence of hysteroepileptic 
attacks in the first mentioned sense, is, however, still a disputed question. 

Malingering. — Finally the occurrence of simulation must be considered. 
Of the most recent instances of this kind I must mention the case reported 
by Leubuscher to the Society for Neurology and Psychiatry. The epileptic 
seizures were simulated by this individual with great accuracy even to biting 
the tongue. Of especial note in this case was the duration of the malingering. 
In a suspicious case pallor of the face, cyanosis and the reaction of the pupils 
must be observed. Voisin lays special stress upon a study of the pulse curve; 
Fere upon the pneumographic curve. 

If we are in a position to observe an attack the diagnosis is much easier. 
But if it is necessary to content ourselves with the description of an eye 
witness all the details should be insisted upon and the intelligence of the 
witness must be taken into consideration. We should search for the remains 
of an attack: cicatrices upon the tongue, other injuries, the small hemor- 
rhages appearing after an attack and all the other phenomena of a nocturnal 
attack. 

TREATMENT 

The therapy of epilepsy must follow causal factors. The treatment of a 
coexisting affection that is in etiologic relation to epilepsy is self evident. 
Such maladies were enumerated in the description of the etiology; I shall 
only mention diseases of the genitalia and of the ear, carious teeth, adenoid 
vegetations, phimosis. Unfortunately we cannot always succeed in relieving 
the epileptic alteration by the removal of these causes; what we can avert 
are the exciting factors. A permanent cure is, therefore, not to be expected. 



TREATMENT 1041 

The conditions are more favorable in reflex epilepsy due to peripheral 
cicatrices. Here operative removal of the source of irritation, excision of 
the cicatrix, provided it is done early enough before an ineradicable diffuse 
condition of irritation appears in the cortex of the brain, cause the attacks 
to disappear completely. It is, therefore, advisable, in these instances, not 
to lose valuable time with useless internal treatment. 

In regard to traumatic epilepsy after injuries to the skull (in so far as 
this is not included within the meaning of reflex epilepsy) there is only an 
indication for operation when there are undoubted partial convulsions of 
the Jacksonian type which point to a cortical lesion, and in those cases show- 
ing the picture of ordinary epilepsy, when there is depression of bone or 
the previously mentioned symptoms (characteristic aura, post-paroxysmal 
signs of absence of function) which render the presence of an anatomical 
lesion likely. The chances are the more unfavorable the more remote the 
trauma and in consequence the more the epileptic change has had an oppor- 
tunity of imbedding itself. Surgery has shown but very few enticing results 
in genuine epilepsy, no matter whether the operation consisted in tying the 
carotid artery, resection of the sympathetic, trephining or ventricular drain- 
age. These cases are, therefore, to be regarded as a surgical noli me tangere. 
Here must also be included symptomatic epilepsy after diseases with cortical 
foci, when the pure picture of Jacksonian epilepsy is not present. 

The therapy of (genuine) epilepsy consists in a careful regulation of diet 
and of the entire manner of living. Alcohol is a dangerous poison for every 
epileptic. Coffee, tea, bouillon (on account of the large amount of creatinin), 
strong spices and tobacco are prohibited; the food should consist largely of 
vegetables and much milk (in the country a milk diet may be employed). 
With nocturnal attacks, light food is to be given at night. Eecently complete 
withdrawal of table salt, following the advice of Eichet and Toulouse, has 
been tried; these authors pointed out that with the withdrawal of table salt 
much smaller doses of the bromids are sufficient. With this treatment it is 
necessary to see that there is no salt in the bread; if there is great repug- 
nance on the part of the patient the food may be salted with small quantities 
of sodium bromid. The patient must be protected from all corporeal and 
psychical injuries. For this purpose it is necessary that the patient have a 
suitable occupation which neither causes physical (bodily exertion, heavy 
work) nor mental (great responsibilities) overexertion. Masturbation and 
sexual excesses are to be prohibited. In mild cases, in which there are long 
intervals between the attacks, an attempt may be made to treat the patient 
by diet and the aid of general hygienic means, perhaps also by cautious 
hydrotherapy. As a rule, however, in order to obtain good results it is neces- 
sary to combine the dietetic treatment with the administration of the bromids. 
This is still the sovereign remedy in the treatment of epilepsy. Potassium 
bromid, sodium bromid or ammonium bromid, or a combination of the three 
salts may be administered in doses of from 4—8, exceptionally from 10-12 
grams daily. In children and in young persons the dose of bromid must be 
calculated according to the weight of the patient (0.1 gram of potassium 
bromid to the kilogram). Erlenmeyer's bromid water and Sandow's effer- 
vescent bromid salt enjoy a great reputation. More recent substitutes are 
bromalin (in doses of 1-2 grams several times daily in powder) and 10$ 



1042 EPILEPSY 

bromipin (a tablespoonful contains 2 grams of potassium bromid). The 
latter is a solution of the bromid salt in sesame oil and has at the same time 
a nutrient value, besides bromid acne is said to be less likely to arise. For 
the latter condition there may be employed prophylactically, warm baths, 
soaping the entire body with soft soap, regulation of the bowels, etc. A mild 
grade of bromid acne does not indicate the immediate withdrawal of the 
bromid; an attempt may be made to combat the condition by the adminis- 
tration of small doses of Fowler's solution. It is sometimes necessary to 
continue the bromid treatment for years and all that is required is to guard 
against the appearance of severe symptoms of intoxication (sleepiness, cardiac 
disturbance, disappearance of the corneal reflex). According to Flechsig the 
good effect of the bromid is increased if a six weeks' treatment with opium 
precedes the administration of the bromid (extract of Opium gradually in- 
creased until 0.9 are taken daily). The opium-bromid treatment may be 
combined with that suggested by Voisin (cool baths; beginning with medium 
temperatures and while shortening the duration of the bath decreasing the 
temperature of the water about 1-|° F. daily). In senile (arteriosclerotic) 
epilepsy it is well to combine the bromid with a cardiac tonic (digitalis, 
strophantus, adonis), for example in the form of Bechterew's prescription 
(inf. adonid. venal. 2.0 :180.0, codeini phosphor. 0.1, natr. bromid 10.0. 
M. D. S. : tablespoonful 3 times daily). Among the substitutes for the 
bromids there may be mentioned: belladonna (extr. belladonnas, fol. bella- 
donnas pulv. aa 1.0, succi liquor, q. s., ut nant pilul. No. 100. 2-6 pills 
daily), atropin (0.0005 3-5 times daily in pill) and borax which has been 
recently recommended (3-5 grams of natr. biborac. in solution). Radix 
artemisiae and zinc oxid are obsolete remedies. During the attack we may 
limit ourselves to the protection of the patient so that he does not injure 
himself; it is advisable to place a cork between the teeth, if possible, to pre- 
vent the tongue from being bitten. To abort the attack the following means 
have been tried: constriction of the member from which the aura arises, 
administration of a tablespoonful of common salt or the inhalation of a few 
drops of amyl nitrite. In the status epilepticus chloral hydrate (2 grams), 
or amyl hydrate (7 grams), by the rectum are advisable. For example 2 
grams of chloral hydrate are given by the rectum and after each succeeding 
attack 1 gram. 

In the preceding description I have given a presentation found in the 
majority of text-books : that genuine epilepsy is a well founded uniform 
disease with definite clinical symptoms to be strictly separated from other 
symptomatic convulsions (uremia, convulsions due to cerebral pressure). 
From the standpoint of clinical description such a conception certainly has 
decided advantages. I cannot close my discussion without again referring 
to the uncertainty still prevalent as to whether what has been mentioned -is 
actually in accordance with the facts and must, therefore, again call attention 
to Fere's opinion. Fere states the following : 1 " A separation of epileptoid 
affections from the great trunk of the neuroses always makes itself felt when 
a new circumstance is recognized which is capable of producing the symptom- 
picture of epilepsy. The realm of so-called idiopathic epilepsy is restricted 

i Ebers's translation. 



TKEATMENT 1043 

from day to day while that of symptomatic epilepsy is constantly being en- 
larged. At the present time we can no longer employ the designation idio- 
pathic epilepsy as synonymous with epilepsy without cause, but only for 
epilepsy with an unknown cause. Xo uniform, definite, definable pathologic 
change can be stated as a cause for epilepsy, but, on the contrary, we must 
regard the view as well founded that the most variable manifestations of 
epilepsy may be referred to very different functional damages or alterations. 
Therefore epilepsy can no longer be considered a uniform pathologic picture 
but only as a group of symptoms." 

If we may apply the valuable animal experiments of Nothnagel, of Hitzig, 
of Binswanger, of Ziehen, and others, to the conditions in man — and this 
we may certainly do — the material for the mysterious mechanism of the 
epileptic attack is present in every brain, and also in the healthy brain and 
need not be produced by a morbid process. How does it happen that in 
one person fortunately this material is not utilized and in another this 
baneful force is set into action ? There are only two possibilities : either 
irritation of an unusual kind or of unusual strength must act upon the brain, 
or the wave of irritation for the occurrence of the mechanism must be situated 
particularly low down. Both possibilities appear to be present in the path- 
ology of epilepsy (I am now employing this expression in its wide sense for 
all epileptiform attacks). On the one hand we note the appearance of 
epileptic attacks, if the view is justified, when an abnormally powerful stimu- 
lation irritates the brain. I need only refer to the appearance of such attacks 
in the different acute intoxications. Perhaps toxemic epilepsy belongs to 
this category, although Binswanger with his conservatism declines to regard 
it as genuine epilepsy. In this condition the explanation why toxic products 
are formed in the organism still remains unanswered provided we do not 
care to assume that the normal organism occasionally develops toxic products 
and, as I must add, eliminates them without further damage and symptoms. 
It would follow that to this group uremic attacks also belong in which 
epileptiform convulsions appear, which in no wise can be differentiated clin- 
ically from genuine epilepsy. Uremic convulsions would then be nothing 
else than acute epilepsy, in Fere's sense. In regard to the second possibility 
we note the presence of epileptic attacks in cases in which irritations are 
present which would not affect the normal organism. I need only refer to 
the processes of puberty, the action of fright, and the like. Here a deep 
position of the wave of irritation must be thought of, and an increased 
faculty for convulsions, a spasmophilia, which is perhaps nothing else than 
the stigma of a congenitally deficient nervous system or one that has become 
so during the life of the individual. The eclamptic convulsions of infancy 
would belong to this group, in which two factors appear to play a role, first 
the increased tendency to convulsions in early life, therefore, a physiologic 
deep position of the wave of irritation, and secondly the neuropathic pre- 
disposition, therefore, a pathologic deficiency of the wave value. Whether 
the epileptic attacks recur, whether they show a cumulative effect or whether 
they do not present themselves again depends upon the greater or lesser 
facility with which this irritation becomes liberated in the central nervous 
system. An increase of this faculty will cause many attacks and even the 
most insignificant stimulus, as it were, a microscopic irritation will bring 



1044 EPILEPSY 

about a seizure. This is probably a similar condition to that which arises 
in the patient with a psychopathic predisposition, his ideas constantly evoking 
constrained conceptions. Whether for this group of " spasmophiles " it is 
necessary to assume a special epileptic change is questionable. Both possi- 
bilities would naturally be in correlation to each other in the manner that 
the deeper the wave of irritation is situated the slighter the stimulation 
necessary to produce the epileptic attack. 

All diseases of the brain might show their effects in two different direc- 
tions, first in that they give rise to abnormal irritation, and this is especially 
true of focal affections, and second that they involve the entire nervous 
system in the sense of decreasing its power and in this manner bring about 
the increased tendency to convulsions; focal diseases perhaps especially in 
relation to their surrounding areas. In favor of the latter possibility, in 
regard to focal diseases, is the clinical fact that often in old cases of en- 
cephalitis Jacksonian seizures appear under the influence of a peripheral 
irritation and after removal of the source of irritation these attacks dis- 
appear. Jacksonian epilepsy differs from general epilepsy only by the fact 
that the irritation is local or by the decrease of resistance being localized. 
The intimate connection of both forms of epilepsy is favored by the close 
association of their clinical phenomena. But also the epileptiform attacks 
in the most varied diseases of the brain, in dementia paralytica, in multiple 
sclerosis, regarded from the same point of view, would have to be included 
with epilepsy. There would be no difference between genuine and sympto- 
matic epilepsy, sensu strictiori there would be no such disease as epilepsy but 
only an epileptic attack. 



HYSTERIA 
By Th. ZIEHEN, Berlin 

Among the organic diseases of the nervous system a precise differentiation 
of the individual forms gives rise to the greatest difficulties. These are 
further increased as we approach the functional neuroses, for here path- 
ological anatomy does not aid us. Therefore it is not surprising that for 
many functional neuroses there is as yet no accurate limitation and definition. 
This is particularly true of the special functional neurosis, hysteria, that we 
are about to consider. As pathological anatomy does not aid us and, as we 
shall soon see, no uniform and exclusive cause for hysteria can be demon- 
strated, we are compelled to search for a comprehensive pathophysiologic 
stand-point under which all, or at most the majority, of functional disturb- 
ances can be grouped so as not to limit ourselves to a simple detailed account 
of its main symptoms and their relations. Such pathophysiologic definitions, 
in functional nervous diseases, must replace the pathologico-anatomical founda- 
tion. In fact such a uniform pathophysiologic stand-point may be recognized 
in the case of hysteria. We may say : Hysteria is characterized by an abnor- 
mal accentuation of emotional activity. These accentuated emotional con- 
ceptions are, in themselves, primarily normal ; but while in the healthy indi- 
vidual such increased emotional conceptions are restricted to a relatively 
limited field in consciousness, sensation, thought and action, in hysteria these 
bounds are exceeded: an accentuated conception of fear of paralysis of the 
arm actually produces the paralysis, the accentuated emotional impression 
severs the play of motive and is discharged in impulsive actions or in spas- 
modically exaggerated expressions of movements. Favored by this increased 
activity it is transformed into hallucinations and illusions or it perverts the 
normal recollection into illusions of memory : for the time being it dominates 
the disposition and the emotions to an extent such as never exists in the 
normal person. Xor is the reverse lacking: non-accentuated emotional con- 
ceptions, in hysteria, are of abnormally feeble effect; thus the absentminded- 
ness and many other symptoms of hysteria, which we shall encounter later 
on, may be explained. An attempt has often been made to characterize 
hysteria by the " psycho genous " nature of its symptoms. And it may be 
readily seen why this was done. The corporeal symptoms of hysteria are in 
fact psychogenous, but this designation is not sufficiently restricted; the bodily 
symptoms of melancholia are also psychogenous. We must state specifically: 
the corporeal symptoms of hysteria depend upon the abnormal increase of 
emotional activity. But even in another respect the definition of hysteria as 
a disease consisting of psychogenous symptoms is inadequate: it is too par- 

1045 



1046 HYSTERIA 

tial, favoring the bodily symptoms while ignoring the psychic ones. Besides 
the psychogenous character of the symptoms, the increased power of sug- 
gestion has been pointed out as a main factor in hysteria. If we understand 
by this increased power of suggestion not only a heightened susceptibility for 
foreign suggestions but above all an increased effectiveness of autosuggestion 
this view approximates very closely that of our own. It is simply less accu- 
rate in so far as, firstly it extends the conception of suggestion unduly, and 
secondly it fails to emphasize the emotional excess of the abnormally active 
impressions. The increased power of suggestion is merely one of the most 
important auxiliary phenomena of the abnormal increase of activity in the 
emotional impressions according to which, in our opinion, the clinical picture 
of hysteria is dominated. 

However, our own view requires a very important amplification. The ex- 
aggerated emotional impressions, of which increased activity is in general 
characteristic of hysteria, need not by any means be actual ones, also the so- 
called latent memory pictures, or, on the contrary, particularly the latent 
memory pictures, in so far as they are exaggerated emotional ones, present 
a pathologic increased activity. To make this important fact clear I shall 
quote the observation of a psychologist. He meets a gentleman every morn- 
ing with whom he is unacquainted, and to whom he never bows. One morning 
he meets him again and bows to him. He is astonished on account of his 
unmotived greeting. The cause is soon found: he dreamt, during the pre- 
ceding night, that he had had a prolonged friendly conversation with this 
gentleman. This memory apparently had a lingering effect. At the moment 
it did not actually come into consciousness, only the latent memory picture 
was active. There are numerous examples which show us the activity of 
such latent memory pictures in actual psychical life. Our entire process of 
thought and our complete emotional life depend upon the actions of such 
latent impressions. But I expressly chose the example just related because 
it may serve as the prototype for the pathologically increased activity of latent 
memory pictures in hysteria. It shows us the conspicuous effect of a single 
latent emotional impression quite isolated in the midst of normal life. In 
hysteria analogous actions accumulate to a pathological degree and extend 
their sphere of action very much further. Again, while in normal life an 
occasional prominent lingering effect — as in our example — is corrected at 
once, in hysteria this correction does not take place on account of the much 
greater activity of such emotional conceptions. 

I recently had an opportunity of observing a very characteristic example 
of the importance of these unconscious emotional impressions. A girl, aged 
18, with a hereditary predisposition and presenting all of the typical symptoms 
of the hysteric, psychopathic constitution, while studying art (which she en- 
joyed very much), showed a special preference for the "creation of Adam" 
as depicted by Michael Angelo in the Sistine Chapel. One day she noted that 
she assumed a constrained position. Only subsequently did it become clear 
to her that this position accurately corresponded to the very characteristic one 
which Adam occupies in this picture. 

It may perhaps be held that in the view just developed hysteria actually 
becomes a psychosis. This is, in fact, correct and we shall soon convince 
ourselves that the origin of all or nearly all symptoms of hysteria are psy- 



ETIOLOGY 1047 

chical, and that, therefore, the psychic condition in hysteria is to be primarily 
considered. On the other hand hysteria differs from the psychoses in the 
ordinary sense to a considerable extent; the psychogenous symptoms, there- 
fore, the expressions of the abnormal activity of emotional impressions, are 
much more diffused in the corporeal region even than is the case in the true 
psychoses. In addition to this the symptoms of hysteria may be especially 
referred to latent memory pictures while in the psychoses actual memory 
pictures have a pathologic effect. This will become clear at once if we con- 
trast hysterical paralysis with the hypochondriac fear of paralysis in the 
many psychoses. The pathologic effect of the impression of paralysis is un- 
mistakable in both instances but at the same time the difference becomes 
obvious : in hysteria there is no knowledge of such an impression of paralysis, 
there is a latent conception which develops an abnormal activity; the con- 
dition is reversed in those hypochondriacs in whom the actual impression of 
paralysis is the decisive element; the patient concerns himself day and night 
with this thought and even speaks of his fears of paralysis. At the same 
time we may convince ourselves of the great difference in the sphere of 
action in both case- ; the latent conception of paralysis in the hysteric pro- 
duces severe corporeal symptoms of paralysis; it completely exclueles the 
region of the paralyzed part from the range of actual associations while the 
psychical life remains relatively hut little influenced) on the other hand in 
hypochondriac conditions the actual impression of paralysis, in the various 
psychoses (and also in hypochondriac neurasthenia), influences the psychical 
processes, the mood, etc., to an intense degree; the corporeal symptoms, how- 
ever, to such an insignificant extent that actual paralyses and similar condi- 
tions are rarely noted. 

Finally, the uniform pathophysiologic concept which we have obtained of 
hysteria prepares us for still another important, principal fact. Although 
hysteria is characterized, in general, by the morbid, exaggerated activity of 
emotional impressions this does not exclude the fact that symptomatically, 
to a certain extent as an addition, a similar pathologically increased activity 
of emotional conceptions may not occasionally appear in other diseases of 
the nervous system. This is confirmed by experience. We find in the various 
functional and especially in the organic affections of the nervous system, for 
example, in brain tumor, in dementia paralytica, etc., occasionally " super- 
imposed " hysterical symptoms. Sometimes these are so marked that we must 
speak of "superimposed hysteria." This observation, therefore, coincides 
with the principle which we have developed. 

With this we have gained a general view-point and at the same time a 
consistent start. Xaturally our subsequent description must show that this 
stand-point is correct and proper. At all events I may state at once that 
it is serviceable if we approach the great variety of hysterical symptoms with 
a guiding thought. 

ETIOLOGY 

Unquestionably the most important causal factor of hysteria is the heredi- 
tary predisposition. I have found this in over one half of all the cases; 
slight predisposing factors in the ascendant not being included. Very often 
there is a marked hereditary predisposition. Here hysteria is, to a certain 



1048 HYSTERIA 

extent, in contrast to neurasthenia, for in the common forms severe heredi- 
tary predisposition plays no important part. The fact is particularly note- 
worthy that the predisposition in hysteria is conspicuously often inherited 
from both parents: father and mother of the patient having suffered from 
hysteria. Briquet in the following sentences has expressed himself very ex- 
plicitly : " Individuals who are descended from hysterical parents show a 
twelve times greater predisposition to hysteria than others/' and, " for the 
daughter of a hysterical mother being attacked by hysteria the probabilities 
are more than one quarter/' But here it must be remembered that besides 
the direct hereditary influence imitation of the pathologic copy and an im- 
proper education are also active. This inheritance from both parents is by 
no means unusual. We recognize quite a number of analogies in which a 
psychopathic form of reaction is inherited from both parents : thus the tend- 
ency to pathologic depression, melancholia, is exceedingly apt to be inherited; 
we may, therefore, readily comprehend that the peculiar psychic mode of 
reaction in hysteria, the abnormally increased activity of emotional impres- 
sions, may also be inherited from both parents. 

In the description of the course of hysteria we shall see that the pro- 
nounced symptoms of hysteria may develop in earliest childhood, or that a 
" psychopathic constitution," in the sense of hysteria, may precede the out- 
break of symptoms for years, in fact may be present from earliest childhood. 
The frequent combination of hysteria with a hereditary psychopathic consti- 
tution, with or without debility, becomes clear in this manner. 

Age also has a predisposing influence. We have just seen that in heredi- 
tary cases the first symptoms may appear early in life, even from the third 
year. The tendency increases as puberty is reached. I, as well as others, 
have treated numerous school-children with well developed hysteria. The 
maximum of morbidity occurs during the period of puberty and the following 
decades. After the 30th year there is a rapid decrease in the number of 
cases, which is only concealed in the male by the common occurrence of the 
traumatic variety of hysteria. Exceptionally the affection is also noted in 
the presenile and senile periods. For the most part a careful examination of 
these presumable forms of presenile and senile cases of hysteria reveals that 
the condition is either a relapse or an exacerbation of a former attack of 
hysteria, or that the hysterical symptoms are only superimposed, upon de- 
mentia arteriosclerotica or senile dementia. I must advise extreme caution 
in making a diagnosis of hysteria that is not superimposed if hysterical 
symptoms appear for the first time in the presenile or senile period. 

The predisposing influence of sex, especially of the feminine, was for- 
merly often exaggerated; nevertheless it cannot be doubted. According to 
my experience, excluding the cases concerned with suits for damages (the 
traumatic) the hysteric morbidity in the female is about 5 times as great as 
that of the male. In my opinion this predisposition may be explained from 
the fact that the characteristic manner of psychical reaction of hysteria is 
decidedly closer to the normal psychical type of woman. It cannot be ex- 
plained from an unequal distribution of acquired etiologic factors iri the two 
sexes; for, I have convinced myself that in the hereditary infantile cases and 
those appearing at puberty, in which an acquired etiologic factor as yet plays 
no role, that the female is much more commonly attacked. 



ETIOLOGY 1049 

In place of hereditary predisposition other deleterious factors may appear 
at any age and in either sex. Among these there must first be mentioned 
prolonged psychical influences which aid the development of an abnormal 
mode of mental reaction. Faulty education is one of the most important of 
these, for it neglects the duty of teaching the child to control its emotions 
and to keep its imagination in check. Association with hysterical persons 
may act in the same manner. The danger of attending spiritualistic seances, 
by which the expectation is actually pitched for the experience of abnormal 
emotional conceptions, is equally dangerous. It is obvious that all of these 
deleterious psychical factors are much more harmful in predisposed indi- 
viduals, but I know of quite a number of cases in which these prolonged 
injurious psychical factors have produced typical hysteria where there was 
no demonstrable predisposition. 

Intense emotional expectation, probably in addition to fright, is the essen- 
tial factor in traumatic hysteria. From our knowledge of traumatic neuroses 
we know that on account of suits for damages the patient observes his own 
condition with exceeding care, particularly in regard to sequels of the acci- 
dent; in addition to this there are also intense emotional impressions in 
regard to the financial consequences of the accident. It is, therefore, readily 
conceivable that trauma gives rise to pathologic conditions by means of these 
prolonged psychical influences which are either hysteria itself or are very 
closely allied. All of these facts become even of greater importance when 
we compare how slight the effect of other prolonged psychic influences is, such 
as sorrow, anger, and the emotional experiences of the constant struggle for 
existence, as well as intellectual overexertion. 

The importance of acute psychical influences, especially of fright, must 
be estimated quite differently. Fright rarely causes hysteria, but it awakens 
a " slumbering " hysteria, either in that hysterical symptoms were absent 
previously (which I regard as exceedingly rare), or that the previously exist- 
ing hysterical symptoms were relatively slight. With Charcot we may regard 
fright as an " agent provocateur " of hysteria. More frequently fright only 
causes the appearance of some new symptom, as a hysterical disturbance of 
speech, or a hysterical paralysis (for example in the arm, the patient having 
fallen upon this member) ; or even a modification of some of the old symp- 
toms. In regard to trauma, in so far as it is associated with fright, a new 
etiologic relationship to hysteria is shown. In this connection it is imma- 
terial whether the trauma consisted of a severe head injury, or a box upon 
the ear, an earthquake, or a lightning stroke. Psychical trauma is the de- 
cisive factor. It may even be that in such cases bodily trauma is entirely 
lacking. 

Those cases are also to be referred to psychical trauma in which the out- 
break of hysteria arises from imitation, from the observation of a spasmodic 
attack, or of chorea, etc., in a fellow pupil, an acquaintance or the like. The 
symptoms, then, often resemble the spasmodic convulsions. This "imita- 
tion" hysteria appears most frequently in children; occasionally in this 
manner small epidemics arise. 

The etiologic relations of hysteria to definite, material, that is, corporeal 
injury is not so clear. And among these trauma would also come into ques- 
tion in so far as it is associated with shock. The question as to whether 



1050 HYSTERIA 

there is pure, traumatic shock-hysteria^— independent of the chronic psychical 
factors mentioned above with which trauma is commonly combined, and 
independent of traumatic fright — has not yet been decided. According to 
different observations, in which neither sorrow nor fright were combined with 
trauma and severe shock, I must answer this question of the rare occurrence 
of true shock-hysteria in the affirmative. 

The importance of chronic intoxication, especially by alcohol and lead, 
is also a disputed question. Cases occasionally do occur, in which, without a 
demonstrable predisposition or cause, there develops, upon the basis of alcohol, 
lead, or morphin, a typical hysterical symptom-complex in addition to the 
symptoms characteristic of these poisons. In part these cases are to be in- 
cluded with superimposed hysteria. The same is true of diseases of metab- 
olism and of the chronic infections, such as malaria. Both factors play only 
a very subordinate part in the etiology of hysteria. 

Nor can an important part be assigned to bodily exhaustion. As im- 
portant as is the latter condition in the development of neurasthenia and 
especially of many neuroses, so rarely is it involved to any considerable extent 
in the development of hysteria. 

Great significance was formerly attached to the female genitalia. Even 
to-day the view is very prevalent that displacements of the uterus, chronic 
parametritis and endometritis as well as chronic oophoritis are capable of 
producing reflex hysteria. Probably in all of the instances, in which there 
is actually a connection, the psychical influences caused by the genital affec- 
tion are the active etiologic factors. The same is most probably true of the 
influence of castration and the climacterium. In both of these conditions 
we do not note the symptoms of hysteria, but pathologic pictures, which must 
be included partly with the vasomotor neuroses, partly with the vasomotor 
form of neurasthenia. 

The other " reflex "-hysterias hardly deserve the name. We can only say 
that some few hysteric symptoms occasionally appear from peripheral irrita- 
tion in a reflex manner. Thus in rare cases a superficial or deep cicatrix 
remaining from a trauma in an individual who has hysteric manifestations 
may be associated with clonic spasm in the neighboring muscles, which upon 
superficial observation appears to be reflex. 

Much more protean is the 

SYMPTOMATOLOGY 

of hysteria which we shall now consider. 1 It is best to subdivide the symptoms 
of hysteria according to their occurrence in definite attacks or as to whether 
they are more or less permanent. The former are called paroxysmal symp- 
toms (occurring in the attack), the latter symptoms of the interval or 
permanent symptoms. The prototype of the first is the ordinary hysterical 
spasmodic paroxysm. The permanent symptoms may be subdivided into 
psychical and somatic. The symptoms of the attack and especially of the 
hysterical convulsion are so intimately associated, consisting of psychical 
and somatic ones, that an analogous subdivision is not advisable. 

1 In women the examination should always be undertaken in the presence of a 
relative, a nurse, or of another physician. 



SYMPTOMATOLOGY 1051 

I shall begin with, the permanent, somatic symptoms, for these are calcu- 
lated to show us the mechanism of the hysterical symptoms in the plainest 
manner. 

Among the 

PERMANENT SOMATIC SYMPTOMS 

the signs of degeneration are least interesting. In correspondence with the 
frequency of a marked hereditary predisposition they are observed much more 
commonly in the hysteric than in the normal person; but they are not char- 
acteristic, for they are also noted in other neuroses and psychoses in which a 
marked hereditary disposition plays a role. 

On the other hand we are in the midst of the characteristic hysterical 
symptoms as soon as we pass to the hysterical 

DISORDERS OF MOTILITY 

and among these we must primarily consider hysterical 

Paralyses. 

We have already informed ourselves in regard to their general patho- 
physiologic origin, from accentuated emotional impressions, and, as a rule, 
from latent emotional impressions. Often the origin is quite clear, as if 
after a fall upon the left shoulder there is paralysis of the left arm or after 
a fatiguing march there is paralysis of both legs. The momentary loss of 
speech or of power in the legs, which sometimes accompanies the effect of 
fright in the normal person, produces a permanent difficulty in speech or a 
permanent paraplegia, etc., in the hysteric. In other cases it is impossible 
to determine the latent conception by which the paralysis has been brought 
about. 

The distribution of the paralysis is usually very characteristic. It ap- 
pears in connection with the popular impression of the divisions of the 
human body and is independent of the anatomical grouping of the motor 
tracts and centers. The right, or the left arm, the right, or the left hand, 
the right, or the left leg, the right, or the left foot correspond to this un- 
sophisticated conception of division. If two extremities are paralyzed these 
are commonly the arm and leg of the same side or both legs. The com- 
bination of both extremities of one half of the body coincides with the naive 
conception of symmetry and is associated with the popular idea of an attack 
of apoplexy. The inclusion of both legs is apparently grounded upon their 
common activity in walking and standing. Thus we are able to understand 
that paralyses limited to a portion of the extremity as well as monoplegias, 
hemiplegias, and paraplegias appear in hysteria. 

However, these forms of paralysis, in which, in the course of a special 
region, all conscious movements are arrested, present only one type of hyster- 
ical paralysis. A second type merely reveals the cessation of a special conscious 
movement or of a group of these, for example, of speech, walking, or stand- 
ing, while the muscles, which do not function during this act, are normal 
under other conditions. Correspondingly we refer to hysterical aphasia, 
hysterical abasia and astasia. Nor do these paralyses correspond with any 



1052 HYSTERIA 

degree of accuracy to the functional grouping of the centers in the central 
nervous system. In this second type the naive ideas of the divisions of our 
body are not decisive for the paralysis, but merely the naive performances of 
our important bodily movements. The paralyses of the first type may be 
designated as psychogenous regional paralyses, those of the second type as 
psychogenous functional paralyses or paralyses of coordination. 

In regard to the two types the following points must be noted: 

The paralyses of the first type, therefore, the regional paralyses, as a rule, 
do not involve the muscles supplied by the facial nerve, nor those of the 
tongue and eyes. The movements of the head and trunk are mostly normal. 
The limitation of the paralysis is generally dependent upon the previously 
mentioned naive ideas. Thus, it occurs that in a regional paralysis of the 
hand the loss of power is not infrequently limited directly to the movements 
of the hand, while motion in the neighboring joints is quite intact. In the 
same manner we may explain the non-involvement of the leg upon the same 
side in a hysterical monoplegia of the arm ; an occurrence which is rarely met 
with in an organic monoplegia. 

Hysterical hemiplegias, paraplegias, and monoplegias, betray their psy- 
chogenous origin in still other directions, particularly in their instability. 
In contrast to the organic form these paralyses may improve suddenly under 
special circumstances, for example, under excitement a slight movement of 
gesticulation may appear in the paralyzed extremity, or being in danger of 
falling the patient puts out the hand for protection. Oppenheim has noted 
that by careful manipulation of the hysterical paralysis, now and then a 
slight contraction may be produced; that a slight condition of innervation 
may be noted under these circumstances I am in a position to confirm. 
Finally it is very significant that if the patient is under the influence of 
alcohol or of an anesthetic (both at the beginning of narcosis as during the 
early period of awakening) the paralyzed extremity may be normal in action. 
It is obvious that in all of these cases the pathologic action of the latent 
emotional conceptions is either compensated for or inhibited for the time 
being. 

In many cases the paralysis is not absolute; on the contrary, there is 
a more or less marked paresis. In these instances it is particularly notice- 
able to what extent the intensity of the paralysis is under the influence of 
psychical conditions. The merest suggestion, such as directing attention to 
the paretic extremity, is sufficient to increase the paresis. If the condition 
concerns a hysterical hemiparesis it will be noted that the gait does not cor- 
respond to that of organic hemiplegia: the tip of the foot is almost never 
dragged and even less frequently thrown out into a convex arc, but is dragged 
in an almost straight direction upon the entire sole, or upon the lateral 
borders of the foot. 

The muscles of the tongue and those supplied by the facial nerve are 
rarely involved in the paralysis, especially in the hemiplegia. Such a con- 
dition is sometimes simulated from the fact that the hemiplegia is occasion- 
ally accompanied with a crossed contracture of the muscles of the tongue and 
those innervated by the facial nerve. Only in the rarest instances is true 
paresis present. Naturally care must be exercised so as not to confound 
slight congenital asymmetry of the facial innervation with paresis. A case 



SYMPTOMATOLOGY 1053 

of true, distinct paresis of the tongue, which I observed recently, revealed 
marked deviation upon protrusion, but it was quite characteristic that the 
paresis, therefore, the deviation, became uncertain when the patient observed 
the condition in a looking-glass. I was convinced that the conception of right 
and left became confused by the image in the glass, the paralysis thereby 
losing its definite character. 

In hysterical paralysis the muscles of the eye are rarely involved and an 
isolated paralysis of hysteric origin is exceedingly infrequent. Only very 
exceptionally have I noted a paralysis of associated lateral movements of the 
eye to one side, which was due to a masked suggestion in the course of the 
examination, the paresis being characterized by the factors previously men- 
tioned. For example the patient does not follow a light held before him 
but with an unexpected noise there is a lateral movement of the eye. A 
hysterical paralysis upward and outward occurs in quite an analogous man- 
ner. In rare cases there is a hysterical paralysis of all movements of the 
eye so that ophthalmoplegia externa is simulated. A true paralysis of con- 
vergence is somewhat more frequent which is also characterized by its in- 
constant nature, for example, its absence upon automatic fixation. I have 
never noted paralysis of accommodation without paralysis of convergence in 
Irysteria. In isolated cases there is unilateral or bilateral ptosis. As a rule 
this condition is simulated by a spasm of the orbicularis oculi; it is then a 
case of hysterical pseudoptosis. True unilateral ptosis is exceedingly rare. 
It has been stated that in hysterical paralysis there is not so much actual 
paralysis as simple relaxation of the levator palpebral This view is un- 
doubtedly correct, but it is equally true of all other forms of hysterical 
paralysis. At the same time it explains in an apt manner the limitation of 
hysterical paralyses : hysteric paralyses only occur in such muscle groups 
as are innervated voluntarily and which may, therefore, be voluntarily re- 
laxed. The external rectus, which we cannot innervate isolatedly, we also can- 
not relax isolatedly and, therefore, we never observe hysterical paralysis of 
this muscle. The few cases of isolated hysterical paralysis of the external. 
or internal rectus, that have been reported in literature, will not stand the 
light of scientific criticism. Eegional hysterical paralyses are nothing else 
than functional paralyses; they differ only from functional paralyses, in the 
restricted sense, that in well developed cases they involve all of the motor 
functions of a portion of the body. 

The hysterical paralyses of the ocular muscles reveal this transition in 
a very demonstrative manner : hysterical paralysis of all bulbar muscles is a 
regional paralysis, which corresponds to the popular conception of the relation 
of the eyes and is absolutely analogous to hysterical paraplegia, while a 
unilateral hysterical glance-paralysis is a paralysis of function, which may 
be compared, for example, to hysterical abasia. 

Quite an analogous condition is met with in the course of innervation 
of the palate, pharynx, and vocal cords. The innervation of the palate or 
pharynx may be arrested or decreased by hysteric paralysis, but here also in 
the inconsequent manner and form which is indicated by its psychogenous 
origin. Hysterical paralyses of the vocal cords are even more frequent. It is 
necessary to be cautious so as not to regard every hysterical loss of voice 
(aphonia) as paralysis; very often this aphonia is dependent upon peculiar 



1054 HYSTERIA 

symptoms of spasm or of incoordination which will be described more fully 
later on. However, even after leaving these cases out of consideration there 
are enough instances of hysterical paralyses of the vocal cords in which the 
genuine psychogenous origin is obvious. The patients declare that it is 
impossible for them to speak aloud, and in fact upon laryngoscopic examina- 
tion it is noted that upon attempts at phonation the normal movement of 
the vocal cords is lacking. In my experience this " paralysis " is always 
bilateral and symmetric. The tension of the vocal cords is normal and only 
in very rare, exceptional cases is there relaxation so that in inspiration and 
expiration they appear to fluctuate with the current of air. In contrast to 
organic paralyses it is very significant that only the constrictors and not the 
dilators of the glottis are involved. 

A general factor of all hysterical regional paralyses is the non-involve- 
ment of the reflexes. The tendon and periosteal reflexes in the paralyzed 
member are not greater than in the homologous non-paralyzed one. But care 
must be exercised that we do not mistake a subsequent non-reflex increase as 
an exaggerated reflex. Occasionally even foot clonus may be simulated in 
the paralyzed extremity in this manner. The same is true of the periosteal 
reflexes. Only after a hysterical paralysis has persisted for many months 
may there be an actual change in the tendon and periosteal reflexes, a con- 
dition that I have positively observed. The behavior of the cutaneous and 
mucous membrane reflexes is somewhat more complicated. It is of clinical 
interest to differentiate between true reflex and resistance reflex. Plantar 
flexion of the toes upon stroking the sole of the foot is a true reflex; dorsal 
flexion of the foot, flexion of the thigh, etc., upon stroking the sole is a 
resistance reflex. In the hysterically paralyzed portions of the body the 
true reflexes are normal, not differing from those of the homologous non- 
paralyzed extremity, while the resistance reflexes are either decreased or abol- 
ished. This is also the reason why the Babinski reflex is never noted in 
hysteria. In testing the reflexes of the skin and the mucous membranes 
many errors are liable to occur. Thus exceptionally in hysterical cases the 
plantar flexion of the toes of the normal sole reflex is suppressed by an 
antagonistic contraction of the extensors of the toes; in others, exceptionally, 
there is dorsal flexion of the toes as a resistance reflex leading to simulation 
of the Babinski reflex. 

Quite as characteristic is the behavior of the electric contractility of the 
hysterically paralyzed muscles; there is no change from the normal. Only in 
rare cases, after the persistence for years of a hysterical paralysis, is there 
a very slight quantitative decrease to direct and indirect galvanic and faradic 
contractility. Nor does mechanical stimulation of the muscles reveal a char- 
acteristic change. 

In general there is no atrophy of the paralyzed muscles. Inactivity 
atrophy develops much later than in organic pyramidal tract paralysis. Only 
in very exceptional cases is marked atrophy noted in hysterical paralysis; 
this presents no severe and especially no qualitative alterations of electric 
contractility. The isolated functional paralyses of hysteria are of great im- 
portance; their definition has already been given. As a rule the most com- 
mon functions are involved, viz., walking, standing, sitting, speech. Accord- 
ingly we refer to hysterical abasia, hysterical astasia, hysterical akathisia, 



SYMPTOMATOLOGY 1055 

and hysterical motor aphasia, that is, anarthria. The latter is often incor- 
rectly designated as hysterical mutism', it is better to reserve this term for 
those psychical, conscious processes upon which the silence depends. Hyster- 
ical abasia is commonly but not invariably associated with hysterical astasia. 
In typical cases this hysterical abasia-astasia is very characteristic: in the 
recumbent posture and in sitting these patients are able to execute all move- 
ments of their legs with ease and with normal power; but so soon as they 
are placed upon their feet " the knees bend as if made of cotton." If the 
condition is one of isolated abasia, standing is normal but the patient is not 
able to move a step ; the soles of his feet appear to cling to the floor. Usually 
abasia is a permanent symptom, i. e., an attempt to walk is impossible as 
soon as the act is undertaken. I am familiar with some cases in which a 
hysterical patient, in the midst of normal walking, was taken with an attack 
of abasia and sometimes also with astasia. These are hysterical attacks of 
abasia and astasia. Naturally it is of the greatest interest, in these cases, 
to determine the accompanying psychical condition. In my experience con- 
scious fear — for example of falling — rarely plays a role, in other words, 
actual hypochondriacal conceptions are rarely involved. But the conscious 
impression of not being able to stand and not being able to walk is not 
present as a rule; the patients are often surprised themselves by these con- 
ditions. If the patient is questioned as to whether she still knows how to 
stand and walk, the answer is sometimes that she does not; more frequently 
she states that she still knows how, but that the limbs refuse to act. This 
is in entire accord with our theoretic considerations at the beginning of this 
article. The circumstances are analogous with hysterical mutism: rarely do 
the patients maintain — in the sense of hysterical " aphasia " — that they no 
longer know the word (word in the motor sense) ; much oftener they state — ■ 
in the sense of a hysterical " anarthria " — that the organs of speech refuse 
function. 

We may still further specialize these functional hysterical paralyses. 
Thus I have seen hysterical patients who were incapable of enunciating some 
special letter of the alphabet, such as r, while all the other letters were 
articulated in a normal manner. 

It is obvious that in these cases of isolated hysterical functional paralysis, 
strictly speaking, we are not concerned with a psychogenous paralysis, but 
with the psychogenous absence of function of some definite coordination. 

Intimately associated with hysterical paralyses are the lrysterical 

Contractures. 

As is the case with paralysis, hysterical contracture often arises in asso- 
ciation with trauma. In some instances severe emotional shock is the acci- 
dental cause. Sometimes contracture arises immediately in connection with 
this, occasionally there is a longer or shorter period of incubation. Often 
the contracture is the result of a hysterical convulsive attack. Less fre- 
quently, as is also the case with hysterical paralysis, is it associated with 
the hysterical somnambulistic condition. Contracture is often accompanied 
with pain, or sensations, which, on their part, represent another hysterical 
symptom or some complicating peripheral affection (caries of a tooth, an 



1056 HYSTERIA 

unguis incarnatus, etc.). I shall refer to apparent reflex contractures 
later on. 

Hysterical contractures of the extremities like paralysis of the extremities 
may be monoplegia hemiplegic or paraplegic. The distribution to the indi- 
vidual muscles and the position which may result is subject to great variation. 
Sometimes an organic contracture may be simulated in the most astonishing 
manner. In other cases the bizarre form of the contracture denotes its 
hysterical nature. Occasionally these contractures give an impression of co- 
ordination; there is an appearance as if the extremity had become rigid in 
the midst of a coordinated movement (Gorgon contracture). 

Often the contracture is limited to a single portion of an extremity; 
isolated contractures of a hand or foot are not rare. In such instances the 
regional character, i. e., the limitation based upon the naive conceptions of 
the divisions of the body, is easily recognized. Specialization in some in- 
stances may even go further than this; thus I know of cases in which only 
the thumb or the second to fourth fingers were involved by the contraction. It 
is unnecessary to describe the individual contractures ; the variation is so great 
that a brief description is impossible, although it is true that some postures 
are more common than others (making a fist, position of writing, crutch 
posture of the leg, etc.) or that some are especially conspicuous (hyperflexion 
of the wrist, hyperextension of the ankle, etc.). Hysterical contracture may 
simulate tetany; as in these cases Chvostek's and Trousseau's signs may be 
temporarily present, the danger of an error in diagnosis is very great. 
Curschmann quite properly emphasizes that the increase of electric con- 
tractility of the motor nerves is the only sign which decides in favor of 
genuine tetany. 

The intensity of these hysterical contractures upon superficial examina- 
tion is hardly less than that of the organic form; on the contrary they 
appear to be more marked and almost extreme in comparison. Accurate 
investigation, however, shows that when the attention is diverted the con- 
traction sometimes becomes decidedly less marked, while inversely if atten- 
tion is aroused, for example, upon an attempt at passive motion, the condition 
is aggravated. In contrast to an organic contraction — in so far as special 
suggestions are not active — it does not lessen in a warm bath. During sleep 
the severest hysterical contraction may disappear completely, while marked 
organic contractures are less obvious but by no means abolished. It has also 
been correctly observed that hysterical contraction does not lessen if the 
point of origin and the insertion of the contracted muscle are approximated, 
while an organic contracture, under these circumstances, shows a certain 
degree of relaxation; in my experience there are many exceptions to this 
rule. Under the influence of an anesthetic hysterical contracture disappears 
completely, and even after the patient awakens from the narcosis the con- 
tracture reappears relatively late. Strong cutaneous irritation often increases 
the contraction. 

Very interesting results are obtained by the examination of the degree 
of voluntary movement in the extremity attacked by the contracture. It has 
previously been stated that a hysterical contracture is often associated with 
hysterical paralysis in the same extremity. But this is by no means invari- 
able; occasionally it is conspicuous — again in contrast to organic contracture 



SYMPTOMATOLOGY 1057 

— that the contracted muscles present no evidence of paresis, as is easily- 
shown by an attempt to overcome the contracture by passive motion. In the 
numerous instances in which contracture is actually accomnanied with com- 
plete paralysis, the relation, in regard to time, of the contracture and the 
paralysis is noteworthy. While in organic paralysis early contracture is 
exceptional it may be looked upon as a rule that hysterical contracture and 
hysterical paralysis appear simultaneously or almost at the same time. 

Just as there are latent organic contractures analogous hysterical condi- 
tions also occur. It is noted in hysteria that while the patient, in general, 
presents no contractures any severe cutaneous irritation is capable of pro- 
ducing one. This so-called diathese de contracture is sometimes limited to 
one extremity, occasionally involves all extremities, and even, as I may state 
in advance, affects the trunk and the head. In place of cutaneous irritation, 
pressure upon the nerve trunks or upon the muscle brings about a similar 
result. The tendon reflexes are as little influenced by hysterical contracture 
as by the paralysis, apart from the purely mechanical decrease in the ex- 
cursus of the reflex movement dependent upon the contracture. 

Next in interest to contractures of the extremity are those involving the 
m,uscles supplied by the facial nerve. In well marked cases this appears in 
association with crossed hemiplegia or -hemiparesis. Sometimes the condition 
is present alone. The branches of the nerve supplying the cheek are com- 
monly most severely affected. As a rule the orbicularis oris is not involved 
nor the orbicularis oculi; this is also the case with the frontalis. A very 
characteristic facial expression appears. In one of my patients the som- 
nambulistic states were always accompanied with a right-sided hemiparesis 
and a left-sided incomplete contraction of the muscles supplied by the facial 
nerve. At first glance it might be supposed that the condition w T as one of 
right-sided facial paralysis, but as soon as the patient was required actively 
to innervate the facial nerve distribution it was noted that the right side 
was entirely intact. Actual hysterical facial paralysis is rare (for example, 
in addition to crossed facial contracture). Another circumstance indicates 
to some extent that the condition is one of left-sided spasm. Upon careful 
observation we may observe clonic contraction at irregular intervals, some- 
times also fibrillary tremor in the left-sided facial muscles. Xot always, but 
quite frequently, this facial contracture is associated with a contracture of 
the muscles of the tongue upon the same side. This is noted from the fact 
that upon protrusion of the organ it deviates to one side, in left-sided con- 
tracture to the right, upon right-sided contracture to the left. Therefore a 
right-sided deviation of the tongue indicates a contracture of the muscles 
of the tongue upon the left side. That there is a contracture and not a 
right-sided paralysis of the tongue may be noted from the attempt to place 
the tongue in a median position when great resistance is encountered. Some- 
times the deviation of the tongue may be noted while the organ is at rest 
upon the floor of the mouth ; simultaneously the contracted half of the tongue 
appears smaller and more arched. Earely in addition to unilateral facial 
contracture and that of the tongue there is a unilateral contracture of the 
muscles of mastication which may even be more prominent; under these 
circumstances when the mouth is opened the lower jaw may deviate (with 
right-sided contracture to the left, and vice versa). More often there is 



1058 HYSTERIA 

a symmetric contracture of the muscles of the jaw, with hysterical trismus, 
as an isolated condition. 

In addition to the contractures that have been described there are also 
isolated contractures in the region of the eyes and forehead that are supplied 
by the facial nerve. Isolated contracture of the orbicularis oculi which the 
inexperienced physician regards as ptosis is, as a rule, dependent upon hyper- 
algesia of the optic region or to hyperesthesia. This will be described later. 
A unilateral contracture of the frontalis, which I have noted to be sometimes 
transitory, at other times more permanent, is independent of sensory dis- 
turbances; it may be associated with a crossed contracture of the orbicularis 
oculi and then produces a very peculiar contortion of the face. 

Contractures also occur in the ocular muscles. According to my experi- 
ence they are always associated ones, for isolated muscles which we cannot 
innervate singly never show hysterical contracture. Convergence contracture 
is most frequent, therefore, a contracture of both recti interni; or an abduc- 
tion contracture, therefore, a contracture of both recti externi. In the latter 
case the axes of both eyes are parallel and immobile. In the former case a 
paralysis of the abducens, in the latter an oculomotor paralysis, may be 
simulated; that is the picture of a complete bilateral ophthalmoplegia externa. 
The difficulties of diagnosis may even be increased by the addition of true 
hysterical paralyses of the ocular muscles, such as were previously described. 
True diplopia and paralysis of the external eye muscles are rare in these 
hysterical contractures, on the contrary hysterical diplopia is, as a rule, mo- 
nocular. Only exceptionally does there appear to be slight asymmetry, or on 
the contrary a congenital asymmetry of innervation in hysterical contracture 
and paralysis of the ocular muscles; then true diplopia may be present. 

Contracture of the levator palpebral is much less frequent. Isolated 
spasm of the ciliary muscle — with or without megalopsia — is sometimes ob- 
served; occasionally it is associated with convergence contracture. In my 
experience contracture of the sphincter iridis and of the dilator pupillae are 
very rare; there is absolutely no reason to regard the very common mydriasis, 
which we find in hysteria as well as in other neuroses, as a contracture. 

Contracture of the muscles which rotate the head, a tonic hysterical tor- 
ticollis, is not rare. The associated nature of the contracture may here also 
be readily determined. Many cases which are now described in literature as 
" mental torticollis " belong to this category. 

Contractures in the trunk appear especially in the extensor muscles. For 
the most part these are unilateral, or at least one side is especially involved, 
and this condition, therefore, produces deviation of the trunk which may 
simulate scoliosis. In addition to the muscles of the back, the glutaei may 
be involved in this contracture. Hysterical pseudo scoliosis is distinguished 
from genuine scoliosis by palpation, which, in the case of the latter, reveals 
a deformity and change in position of the vertebrae and ribs, and in the 
former only a spasmodic contraction of the muscles of the back. 

In addition to contractures we also see other motor irritative phenomena, 
particularly clonic spasms. In so far as these are limited to the hysterical 
attack they will be described later on. Here we are concerned only with 
those that appear in addition to the hysterical attack, being more or less 
continuous. 



SYMPTOMATOLOGY 1059 

Clonic Muscular Spasms 

Hysterical myoclonus cases may be divided in a similar manner to hyster- 
ical paralyses, according to whether they involve the muscles of a special 
region (in the sense of the naive conception of the divisions of the human 
body) or according to the muscles which act conjointly in a definite, isolated 
function or coordinated movement; but here also there is no sharp line of 
demarcation. 

Regional clonic spasms in one or more extremities is very rare. But I 
have seen such cases, in which, for example, an arm or a hand revealed con- 
tinuous clonic spasms for weeks without there being a possibility of recog- 
nizing a definite coordination of the spasmodic movements. Like all clonic 
muscular spasms of hysteria these also show a tendency to paroxysmal in- 
crease; sometimes there is actual intermission in the attacks. As the clonic 
spasm sometimes changes into the tonic form, just as inversely clonic con- 
tractions appear in an extremity which is the seat of hysterical contracture, 
the most varied and mixed pictures of contracture and myoclonus are pre- 
sented. 

Eegional muscular spasms are much more common in the area supplied 
by the facial nerve. If, as is still very commonly done, all clonic facial 
spasms are designated as tic convulsif, this regional muscular facial spasm 
as it appears in hysteria ma)' be designated hysterical tic convulsif. While 
true tic convulsif always depends upon an abnormal irritative condition within 
the lower reflex arc (trigeminal-facial, optic-facial), due to organic or reflex 
causes, the hysterical tic is due to psychogenous motor stimulation, in which 
perhaps peripheral irritation may be active, but which at all events does not 
arise simply in the course of the lower reflex arc. This is at once seen during 
the examination, for all organic symptoms are absent in the distribution of 
the facial nerve, while in the case of true tic convulsif they are common 
(disturbance of electric contractility, abnormal associated movements, such 
as distortion of the angle of the mouth upon forcibly closing the eyes, etc.), 
further from the fact that the relation which true tic shows to blinking is 
absent or nearly so, and, finally, that under psychical influences, emotion, 
under observation, suggestion, etc., it varies to a much greater extent than 
does the genuine tic. It is much more closely allied to tic impulsif of the 
facial region, such as we observe as an accompanying symptom of the maladie 
des tics. Besides the latter is often merely a symptom-complex belonging to 
hysteria. Sometimes the clonus attacks principally the orbicularis oculi, 
sometimes the muscles of the cheek ; the muscles of the chin and of the fore- 
head are less often affected and the orbicularis oris with exceeding rarity. 
Combination with tonic, facial spasm, facial contracture, occurs quite often. 
It is quite remarkable how often these clonic, facial spasms appear in 
traumatic hysteria. In this condition they are often voluntarily exaggerated. 
As a rule the individual contractions are quite uniform and limited to one 
half of the face. I have seen them exceptionally with regular intermissions 
even appear in both sides of the face. 

Hysterical clonus of the muscles which rotate the head, therefore, a hyster- 
ical clonic torticollis, or, we might say, a hysterical tic impulsif of the head 
movements, is quite common. Many clonic forms of mental torticollis belong 



1060 HYSTERIA 

to this category. Clonic spasm of the muscles of the tongue and jaw are 
less frequent. 

Clonic spasm of the diaphragm, hysterical singultus, is a very common 
condition. Occasionally it appears in paroxysms, at other times it is almost 
continuous for days ; it always ceases during sleep. It is frequently associated 
with hysterical disorders of respiration, which will be described more in 
detail later on, or it alternates with these. Not uncommonly is it accom- 
panied with clonic contractions of the muscles which rotate the head and 
also by those of the muscles of the jaw. Clonic spasm of the abdominal 
muscles (hysterical telly-dance) is sometimes observed. 

In addition to these regional clonic spasms there are clonic spasms re- 
sembling coordinated movements. It is usual no longer to employ the 
designation clonic to such spasmodic movements; we shall, therefore, refer 
briefly to localized coordinated spasms. These appear in any region of the 
body. I shall quote a few examples. 

I am treating a lady who suffers from severe carcinomatous metastasis of the 
right brachial plexus. Palpation and the results of the neuro-pathologic examination 
leave no doubt of the diagnosis ( partial DeR, etc. ) . For some time peculiar clonic, 
and, as she states herself, involuntary spasms have appeared in the right arm. She 
throws the right arm to and fro in a very complicated manner. These spasmodic, 
coordinated movements may be produced, arrested, and transformed by suggestion. 
Undoubtedly, as other characteristic hysterical symptoms, stigmata, are present, the 
condition is one of a serious organic disease which has been overshadowed by a 
hysterical symptom-complex. 

In other cases these coordinated hysterical muscular spasms imitate some 
" occupation " movement ; and, even more frequently, they are noted as the 
result of emotional expression or as a movement of resistance. Under these 
circumstances they cannot be sharply separated from the previously men- 
tioned tics. Hysterical coughing and barking must also be included. The 
former may appear in paroxysms, sometimes almost continuous. Hysterical 
barking imitates the various cries of animals. A peculiar inspiratory stridor 
is also occasionally noted. 

Besides, these coordinated spasms interfere with normal movements and 
particularly disturb the regular rhythm of the respiration. Most commonly 
there is a simple acceleration of respiration (hysterical tachypnea) , but the 
rhythm of the respiration may be altered in a very much more complicated 
manner: thus there are sighing inspirations, concomitant distortions of the 
face and rotation of the head, etc. Disturbance of the normal gait by ac- 
cessory, coordinate, spasmodic movements is even more common. The patients 
hop in a peculiar manner or they walk with a peculiar, bizarre gait (gait- 
stuttering, etc.). If we add that these spasmodic disturbances in gait may 
be associated, in the most varied manner, with hysterical paralyses and con- 
tractures of the legs, the enormous variety of hysterical disorders in walking, 
which we encounter clinically, will become manifest. It is not rare for 
hysterical abasia to run through some of these complicated changes in gait 
in the course of recovery. 

Almost quite as common are analogous disturbances of normal speech 
from coordinative, spasmodic movements. Hysterical stuttering is most fre- 
quent. If we consider the acoustic aspect it resembles the genuine stuttering 



SYMPTOMATOLOGY 1061 

neurosis, for it also consists of an interruption of the continuity of speech 
which is noted in individual letters or — much more common than in the true 
neurosis — in separate syllables. The stuttered letter or syllable is prolonged, 
at the same time there is an abortive effect, i. e., there is an incomplete, final 
articulation, but on the other hand it may be repeated several times suc- 
cessively. In hysterical stuttering, just as in the genuine form, certain letters 
are especially affected; as a rule the disturbance is more general than in 
the true neurosis. The processes of innervation, superficially considered, are 
almost identical to those of the true neurosis. Only a careful examination 
reveals differences. The hysterical stutterer shows the defect as a rule — pro- 
vided there are no special suggestive influences — in whispering and in singing 
just as much as in ordinary speech, in contrast to the genuine stutterer. It 
is also significant that the repetition of the same word does not reveal the 
same favorable influence as in the true neurosis. The prolongation of the 
vowels does not favorably influence hysterical stuttering as is the case in 
the genuine variety. Common to both is the increase under excitement. The 
accompanying respiratory disturbances and the tic movements are sometimes 
quite similar. 

Hysterical stuttering may occur in connection with all of the other hyster- 
ical symptoms after a longer or shorter period of latency, in connection with 
psychical shock or other accidental causes; sometimes analogous to the 
disturbances in gait, it is a transitional symptom which may be noted 
during convalescence from hysterical paralysis of speech. The inverse is 
also true, hysterical stuttering may be a prodrome of hysterical paralysis of 
speech. 

Here also spastic, hysterical aphonia must be included: as soon as the 
patient attempts to talk a tonic spasm of the constrictors of the glottis ap- 
pears which prevents the production of voice and even gives rise to dyspnea. 
Speech disturbances of higher coordination, such as coprolalia, are much less 
common. In one case of well developed hysteria I observed this symptom in 
the manner that each hysterical eructation — I shall recur to this spasmodic 
symptom of hysteria — was accompanied by the expression of some indecent 
word or phrase. 

Another peculiar, motor, irritative symptom is hysterical 

Tremor. 

This symptom presents many varieties in hysteria. We may state at 
once that there is no form of tremor which is not occasionally imitated by 
the hysterical form. It occurs in any intensity and in all degrees of rapidity. 
Only a tremor of slight excursus and, on the other hand, of great rapidity 
as well as a very slow one is rarely noted. Upon an attempt at voluntary 
suppression there is often an increase. Sometimes the tremor is most pro- 
nounced during rest ; at other times it is the static, then again the locomotor, 
tremor (the latter is also designated intention tremor) which is most marked. 
Accordingly the tremor of paralysis agitans, the tremor of chronic intoxica- 
tion, the tremor of multiple sclerosis are imitated. In my experience the 
tremor is often combined with a mild but psychogenous tension of the af- 
fected extremity. Like the clonic muscular spasms, described above, the 



1062 HYSTERIA 

tremor may be paroxysmal, at other times continuous, during the entire 
waking period. Sometimes it is limited — and this appears to me to be the 
most frequent variety — to an arm or both arms, at other times it attacks 
a leg or both legs. A tremor affecting only one or both legs is quite rare. 
A strict, regular rhythm is infrequent; on the contrary, it is usual for 
the tremor to be subject to great variations both as regards excursus as 
well as tempo. I have often noted that after a series of 5-10-15 marked 
and rapid movements there is a remission which is again succeeded by a 
new series of active tremor movements, just as if the patient were starting 
anew. 

Eemarkable combinations of the tremor with paralysis and contracture 
are noted. Here Charcot's trepidante abasia must be mentioned. Finally it 
must be stated that hysterical tremor upon an increase of the excursus may 
be transformed into the previously described clonic spasm. Thus hysterical 
tremor of the eyelids cannot be sharply separated from the blepharo clonus 
that has been mentioned, and even when the tremor arises in the extremities 
there is no well defined limit. Characteristic of all forms of hysterical tremor 
is its dependence upon psychical conditions, particularly upon exaggerated 
emotional impressions and upon suggestion. Naturally in utilizing this cri- 
terium we must be particularly cautious in judging tremor, for non-hysterical 
varieties of tremor, even those due to organic lesions, are often greatly influ- 
enced by the emotions. The most certain method appears to me to consist in 
an intellectual and sensory diversion (listening to the ticking of a watch 
which is gradually removed to greater distances from the ear, having the 
patient spell his name backward, etc.), this method often proving valuable. 
If such diversions bring about a marked decrease of the tremor this favors 
the hysterical form. A single examination of the tremor is never conclusive, 
and all the other symptoms must be taken into consideration. For many 
years I have employed the graphic method in investigating the tremor, and 
I must emphasize that this process is not sufficient to decide with certainty 
whether the tremor is hysteric or not. Simulated tremor especially cannot 
be differentiated from the hysterical variety in this manner. 

Hysterical chorea may easily be differentiated from hysterical tremor by 
the irregularity of the movements. Sometimes the choreiform movements are 
distributed over the entire body, at other times they are unilateral or are 
at least most prominent upon one side. Exceptionally they are increased to 
the severest jactitation. 

Disturbances of Coordination 

resembling ataxia are rare. When they are noted they are due almost entirely 
to sensory disturbance and will, therefore, be described later on. Only in 
one case did I observe an ataxia which was not due to sensory involvement; 
in another case the diagnosis — hysteria or a beginning cerebellar disease — 
remained doubtful. Naturally I do not here take into consideration the 
numerous cases in which, by special suggestion, such as the finger-nose test 
when the patient is not instructed with sufficient caution, an ataxia is directly 
provoked. 

Not quite so varied but diagnostically just as important are the hysterical 



SYMPTOMATOLOGY 1063 



SENSORY DISTURBANCES 

I shall begin with the disorders of the contact and pain senses. Accord- 
ing to their distribution we differentiate three main varieties: 

(1) The areal or insular type; 

(2) The unilateral type; 

(3) The geometrically limited, or as I prefer, the regional 1 type. 

Insular, sensory disturbance, in my experience, is the rarest variety. Some- 
times there are hypesthetic, anesthetic or hyperesthetic islets; occasionally it 
is impossible to decide whether the condition of these areas, or the condition 
of the surrounding parts, is pathologic, whether we are dealing with a hyper- 
esthetic area upon a normal base or with a normal islet upon a hyperesthetic 
base and vice versa. Often a sensory alteration in the islet may be in con- 
trast with the sensation of the neighboring parts. With relative frequency 
there is only a sensation of pain instead of an alteration in contact : then we 
are dealing with relative or absolute hypalgesia or analgesia, that is with 
hyperalgesic areas. Finally, contact and pain senses are altered to the same 
extent. The size of the islets varies within wide limits. In general the 
extent of a single islet increases proximally. In my experience the diameter 
varies between 1 and over 10 cm. The shape is sometimes almost circular, 
at other times elliptical. Occasionally the areas are close together, again 
they may be separated by some distance. The outline, the diameter as well 
as the situation, are nothing less than constant. Only when a structure of 
the surface of the body, such as the nipple, forms a starting point, is the 
islet more constant ; in this case the areal sensory disturbance passes into the 
regional form which will soon be described. The influence of suggestion is 
very great upon this insular, sensory disturbance. We often gain an impres- 
sion that only by a comparative examination of the sensation of neighboring 
areas, in the sense of a concealed suggestion, is the insular sensory disturbance 
produced. At times the areas are distributed over the entire body, at other 
times they are unilateral or are found merely upon the trunk, etc. 

If the condition is not one of anesthesia but of hypesthesia, it is of in- 
terest to determine the errors in localization which the patient makes in 
indicating these areas. As a rule these are considerable, but it must be stated 
that in a large number of contacts, now and then, very slight errors occur, 
while in the case of simulation these mistakes are always very great. 

Unilateral sensory disturbance is much more common. Here also the con- 
dition may be hemihypesthesia, hemianesthesia, hemihyperesthesia, that is, 
hemihyperalgesia, hemianalgesia, or hemihyperalgesia. Here also it is some- 
times difficult to decide which half of the body shows the pathologic sensory 
disturbance: we can merely determine the contrast, only a relative sensory 
disturbance. I ordinarily compare pricks of a pin, punctiform contact with 
a fine head of a pin, strokes with the point and head, the comparison being 
made repeatedly upon the left and right sides of the body. A pair of com- 
passes may be employed with advantage upon the trunk, in the face, upon 
the hands and feet, both points being placed upon the part simultaneously. 

1 It is advisable in my opinion to reserve the designation " region " for the limi- 
tations here proposed by me. 



1064 HYSTERIA 

Better results may even be obtained with the pendular esthesiometer. A uni- 
lateral sensory disturbance is commonly limited by the median line of the 
body, i. e., the limit varies as many centimeters from the median line as the 
error amounts to which the normal person would make if he were required 
to indicate the median line with his eyes closed. The reports, according to 
which the sensory disturbance is accurately defined by the median line, 
belong to the realm of fable. The psychogenous origin of the hysterical, 
sensory disturbance is revealed by this fact. Often the mucous membranes 
are involved in this unilateral disturbance, as well as the deeper parts, such 
as the fascia, muscles, periosteum, joints, etc. Occasionally some parts are 
excluded; thus a smaller or larger area adjacent to the median line, or the 
hairy scalp, may be exempt. On the other hand the sensory disturbance may 
extend beyond the median line. These limitations as well as extensions are 
included in the regional sensory derangement which will soon be described. 
A pure, unilateral, sensory disturbance is only a special instance of the 
regional form which we separate for purely practical reasons. Unilateral 
sensory derangement is somewhat more refractory to suggestion than the 
insular variety, but it is much more persistent. Apparently the idea of the 
" right or left side " of the body is a much firmer support than the conception 
of insular areas. Nevertheless the influence of suggestion may be demon- 
strated in many cases. Thus it is possible — quite apart from hypnotic sug- 
gestion — by placing a magnet or a watch, etc., or by stroking accompanied 
with suitable suggestion which need not always be verbal and explicit, to 
transfer, the sensory disturbance from one side of the body to the other. This 
process is known as transference, and I must add that a similar transference 
is also possible with the previously described motor symptoms. The psychog- 
enous origin of the unilateral, sensory disturbance occasionally shows itself 
when pin pricks, that are unexpectedly used for testing the hemianalgesic 
side, produce movements of resistance or flight. We may also often note that 
even unexpected pin pricks do not give rise to pain or that the pain sense is 
greatly decreased. It is interesting that — like most of the other unilateral 
symptoms of hysteria — the unilateral disturbance is found more often on the 
left than upon the right. This is probably explained from the fact that on 
account of a decreased employment the conception of non-use is the more 
obvious; it must also be considered that in fact it is represented by fewer 
motile, tactile and other conceptions in the cortex of the brain. Finally, 
it is possible that left-sided disturbances are less disagreeable than right- 
sided ones; there are actual facts which favor the view that factors of this 
nature are not without influence upon the localization and the choice of 
hysterical symptoms. 

Regional, sensory disturbance essentially corresponds to regional paralysis. 
We are concerned with a sensory alteration, the limits of which are set by the 
naive conceptions of the divisions of the human body. Thus an anesthetic 
region may be limited to the hand; then we refer to the glove-shaped form. 
Or it may be limited to the arm ; if the hand is not involved we speak of the 
sleeve form of sensory change. Localization to the shoulder furnishes the 
epaulette variety, that to the upper trunk the waistcoat form, etc. Analogous 
limitations are found in the leg (the splint form, the bathing- tights form, 
etc.). The localization about a joint is apparently suggested by such a di- 



SYMPTOMATOLOGY 1065 

visional conception; here we find the cuff-like, sensory alteration ahout the 
wrist, etc. The hat or hood variety is also common, therefore, a limitation 
of the sensory alteration to the hairy scalp perhaps including the forehead. 
Even greater and more special limitations have been noted. I have seen 
patients in whom the sensory disturbance was limited to a breast, or an eye, 
or even to the surrounding areas of the breast. The genital region, including 
the genital mucous membrane, may be the seat of this regional, sensory dis- 
turbance. These " regions " may be distributed in great number all over the 
body; on the other hand there may be but a single one. In the former in- 
stance they may be exclusively, or at least predominantly, limited to one side 
of the body and are then associated with the unilateral type. These regional, 
sensory alterations have been described as geometric. 1 In fact the individual 
region, as is to be expected from its psychogenous origin, is bounded by 
relatively simple figures; in the extremities by more or less incomplete circles 
the so-called amputation line: upon the trunk by almost straight lines. The 
designation " geometric " is improper in so far as there can be no question 
of mathematical accuracy, nor does the conception of geometric figures play 
the slightest role in the development of the disturbance. If the patients are 
examined more frequently and with greater care it will be observed that 
the limits are neither sharply defined nor are they at all constant and that 
they may vary to the same extent as do the popular conceptions of the limita- 
tions of the hand, foot, etc. It may often be shown directly that the limits 
may be displaced by suggestion or that the regions may be obliterated in toto. 
In regard to the relation of tactile and pain sensations in these areas the 
same is true as was stated of the insular and unilateral forms: sometimes 
there is merely esthesia, sometimes algesia, at other times both are disturbed. 
Hyperesthesia and hyperalgesia are much rarer than anesthesia, hypesthesia, 
and hypalgesia. The involvement of the mucous membranes and the deeper 
areas is not uncommon in the regional form. It is interesting to determine 
the relations of the boundaries of the different regions to one another. This 
is accomplished by a number of contacts going toward and away from the 
anesthetic zone. In the former instance, in the zoni petal test, a slowly ap- 
proaching stroke of a pin from the normal areas is conducted to the anesthetic 
region and the patient is asked to report when contact or pain ceases. In the 
latter case, the zonifugal test, we start from the anesthetic region and the 
patient is asked to state when the tactile sense becomes apparent. It is in 
consonance with normal psychological laws that the anesthetic zone is found 
to be smaller upon zonipetal testing than with zonifugal. In my experience 
this may be most often demonstrated in hysteria — in contrast to simulation — 
but there are occasional exceptions. 2 

Among the regional hyperalgesias another group must be emphasized on 
account of its practical importance. These are the arthritic hyperalgesias. 3 

1 The designation " segmentary, sensory disturbance " is quite improper, because 
it is liable to produce confusion with the sensory disturbances dependent upon indi- 
vidual segments of the cord. 

2 These exceptions then, in part, correspond to the displacement turgescence in 
taking the field of vision described by Forster. 

3 The joints, in addition to their specific kinesthetic nerve terminations by which 
the specific sensations of movement and position are transmitted, also have nerve ter- 
minations, which, like those of the skin, transmit sensations of contact and pain. 



1066 HYSTERIA 

In my experience they are usually monoarticular. The knee and ankle joint 
are most often involved. As these conditions are frequently associated with 
spontaneous pain and also with vasomotor disturbance they will receive con- 
sideration later on. These symptoms appear with every active or passive move- 
ment in the joint and sometimes even from the pressure of the bones upon one 
another in the same joint. The surrounding cutaneous region is often, but 
not invariably, involved in the hyperalgesia. Such patients naturally avoid 
any movement in the affected joint. 

There may be absolutely no connection between the sensory disturbances 
and the motor phenomena previously described. In many cases, however, 
they are associated. Thus hysterical hemiplegia and contracture may be ac- 
companied with hemianesthesia or hemihypesthesia, hemianalgesia or hemi- 
hypalgesia, etc. Only hysterical, functional paralyses are very often not asso- 
ciated with analogous sensory alterations. 

In rare instances there is complete anesthesia or hypesthesia distributed 
over the entire body. Analgesia or hypalgesia is somewhat more common. 
Particularly in hysterical somnambulistic conditions is this met with quite 
as often as the other varieties of sensory disturbance. The nasal mucous 
membrane, which is especially sensitive in the normal person, may be involved 
in this hypalgesia or anesthesia. But it must be remembered that also in well 
persons, particularly those that are not hysterical, there may sometimes be a 
conspicuous lack of the pain sense distributed over the entire surface of the 
body — exceptionally even to the nasal mucous membrane as an individual 
peculiarity. 

The hysterical pressure points show an especially circumscribed local area 
of sensitiveness. The most important of these are the jugular point (in the 
incisura jugularis sterni), the inframammillary point, the iliac point (about 
midway between the median line and the anterior spine of the ilium), the 
inguinal point, the epigastric point, the supraorbital and parietal points 
(sometimes at the center of the saggital suture, sometimes 1-2-3 cm. to the 
side of it). The sensitiveness of the iliac region was formerly designated as 
ovarie ; we know now that this pressure point is not connected with the ovary. 
Occasionally there is a circumscribed point restricted to the ovary which is 
sensitive to pressure, but it can only be recognized by internal examination. 
An abnormal sensitiveness of the testicles to pressure is not infrequent. The 
intercostal spaces may present the same condition, but this sensitiveness to 
pressure by no means corresponds to the location of the intercostal nerves. 
This condition is often most marked in the mammillary and axillary lines. 
Pressure points in the hypochondrium, upon the sternum, and upon the 
mastoid process are much rarer. Sensitiveness of some few or of all of the 
spinous processes of the vertebrae and of the transverse processes of the cervical 
vertebrae is common. There are also t characteristic pressure points in the 
course of the large joints. It is exceptional to find all of the pressure points 
in one and the same case ; on the contrary we usually find but a limited num- 
ber. It is very rare to find all of them absent. They differ from the analo- 
gous pressure points in neurasthenia, firstly, by the fact that they are limited 
or at least most marked upon one side of the body, and secondly, that pressure 
not only produces pain but also symptoms of an attack (oppression, etc.) 
or an actual attack, and as it is usually expressed have a " hysterogenous" 



SYMPTOMATOLOGY 1067 

effect. Cutaneous sensation in the region of the pressure points is often nor- 
mal. On the other hand there may be regional hyperalgesia which corresponds 
to the form previously described and may be used as a differential factor 
from neurasthenia. If there is hemianesthesia the pressure points are situ- 
ated upon the non-anesthetic side, but I have found them often enough upon 
the same side. There is no satisfactory explanation for the peculiar localiza- 
tion of these pressure points. They rarely correspond to the larger nerve 
trunks although even these often show a greater degree of sensitiveness (not 
a sensation of stretching). Suggestive factors may be decisive. 1 Therefore, 
if the attention is diverted they may disappear. 

Thermoanesthesia, as a rule, goes hand in hand with the contact and 
pain senses. If only the latter, as is often the case, is disturbed, thermo- 
esthesia is intact; but exceptionally there is a parallelism between the tem- 
perature and pain senses. We must be prepared for all kinds of imagin- 
able dissociations of sensation in the case of hysteria. More common than 
thermohypesthesia and thermoanesthesia is thermohypalgesia and thermo- 
analgesia. 

The kinesthetic, sensory disturbances occupy a somewhat more substantive 
position. They are most frequently unilateral; they often accompany hys- 
terical hemiplegia or hemiparesis and, therefore, are often combined with 
hemianesthesia. Occasionally I have noted the condition alone. It is very 
significant that in spite of the existence of severe kinanesthesia there is no, 
or but very slight, ataxia. In other cases the patient may present ataxia with 
the finger-nose and knee-heel tests upon the kinanesthetic side, but not during 
eating, buttoning their clothes, walking, etc. In still other cases hysterical 
kinanesthesia may produce disturbances in coordination which may involve all 
movements of the kinanesthetic members. 

The functions of the special senses are often affected in hysteria. Most 
often we meet with hysterical 

Disturbances of Sight. 

The severest hysterical disturbance of sight is complete bilateral amauro- 
sis. It is quite rare and occurs in connection with a hysterical attack or after 
severe emotion. Jean Paul has furnished us with a remarkable illustration 
in his " Titan." The pupils are oftener dilated than contracted. The re- 
action to light (upon careful examination) is always retained, but it may be 
slight, delayed and sluggish. Amaurosis may last but a few minutes, at other 
times it remains for hours or days; some authors have reported cases in 
which the condition persisted for a year. Often amaurosis is not complete; 
the patient maintains that smoke or fog dims the field of vision. Amaurosis 
may be limited to one eye. 

In contrast to amaurosis, in other cases, there is a unilateral or — more 
frequently — bilateral optic hyperesthesia. 2 In severe cases there is optic 
hyperalgesia. Even feeble light dazzles and may actually be painful. If the 
eyes are illuminated there is continued blinking or an almost tonic contraction 

1 In this connection it must be remembered that these are areas which even in 
the normal person are somewhat more sensitive to pressure. 

2 The designation " hyperesthesia retince " is entirely incorrect. 



1068 HYSTERIA 

of the orbicularis oculi. Often the previously mentioned clonic or tonic 
blepharospasm is due to this optic hyperesthesia or hyperalgesia ; x in these 
instances it disappears or at least lessens in the dark. 

By far the most frequent hysterical disturbance in sight is concentric lim- 
itation of the field of vision. 2 This can only be demonstrated with absolute 
certainty by the perimeter; only in the severest cases is the finger test suffi- 
cient. The field should be determined for white as well as for colors. If 
we are dealing with colors it is necessary to instruct the patient that he is 
not to report the time when he sees the test object but only the moment 
when he recognizes the color. It is also advisable to have the colors tested 
in varied succession; not red at first in all meridians and then green in all 
meridians, etc. Each color must be tested at least 3 times in every meridian; 
with a single test an accidental error may give a very incorrect result, and 
besides, from the variation of the figures upon repeated tests, valuable diag- 
nostic factors may often be obtained. If this causes the examination to be 
too lengthy, there should be an intermission, or several sittings may be neces- 
sary. Finally, it is necessary to test the field of vision of a normal person 
immediately before or after this examination for comparison. On account 
of the frequency of careless investigations of the fields of vision and on ac- 
count of their difficulty and importance in hysteria these few remarks in 
regard to the method of examination do not appear to me to be superfluous. 
If the fields of vision for the individual colors, obtained after careful exam- 
ination, are compared, it will be found that the limitation is often uniform; 
then the normal sequence of the color fields, arranged according to their ex- 
tent (blue — red — green), is also retained. On the other hand occasionally 
the limitation for one color is greater than the others; thus the limitation 
for blue may be especially marked; I have repeatedly observed that the color 
field for green is larger than that for red. Usually the limitation may be 
demonstrated in both eyes. If the condition is associated with hemianesthesia, 
as a rule it is greater in the eye upon the hemianesthetic side. Occasionally 
in a case of this kind it is noted that the limitation in the right-sided half 
of the field of vision in right-sided hemianesthesia, in the left side in left- 
sided hemianesthesia in both eyes is especially marked. True hemianopsia — 
apart from the effect of very special suggestion — has never been observed in 
my experience. 

It is quite remarkable that even in marked hysterical limitation of the 
field of vision orientation in space does not suffer. From this it is apparent 
that the peripheral areas of the field of vision, which are lacking upon exam- 
ination with the perimeter, are by no means excluded completely. This may 
be demonstrated by the stereoscope. If the field of vision is projected at 
various distances upon a blackboard a similar " tubular field of vision " 
may be found, such as is otherwise only detected in malingerers. In contrast 

i In some cases the causal relation is inverted ; optic hyperesthesia forms an auto- 
suggestive complement of the blepharospasm. In rare cases a blepharospasm, that 
has persisted for a year, may produce photophobia which may be regarded as physio- 
logic on account of the long continued absence of light. 

2 An increase of the fields of vision beyond the normal limits does not occur. It 
must be remembered that also in normal persons there may occasionally be very wide 
fields of vision. 



SYMPTOMATOLOGY 1069 

to neurasthenia the concentric field of limitation persists in hysteria inde- 
pendent of fatigue. 

Central (macular) acuity of vision and central (macular) sensitiveness 
for colors, apart from the previously mentioned amaurosis, are rarely dis- 
turbed in hysteria. Central confusion of colors — only due to suggestion dur- 
ing the examination — is very infrequent. 

Much less common are hysterical 

Disturbances in Hearing. 

Like those of sight, they may be unilateral and bilateral. Sometimes 
there is complete deafness, at other times only more or less difficulty in hear- 
ing. Occasionally there are definite, but never sharply, limited and complete 
breaks in Bezold's tone scale. Einne's test in unilateral hysterical deafness 
— under the influence of special auto-suggestion — may, exceptionally, be neg- 
ative; lateralization to the normal side may also take place with Weber's test. 
With the ordinary tests for hearing the hysterical patient may react just 
as a malingerer. If there is hemianesthesia we often find, especially upon 
the anesthetic side, a decrease in the acuity of hearing. 

Acoustic hyperesthesia and hyperalgesia are not uncommon; they are usu- 
ally bilateral. 

Disturbances in Taste and Smell 

occur in the same manner as do the other sensory disturbances of hysteria. 
We, therefore, recognize a unilateral and bilateral ageusia, hypogeusia, anos- 
mia, hyposmia, hypergeusia and hyperosmia. Unilateral decrease of taste 
and smell ordinarily accompany hemianesthesia. 

From these statements it will be observed that quite often hemianesthesia, 
in the restricted sense, i. e., unilateral cessation of the contact sense and the 
accompanying sensitiveness to pain, are combined with a unilateral arrest 
of the function of organs of special sense upon the same side. This entire 
symptom-complex has been designated "mixed sensory-sensorial hemianes- 
thesia." 

For the sake of completeness I must add that the vestibular functions are 
sometimes damaged in hysteria, usually (but not constantly!) bilaterally. 
Such patients have a gait which closely resembles the tottering walk of cere- 
bellar disease. Tormenting, sometimes almost continuous, pseudo-movements 
of objects appear in these cases. Some of the brief or prolonged " attacks of 
vertigo " of hysteria may be explained in this manner and would, therefore, 
be analogous to paroxysmal amaurosis, etc., of hysteria. 

Of specially practical importance are the sensory disturbances of the sexual 
sphere. Besides anesthesia and hyperesthesia of the mucous membrane (of 
the vulva and vagina) a decrease and increase of the normal sensations play 
a great role, therefore, sexual anhedonia and hyperhedonia. 

There are also pathologic increase and decrease of hunger, appetite, and 
thirst. Alimia and bulimia, hysterical anorexia, adipsia and hyperdipsia must 
be mentioned. The latter may lead to polyuria which may resemble the 
severest varieties of polyuria from disorder of metabolism. 

In any of the qualities of the special senses that have been discussed a 
positive or negative emotional phase may develop which may produce path- 



1070 HYSTERIA 

ologic, sensory qualities in any one of them. Pitres has described haphal- 
gesia, a condition in which contact with metals and other absolutely painless 
substances is accompanied by an intense feeling of pain. I know of quite a 
number of hysterical patients who maintain positively that the viewing of 
pointed articles, as the points of a compass, is associated with intense pain 
in the eyes. This aichmalgesia differs from aichmophobia by the complete 
absence of fear. Analogous conditions in the psychical life of normal indi- 
viduals are closely associated. To this category belong many special forms 
of copiopia described by Forster. These "selective hyperalgesias" as they 
should be called, are especially conspicuous among the last mentioned func- 
tions of special sense : the idiosyncrasy of many hysterics to certain foods, the 
condition known as pica being here included. Even the preference of hysterics 
for certain colors, such as bright red, for certain odors, such as strong per- 
fume, should not always be referred to hypesthesia of the organ of special 
sense as is done by Charcot, but more often to a selective, pathologic emotional 
condition, in the sense described above. I need hardly state that all of these 
specialized, or as Janet says, systemized pathological emotional impressions 
depend upon associations of impressions which are decisive for the selection 
or specialization. In aichmalgesia this association of impressions is apparent. 
In many other cases the proof of the selective association is very difficult to 
obtain. I must reiterate emphatically that in these " ps}^chogenous " de- 
rangements of hysteria we are concerned with unconscious association 
processes. It is, therefore, readily understood that the patient is unable to 
give any reliable data in regard to the development of his idiosyncrasy; we 
are even compelled, in the endeavor to give an explanation, to content our- 
selves with hypotheses. Thus we may presume that the haphalgesia for 
metals is unconsciously dependent upon the association of the idea of a 
" weapon," the preference for bright red upon the association of the impres- 
sion of the unusual or conspicuous. At all events these disturbances are 
intimately connected with the psychical sphere; in a restricted sense they are 
even more definitely psychogenous than the simple derangements with which 
we were first made familiar. But they are in apparent analogy to the special, 
motor, functional derangements. Their complete comprehension might be 
attained from the law of reflexion and irradiation of emotional conceptions, 
to which I must refer the reader. 

Up to this point we have considered only the sensory disturbances of 
hysteria originating from stimulation outside of the nervous system. In 
addition to these there are also 

Spontaneous Pains 

the source of irritation for which we must seek exclusively within the nervous 
system itself. Often these are the symptoms which cause us the greatest 
difficulties in treatment. As regards distribution the regional type is by far 
the most common. I designate these regional pains as topoalgia. 1 The pain 
is frequently localized in a joint, especially in the hip or knee joint. Then 
we refer to hysterical arthralgia. Here must also be mentioned hysterical 

1 1 give this term a broader meaning than was originally intended by Blocq with 
his topoalgia. Psychalgias have also been mentioned. 



SYMPTOMATOLOGY 107 1 

rachialgia, a regional, spontaneous pain, appearing along the entire extent 
of the vertebral column or localized in some of its divisions ; further, coccygal- 
gia or coccygodynia, sacralgia, mastalgia, etc. Hysterical cardialgia is also 
very common. Localization of the pain may sometimes imitate bladder or 
renal colic. I remember a case in which the symptoms so closely resembled 
hepatic colic that an operation was performed, but only normal relations were 
present. Limitation of the pain to a single extremity is also noted. In the 
head the pain is most frequent in the parietal region; more rarely it may 
have an occipital or temporal localization. It is also called clavus. Some- 
times it is median, at other times unilateral. The unilateral type, hysterical 
hernial gia, is less common than the regional form. On the other hand pains 
are often observed which, upon superficial examination, appear to follow 
accurately the distribution of a nerve trunk, therefore, resembling true neural- 
gia. If these hysterical pseudoneuralgias are carefully examined it is found 
that the pain is not limited to the distribution of a peripheral nerve, but that 
they are modified by the manner in which the physician investigates the 
sensory condition or by questions giving rise to that suggestion. 1 Apparently 
these are, therefore, only regional, spontaneous pains of psychogenous origin, 
topoalgias in our sense of the term. Hysterical intercostal and trigeminal 
neuralgias are most frequent. In my experience these pseudoneuralgias are 
never accompanied with herpes. The trigeminal neuralgia may be limited to 
one branch. When the pains are situated in the cardiac region they may 
radiate to the left arm and resemble angina pectoris, particularly since the 
pulse in these attacks of pain is rapid and labile. In a similar manner there 
may be a resemblance to sciatica. 

The first appearance of pain may be in connection with emotional shock 
or a spasmodic attack ; sometimes it is actually due to some peripheral irrita- 
tion. Thus I have repeatedly seen an exceedingly tenacious topoalgia of the 
leg in connection with some gynecological affection, such as a hematocele. 
The irradiation of pain due to the latter condition is apparently the cause of 
the pseudoneuralgia and determines its localization. 

The pains are stated by the patient to be extremely intense. At times 
they are continuous, at other times paroxysmal. External irritation, such 
as cold, pressure, etc., which in true neuralgia and especially in neuritis, as a 
rule, produces an exacerbation, usually has no constant influence. But the 
significance of psychical factors is all the more noticeable. Diverting the 
attention is of decisive importance. I knew a married woman whose exceed- 
ingly tenacious neuralgia disappeared after she had procured a lover. Quite 
as characteristic is the influence of skilfully concealed suggestions. 

Sensation may be entirely intact in the region of the topoalgia; more 
often there is regional hyperesthesia and especially regional hyperalgesia. 2 
Clavus may be associated with a more or less extensive hyperalgesia of the 
scalp, hysterical coxalgia, i. e., hysterical arthralgia of the hip joint with 
hyperalgesia in the shape of " half a loin cloth " or with hyperalgesia of 

i Even an insignificant, actually subsistent organic pain may act in this sense. 

2 In those cases in which hysterical topoalgia, as mentioned above, occurs in con- 
nection with an organic disease of the internal organs, the hyperalgesia zone may cor- 
respond to Head's irradiation zones. In the main, however, the regional hyperalge- 
sias of hysteria have nothing in common with Head's zones. 



1072 HYSTERIA 

the capsule of the hip joint, etc. In addition there is often an additional 
pressure-point. Thus originates the triad: topoalgia, regional hyperalgesia 
and pressure-point that we meet with so often in practice. 

Marked motor symptoms appear in addition to these spontaneous pains 
of hysteria. Among these is the avoidance of movement due to the fear of 
pain; this occurs especially in that part of the body that is the seat of the 
topoalgia. This fear of movement occurs especially in those cases in which 
the topoalgia is combined with hyperalgesia of the joint, each movement 
increasing the pain. Hyperalgesia of the joint without topoalgia is sufficient 
to produce this fear of motion. If these topoalgias or hyperalgesias are very 
much distributed they may cause an almost absolute immotility. This con- 
dition has been designated acinesia algera. In an analogous manner hysterical 
cardialgia, combined with hysterical hyperalgesia of the mucous membrane, 
may give rise to almost complete refusal of food (aphagia algera), etc. The 
conscious motive in the avoidance of pain need not always be present; there 
are also conditions in which motion is avoided, where it is necessary for us 
to postulate a topoalgia as an unconscious psychogenous factor. 

In other cases the movements are not arrested, but are modified in a 
characteristic manner so as to protect the joint in which there is topoalgia. 
Thus in hysterical coxalgia and gonalgia there is limping, etc. The exceed- 
ingly numerous hysterical disturbances of gait are still further increased by 
this condition. 

The motor, irritative symptoms which occur in connection with topoalgia 
are of great importance. Thus the clonic muscular spasms may develop sec- 
ondarily as the result of topoalgia. In hysterical pseudoneuralgia of the fifth 
nerve there is a hysterical tic which may simulate tic convulsif such as ac- 
companies true tic douloureux. Hysterical brachialgia may lead to peculiar 
clonic spasms of the arms, etc. Hyperalgesia often plays a similar part to 
topoalgia. Probably vomiting, which is often observed in hysterical cardi- 
algia, must be embraced here. I include, further, among these secondary, 
motor, irritative symptoms the contractures which appear occasionally in 
the area of the topoalgia. Such hysterical contractures, the result of pain, 
are most often observed in arthritic topoalgia, therefore, in hysterical coxalgia 
and gonalgia. The extremity may assume the position which is character- 
istic of coxitis or gonitis. In some cases it has appeared to me that hysterical 
contracture and hysterical arthralgia have appeared simultaneously and that 
they are coordinated. 

These spontaneous pains of hysteria are of additional importance from 
the fact that hysteria is often complicated with true migraine. I have already 
stated that among the spontaneous pains of hysteria there is also a unilateral 
" regional " headache. This is not, as a rule, associated with symptoms on 
the part of the sympathetic nerve nor with vomiting, and can, therefore, not 
be regarded as migraine. We only observe genuine attacks of migraine as a 
complication. 

In addition to pain there are also other sensory and sensorial irritative 
symptoms which appear occasionally, such as paresthesia and, in so far as 
they are accompanied by pain, also paralgias; further pruritus, and on the 
part of the organs of special sense, tinnitus aurium, sparks before the eyes, 
etc. The attacks of vertigo of the hysteric sometimes present peculiarities 



SYMPTOMATOLOGY 1073 

which may be due to an irritative condition in the vestibular distribution. 
All of these sensory symptoms may pass into true hallucinations. It will, 
therefore, not appear strange that — especially in regard to their psychogenous 
development — these, as well as the topoalgias, have been designated as " pain 
hallucinations." 

In contrast to the manifold disturbances of motion and sensation which 
we have learned to recognize, the alterations on the part of the 

REFLEXES 

are of decidedly less importance. 

In testing the tendon reflexes we often find an active, symmetrical increase 
in all of them. Xo special importance is to be attached to this, as the same 
condition is likely to occur in any of the other functional neuroses and in the 
neuropathic predisposition. In many cases the tendon reflexes are entirely 
normal. A decided decrease or the entire absence of the reflexes is exceed- 
ingly rare in uncomplicated hysteria. It is especially noteworthy that the 
tendon reflexes of the paralyzed members, in the course of hysterical pa- 
ralysis, are not influenced. This is an important differential point from 
organic paralysis. An increase of the tendon reflexes is sometimes simu- 
lated, in that the patient develops more or less conscious movements of re- 
sistance to the reflex action. This is often easily recognized from the fact 
that the reflex movement occurs too soon or — what is more common — appears 
after a short pause. Even foot clonus may appear from such psychogenous 
additions, especially if the test is performed by the physician in a manner 
that may convey an idea of suggestion. A true, well developed foot clonus x 
has been noted by me as a transitory symptom in hysterical somnambulism. 
Hysterical anesthesia has as little influence upon the tendon reflexes as 
hysterical paralysis, even when the kinesthetic sensations have been com- 
pletely abolished. It must be stated in addition that the tonus, as tested 
by passive movements of the joints, is never decidedly decreased or even 
abolished; only slight hypotonia and hypertonia is sometimes noted. 

The pupillary reflexes are always retained. Especially rigidity to light, 
as I may state from my experience, is never observed. The isolated cases of 
hysterical rigidity to light of the pupils, mentioned in literature, are open 
to doubt; sometimes the test was not performed carefully enough, and at 
other times it was apparent that the patient had produced the condition 
artificially by atropin, etc. ; finally, syphilitic disease of the brain, combined 
with hysteria, must not be forgotten. But it must be admitted that occa- 
sionally the reaction to light, in consequence of a spasm of the sphincter or 
of the dilator, may be slight or may appear very sluggish. Convergence is 
absent only when convergence and accommodation are absent from hysterical 
paralysis; there can be no question of an absence of reflex, for the reaction 
is lacking only because the stimulus is lacking, and in addition we are not 
dealing with a true reflex. 

I must state in advance that in regard to the cutaneous and mucous mem- 
brane reflexes the condition, in our clinical examination, is by no means so 
simple as might seem from older views. The true reflex motion is, as a rule, 

i Indications of a foot clonus are often found. 



1074 HYSTERIA 

accompanied by a psychogenons double. This is best observed in the sole 
reflex. Upon tactile irritation of the skin of the sole the following move- 
ments may be observed: 

(1) Plantar flexion of the toes. 

(2) Contraction of the tensor fasciae lata?, occasionally also of the quad- 
riceps. 

(3) Dorsal flexion of the foot. 

(4) Flexion at the knee and hip joints. 

Among these 4 components of the sole reflex the third and fourth reveal, 
in a conspicuous degree, the character of resistance and flight movements. 
The same is also true of other reflexes. Unfortunately we do not know in 
what portions of the central nervous system the point of origin of the indi- 
vidual components of these movements is to be sought. This much is, how- 
ever, certain, that the individual components, in pathologic conditions, vary 
in their behavior to one another. In hysteria we note a more or less general 
decrease or even arrest of all cutaneous and mucous membrane reflexes. Espe- 
cially the palate and retching reflexes, the conjunctival reflex (the palpebral 
as well as the episcleral) and the sole of the foot reflex are entirely abolished. 
It is not necessary that anesthesia accompany this condition. Great im- 
portance must not be attached to this absence of reflexes, as it may occur 
as an individual peculiarity in normal persons. Very much rarer is the 
absence of the epigastric reflex; naturally under the supposition that the test 
is made under conditions which permit the production of the reflex (flac- 
cidity of the abdominal walls, etc.). It is also rare for the corneal reflex to 
be absent. 

The behavior of the reflexes in hysterical hemiplegia and hysterical hemi- 
anesthesia is of greater importance. It is characteristic of the former that 
the Babinski reflex (dorsal flexion of the toes, especially of the great toe, 
instead of plantar flexion) and also Oppenheim's sign (dorsal flexion of the 
foot and of the toes upon stroking the medial surface of the lower leg) are 
never observed; but care must be exercised that psychogenous movements of 
resistance are not mistaken for the reflex. This latter condition is usually 
most distinct in the sole reflex, and I have found that, as a rule, the 4 com- 
ponents are most affected. The fact must not be concealed that other in- 
vestigators, even recently — for example Crocq — have arrived at different 
results. In hysterical hemianesthesia a similar condition is noted; upon the 
anesthetic or hypesthetic half of the body the reflexes are decreased and 
especially in those components which — such as the movements of resistance — 
also under normal conditions are under psychical control. This permits us 
to comprehend that the winking reflex — and not only in complete amaurosis 
— is often decreased. 

Perhaps more often a pathologic increase of the reflexes is noted in 
hysteria. These also affect particularly or even exclusively the complicated 
resistance components of the reflexes. These are patients who upon the 
slightest touch of the sole not only raise the leg but bend the entire body; 
upon illuminating the eye they not only close the organ tightly but also 
draw back, etc. These psychogenous pseudoreflex movements are not only 
called forth by the normal reflexogenous zones but in severe cases from any 
area of the skin. Very often there is an underlying general hyperalgesia or 



SYMPTOMATOLOGY 1075 

hyperesthesia. It may often be noted that pressure upon one of the pressure- 
points gives rise to decided pseudoreflex movements. I am, however, familiar 
with positive cases in which hyperesthesia, hyperalgesia and sensitiveness to 
pressure over the pressure-points were emphatically denied, and nevertheless 
this exaggerated pseudoreflex irritability was present to a marked degree. 
Occasionally it may be unilateral and is then often associated with hemi- 
hyperalgesia. If the pseudoreflex irritability is greatly increased there may 
be pseudoreflex, clonic and tonic spasms, which throughout resemble the 
primary motor irritative symptoms that we have already learned to recognize. 
We may be much briefer in regard to the symptoms of hysteria in the 
field of the 

SYMPATHETIC NERVOUS SYSTEM 

We shall begin with the vasomotor phenomena. A slight tachycardia is 
common. Usually it appears paroxysmally ; severe attacks are rare. Brady- 
cardia is even less common. The surface temperature in hysterical paralyses 
is frequently decreased in the paralyzed extremity, even if the paralysis has 
been present but a brief time. The characteristic initial rise of the surface 
temperature present in most organic paralyses I have never observed in 
hysterical paralysis. Blood pressure shows exceedingly variable conditions. 

Peculiar forms of edema are very conspicuous. These are occasionally 
observed in hysteria. We may differentiate 3 forms : 

(1) Quincke's circumscribed edema, 

(2) Blue edema and 

(3) White edema. 

True circumscribed edema corresponds to the angioneurotic edema of 
Quincke and is very rare in hysteria. I believe it likely that when present 
it is chiefly a complication. 

Blue edema, also called Charcot's edema, is somewhat more common. In 
this condition as in Quincke's edema there is no marked pitting upon pres- 
sure. The extent varies greatly, but it is usually not distributed over large 
areas. I have repeatedly seen the edema limited to the neighborhood of a 
joint, which, in itself, was not involved. The surface temperature in the 
area of the edema is decreased. It appears least often in the face. I have 
observed it most commonly in the hand and forearm, especially upon one 
side, but it is not rare in the upper arm and in the leg. It is frequently 
complicated with other local symptoms of hysteria, such as regional paralysis, 
regional contracture, regional anesthesia, analgesia or hyperesthesia and hy- 
peralgesia; the last named is then commonly limited to the joint in the 
surrounding of which the edema has appeared. It is often accompanied with 
a regional, articular topoalgia. Sometimes it appears gradually, at other 
times acutely. Sometimes its development is connected with an insignificant 
trauma. Its course may be very chronic. Under psychical influence it may 
disappear very rapidly. 

The white, or Sydenham's edema differs only by the pallor of the skin. 
As there are many transitional forms (apparently also an cedeme rouge) I 
doubt very much whether the white and the blue edema are essentially differ- 
ent. The associated symptoms and the course are identical. 

It has been stated that the mucous membranes are not involved by these 



1076 HYSTERIA 

pseudo-edemas; nevertheless, in a case of severe hysteria, in which there had 
formerly been hysterical aphonia I observed an edema of the mucous mem- 
brane of the palate and of the false vocal cords, which persisted for a year. 
The case was watched by laryngologists and was regarded by them finally 
as neuropathic. 

It is not known to what vasomotor disturbance this form of edema is due. 
It is generally supposed that it is the result of vasomotor spasm or the like. 

Other cutaneous vasomotor affections have been described in hysteria, but 
in this connection I cannot enjoin sufficient caution. These are, as a rule, 
either accidental complications or — more frequently — injuries which the pa- 
tients produce purposely. A tendency to hemorrhage resembling hemophilia 
appears exceptionally, but especially in this matter there should be great 
scepticism on the part of the physician. In the only case in which I was 
able to avoid an error by personal observation there were not only cutaneous 
but also intestinal and pulmonary hemorrhages. The patient perished from 
the continued hemorrhage. The autopsy revealed no sufficient cause for the 
hemorrhage. It is not unlikely that the insufficient and monotonous form 
of nourishment of the patient causing anorexia played an important role in 
the condition. On the other hand we are often astonished in hysterical 
patients at the slight bleeding after injury. 

If we were to credit the reports of the patients, secretory disturbances 
would be very common. Thus the patients complain of lachrymation, rhinor- 
rhea, sialorrhea, hidrorrhea (hyperidrosis), galactorrhea (from the breasts), 
etc. Most of these cases are found to be simulated. Nevertheless, some few 
disorders may be present. Colporrhea has been observed. It cannot be 
doubted that the excretion of urine is subject to great variation and this 
independent of the intake of fluid. Oliguria and polyuria may alternate. 
The influence of emotional disturbance is readily recognized in this. The 
cases of anuria lasting many days are deceptions. 

Disturbances of metabolism do not occur in hysteria, at least not outside 
of the hysterical attack. 

The so-called hysterical fever is almost always artificially produced. If 
the physicians who have described this condition would observe the matches 
at the bedside of the patient most of these cases would be unmasked. Only 
when the physician takes the temperature with his own thermometer in the 
axilla of the patient or in the rectum are we certain that there is no de- 
ception. Although we have often been told by the patient and by the family 
of temperatures up to 110° F. I have never found temperatures above 
101.5° F. ; naturally apart from cases in which there is no complication such 
as miliary tuberculosis I can only admit that under the influence of emotion, 
etc., more readily than in normal persons, slight rises in temperature may 
occur, but the designation " fever " is entirely misplaced when applied to 
this condition. 

In regard to menstruation I believe that in hysteria — quite apart from 
any complication of the genital organs — irregularities are more common than 
under normal conditions and especially than in other nervous affections. 
Here also emotional disturbances appear to be of importance. 

Disturbances in the innervation of the smooth muscles of the intestines 
have also been observed. At times these conditions are spasmodic, at other 



SYMPTOMATOLOGY 1077 

times paralytic. Here, in addition to hysterical vomiting, which may be 
independent of cardialgia and hyperalgesia of the mucous membrane of the 
stomach, hysterical eructations, hysterical meteorism, hysterical borborygmi, 
etc., have been reported. 

While the corporeal symptoms of hysteria are exceedingly variable the 

PSYCHICAL PERMANENT SYMPTOMS 

on the whole are much more uniform. We include these psychical permanent 
symptoms under the term "hysterical psychopathic constitution." I 
may limit myself, in the description of this condition, to pointing out the 
connection with the corporeal symptoms and its influence upon the latter. 
The psychopathic constitution is characterized by the following factors : the 
variation in sensation and emotional conditions and especially the tendency 
to extremes, the pathologic absent-mindedness, the pathologic increase of 
phantasy, the inclination to paramnesia, the faculty of translating emotional 
impressions into hallucinations and even into "fixed ideas," the entire ab- 
sence of objectivity and finally the abnormal suggestibility. 

It is obvious that these psychical symptoms are in intimate relation and 
are all subject to the general principle which dominates the entire symp- 
tomatology of hysteria 1 : they may all be referred to the abnormally increased 
activity of emotional impressions. That the corporeal symptoms, in many 
cases, are also due to this is plain from our preceding description. It is self 
evident from this condition of affairs that the bodily symptoms of hysteria 
are in close relation to the psychical ones. I shall only indicate a few prac- 
tical and important examples. 

The intensity of the corporeal symptoms, in many cases, depends upon 
pathologic variations of the emotions; we have indicated this dependence 
several times. Many symptoms are first produced by this variation in the 
perceptions, many are increased by any emotional irritation. On the other 
hand a marked swaying of the emotional condition, in the sense of a diver- 
sion, may cause a tenacious hysterical symptom suddenly to disappear. Al- 
most quite as significant is the role of the pathologic absent-mindedness for 
the corporeal symptoms. The hysteric is confused, i. e., inattentive in the 
sense of a defect of tenacity or concentration of attention, in so far as these 
emotional impressions are concerned. It must, therefore, not excite surprise 
if, in taking the field of vision, in dynamometric and ergographic investi- 
gations, etc., often very dissimilar results are obtained. Even the personality 
of the investigator may play a part. Janet and other authors have even 
attempted to refer the hysterical anesthesias and paralyses to pathologic in- 
attention. Although an analogy exists between the hysterical symptoms of 
absence of function and physiologic inattention, Janet's view is entirely too 
one-sided. The active causative factor which also dominates the hysterical 
symptoms of absence of function, namely the importance of the conception 
of not being able to move, or to feel, etc., is overlooked. The tendency to 
abnormal imagination and to the transformation of memory impressions is 

1 Thus it happens that in the history of the teachings of hysteria almost every 
one of the psychical symptoms that have been mentioned has been regarded by some 
author as the underlying principle of all of the others. 



1078 HYSTERIA 

particularly active in giving a report of the corporeal symptoms. The precise 
observation of the neurasthenic will not be seen in hysteria. Contradictions 
in subsequent reports and even in one and the same report are common. 
The tendency to hallucination processes we have already learned to recognize 
in considering hysterical topoalgias. We have seen that the latter are actually 
designated " pain hallucinations." In fact some authorities have gone further 
than this and have called the hysterical anesthesias "negative hallucinations." 
I need scarcely state that this is nothing more than an ingenious play upon 
words. Finally, the pathologic suggestibility in regard to the corporeal 
symptoms may be demonstrated everywhere. Often this is decisive for our 
diagnosis. We have seen that by " transference " it is possible to change the 
paralysis and the sensory disturbances from one side of the body to the 
other, that the result of the examination of the sensory and motor conditions 
is often dependent upon concealed suggestion or is altered by this influence, 
that frequently only by a concealed suggestion, contained in the manner of 
the examination, one or another symptoms is provoked. If we expand this 
conception of suggestion to the extent that it includes auto-suggestion, all 
or nearly all symptoms of hysteria become suggestive symptoms; in this en- 
ticing generalization I must, however, call attention to the considerations 
detailed at the beginning of this article. 

PAROXYSMAL SYMPTOMS 

We now turn to the paroxysmal symptoms of hysteria, therefore, to the 

HYSTERICAL SPASMODIC ATTACK AND ITS VARIATIONS 

We shall first describe the ordinary, hysterical, spasmodic attack in its 
severest and most complete phase, to a certain extent the classical type. A 
severe attack consists of the following phases : 

(1) The remote prodromes; 

(2) The immediate prodromes, the aura; 

(3) The epileptoid phase; 

(4) The phase of coordinated (in part mimical) spasms; 

(5) The delirious phase. 

The remote prodromes, as a rule, consist in a change of the disposition 
such as an increased irritability, with more or less insufficiency of motive 
for this depression. An indefinite fear is also frequent which shows its 
hysterical character from the association with corporeal accompanying symp- 
toms, such as a sensation of constriction in the throat (globus) and oppression 
upon the chest; the patient frequently complains of numbness and formica- 
tion in the hands and feet. There is often an increase of the previously 
described pseudoreflex irritability. Sometimes there is acceleration of the 
pulse or it is at least very labile, at other times there is merely a subjective 
sensation of cardiac palpitation. The stage of remote prodromes may last 
from a few hours to a few days. 

The immediate prodromes consist of the hysterical aura. Sometimes this 
is merely a sudden increase of the sensations of fear, constriction and oppres- 
sion just described. At other times the patient reports that a spasmodic 
sensation rises from the epigastrium, from the deeper parts of the abdomen, 



SYMPTOMATOLOGY 1079 

from the region of the heart or from the extremities. " Everything becomes 
numb/*' " there is a spasm all over/' " a ball rises to the head." In connection 
with the aura the patient slips to the floor ; sometimes there is an impression 
that he throws himself upon the ground. Rarely is there such an abrupt fall 
as in the epileptic attack. Injuries due to the fall are, therefore, extremely 
rare. Where these are noted in the hysterical attack they are almost always 
due to the powerful spasmodic contractions of the later stages. 

The epileptoid phase consists of tonic and clonic spasms which resemble 
those of the epileptic attack. The tonic spasms often precede the clonic, as 
is the rule in the epileptic attack. The head is spasmodically thrown to and 
fro; the eyes are commonly spasmodically closed; the angle of the mouth is 
distorted by the spasm; arms and legs are in tonic extensor or flexor spasm; 
the jaws are pressed together, rarely is the mouth wide open; the muscles 
of the trunk often present tonic rigidity. In consequence of a transitory 
arrest of respiration (usually in the inspiratory position) there is occasionally 
slight cyanosis. After from 30 seconds to a minute this tonic spasm is re- 
placed by clonic convulsions. These are at first apparently distributed over 
the entire body and, therefore, also at the first glance resemble epilepsy. 
Upon careful examination, however, significant differences are noted in regard 
to the tonic as w r ell as the clonic convulsions. Above all, the muscle con- 
tractions are not so uniformly distributed over the entire body. There is a 
certain " choice " and " coordination " even in the epileptoid phase which is 
even more distinct in the succeeding stage. The tonic trunk and head con- 
tractions are more complicated than is generally noted in epilepsy. The 
tonic spasm of the extremities brings about pos'tures which indicate that 
psychical, selective factors are operative. The " uniform " generality of the 
epileptic attack, in which, according to physiologic laws, the flexors pre- 
dominate over, the extensors, is lacking. This choice is even more distinct 
in the clonic spasm. Many authors, therefore, include this with the succeed- 
ing stage. It is especially noteworthy that the hands and arms rather present 
a coordinated tremor than an incoordinated clonic spasm. The legs often 
show kicking and stamping movements which resemble voluntary movements 
or those due to emotion. 

THE PHASE OF COORDINATED SPASMODIC MOVEMENTS 

arises quite gradually from the epileptoid stage. I designate it briefly as the 
stage of jactitation. The patient now assumes complicated positions and 
performs complicated movements which distinctly betray coordination, that 
is, selection. Very frequently these resemble greatly exaggerated forms of 
expression and are, therefore, similar to those of the next stage. Some of 
these positions and movements have a special name, on account of their 
frequency or their conspicuousness. We speak of an arc de cercle, when the 
patient arches the trunk in opisthotonus to such an extent that finally only 
the tips of the toes and the occiput or — when the head is even more strongly 
retracted — even the forehead are in contact with the floor or the bed. Quite 
as common are the contortions of the trunk, sometimes winding movements, 
at other times a rigid lateral curvature. The head is thrown about, the 
hair flies around the head, sometimes following the rotations of the trunk. 
70 



1080 HYSTERIA 

The abdominal muscles are often involved in these spasmodic contractions 
(hysterical belly-dance). The pelvis often presents coitus-like movements. 
Sometimes the entire body is thrown into the air or turned to the side. The 
face is distorted by spasmodic grimaces. The eyes are usually wide open. 
The movements of the extremities are often very vigorous: the hands are 
balled into fists, then pronated and supinated to the extreme, the arms show 
the motions of striking, pushing, shaking or throwing, now they are pressed 
to the side or upon the chest, then extended high over the head or posteriorly, 
the legs stamp upon the bed or are thrown in the air, the thighs flexed upon 
the chest, sometimes extended to the extreme, widely separated or crossed. 
The movements are so violent that now and then injuries are produced. 
Often there are inarticulate sounds. Spasmodic crying or laughing may now 
be present. The respiration is accelerated, gasping and irregular. The total- 
ity of these movements has also been designated as " grands mouvements." 

The delirious phase is a continuation of the stage of coordinated and 
mimical spasmodic movements. The separation of these two stages is quite 
arbitrary and cannot always be accomplished. At all events it is character- 
istic that in the delirious phase the spasmodic movements gradually imitate 
definite emotions and impressions and consequently are better organized, in 
contrast to the irregular and indefinite irritation of the preceding stage. 
The patients laugh, cry, scream, bite, make grimaces, etc. One of my patients 
constantly inclines to the right side and with a furious expression in her 
face bites through her shirt into the right arm until blood flows, so that 
it is necessary to protect this member by a suitable bandage. In other cases 
religious exaltation may be noted in the facial expression or there may be 
the ecstasy of love or distraction. The patient assumes theatrical postures. 
There is forward bending or praying, kneeling and the like. The patient is 
no longer confined to the bed; he jumps up, runs away or attacks his sur- 
roundings. Sometimes he appears to listen or stares into space. Often scant 
expressions betray the psychical processes from which the peculiar gestures 
of the patient result. He utters threats or cries of fear and for help; or 
he prays or preaches ; or there is murmuring, attempts to embrace those about 
him, with expressions of love. A patient of mine who had biting-attacks 
always stated in this stage "now I will bite off the head of my father-in- 
law/' He was upon strained terms with the latter. In fact, the emotional 
conditions of the extraparoxysmal, relatively normal conditions, are often 
reflected in these deliria. Very frequently the gestures and expressions are 
in immediate connection with an emotional experience which may be very 
remote, but consciously or unconsciously is still active. An attempt at rape, 
a dispute with a neighbor, an insult or a punishment, an escape from danger 
often form the guiding motive. The patient re-enacts the experience in all 
of its details. We cannot escape the impression, from the actions of the 
patient, that there is a hallucination of his real experience. Besides, his 
exclamations indicate very distinctly that not only hallucinations and active 
imaginary conceptions but also illusions and delusions appear in the delirious 
stage. The patient speaks of men, animals, ghosts, etc., which he sees, occa- 
sionally also of voices that he hears. Therefore cases of mistaken identity 
are very common. Orientation as regards space and time are altered in a 
corresponding manner. The designation " delirious " for this stage is based 



SYMPTOMATOLOGY 1081 

upon the existence of these illusions of the senses and of the hallucinatory 
conceptions in connection with the loss of orientation. The patient some- 
times does not react to questions, at other times only in the sense of the 
delirious situation; rarely is the answer correct; often the delirium continues 
and while apparently unheeding the question, nevertheless, the patient shows 
by his subsequent behavior that he has understood. The delirium may often 
be greatly influenced by skillful suggestion. 

While the epileptoid phase is very brief, the jactitory and the delirious 
phases may last for many hours. Yery frequently there is a remittent course. 
After brief intervals the coordinated, spasmodic movements and the deliria 
reappear. 

The termination of the attack is rarely abrupt; there is commonly a 
gradual decline. The patient either returns at once to his previous condition 
or for a longer or shorter time there is a sleep-like stage. Actual sleep is 
very rare. After the attack there is either complete or partial amnesia. As 
a rule the patients maintain that they remember nothing of the attack; but 
by skillful questions we may convince ourselves that at least some recollection 
is retained. Exceptionally there is even a complete and intact memory of 
the entire seizure. The partial amnesia occasionally reveals a remarkable 
" choice " of memory. 

We shall now discuss a few of the accompanying symptoms of the typical 
attack. First the question arises: Is the patient actually unconscious during 
the paroxysm? The patient answers this question in the affirmative in most 
instances and probably on account of the amnesia. We must answer a de- 
cided no to this question. The behavior during the delirious and jactitory 
stages permits of no doubt that in both of these phases psychical processes 
are in action; there can, therefore, be no question of loss of consciousness. 
But even in the epileptoid phase there are indications that stimulation of 
various kinds may modify the attack and, hence, that psychical factors are 
not lacking. We then have an important differential factor from true epilepsy 
in which loss of consciousness is characteristic. 

The pupils which during the epileptic attack are most often non-responsive 
to light always react during the hysterical attack. It has been recently 
reported that exceptionally during the hysterical seizure there may be rigidity 
of the pupil to light. In the numerous instances in which I have had an 
opportunity of observing a hysterical seizure I have never observed a loss 
of the light reflex. The examination during the attack is so exceedingly 
difficult that the reports in regard to pupillary rigidity must be accepted 
with extreme caution. The cases in which experienced observers have re- 
ported pupillary rigidity were most likely cases of hystero-epilepsy. The 
pupil usually increases in size during the attack. The tendon reflexes are 
retained. Foot clonus occurs, the cutaneous and mucous membrane reflexes 
do not vary; they may be decreased but then the condition is symmetrical. 
Quite often during the attack, and then especially in the later stages there is 
a marked increase of the pseudoreflex irritability. The reaction to pin pricks 
may be absent, slight, or increased; at all events the susceptibility to pin 
pricks during the attack does not always correspond to the condition in the 
absence of attacks. Thus we may find that a patient who has marked hyper- 
algesia when free from attacks may not react to pin pricks even of the nasal 



1082 HYSTERIA 

mucous membrane during the attack. Unilateral differences in sensation 
prior to the attack may disappear in the seizure; it is much rarer for these 
to appear during the attack. The relation of the pressure-points is also note- 
worthy. Pressure upon these occasionally increases the severity of the attack 
or may produce a new attack, or an increase of the symptoms in an attack 
that is just subsiding. In other cases pressure upon this or that point may 
abort an attack. Exceptionally in one and the same case pressure may either 
increase or control a seizure. Therefore we speak of hysterogenic and hystero- 
frenic points. The pain sense of these different pressure-points also varies 
greatly during the attack; sometimes it is decreased or disappears, at other 
times it is increased; nor is it rare for new points to appear either bilaterally 
or unilaterally. It is obvious that in all of these tests suggestion is a factor. 

The severe or " grand " hysterical attack just described does not always 
pursue the course that has been indicated here. The remote prodromes may 
be absent and it is not rare for the aura to be lacking. Further the epileptoid 
phase may be ill-defined or absent. Finally the jactitory stage and the de- 
lirious may be combined; or these two stages may alternate. It is of im- 
portance to know that the two last stages may be much shortened so that 
the attack may be limited to the aura and the epileptoid phase; at most a 
rudimentary condition of the two last stages may be present consisting of 
spasmodic weeping, laughing or sobbing. Very often the entire attack may 
be abortive, i. e., it never passes beyond the aura. Upon the whole these 
mild, shortened, or abortive attacks are much more common than the severe 
ones. The duration of these incomplete attacks is also much briefer. I have 
seen seizures which were limited to a few seconds and these were often mis- 
taken for syncope, vertigo or epileptic petit mal. 

We must now call attention to some of the important variations of the 
course. The epileptoid phase may run its course under the picture of a 
Jacksonian attack or at least as a unilateral convulsive seizure. The reason 
for this unusual course is very apparent in some cases. I am treating, at 
the present time, a patient who had a tumor of the right motor region re- 
moved which was the cause of Jacksonian epilepsy. Subsequently the patient 
developed, in addition to other conspicuous hysterical symptoms, " auto- 
mimical " spasmodic attacks which, to the inexperienced observer, bear great 
resemblance to Jacksonian epilepsy; nevertheless, on account of the great 
influence of suggestion upon the course and origin of the seizures they were 
unmistakably hysterical. In other instances seeing or hearing the account 
of a Jacksonian attack must be considered. In still other cases the unilateral 
persistence of the clonic and tonic spasms must be referred to the unilateral 
presence of definite permanent symptoms, such as a topoalgia. These in- 
complete motor attacks cannot be definitely differentiated from the localized 
(regional) muscular spasms due to permanent symptoms which, as we have 
seen, not infrequently are paroxysmally aggravated or in fact only appear in 
paroxysms. 

More remote from the typical hysterical attack are the cataleptic seizures. 
Even in the course of an ordinary hysterical attack we sometimes note cata- 
lepsy, especially in the delirious phase; passive motion no longer meets with 
resistance, no more than in kneading wax (hence the designation " flexibilitas 
cerea"), and the limbs remain for a long time in the position given to them 



SYMPTOMATOLOGY 1083 

by passive movements. These cataleptic conditions may be present alone 
almost unaccompanied by other hysterical symptoms of the attack. We then 
refer to hysterical " cataleptic attacks" Exceptionally catalepsy may be 
unilateral or limited to one extremity. There is no or but very feeble reac- 
tion to pin pricks or calls. The eyes may be open or closed. The pupils 
do not show the characteristic miosis of sleep. The cutaneous and mucous 
membrane reflexes may be decreased. The tendon reflexes are not altered. 
After the patient awakens, which may sometimes be brought about artificially 
by powerful cutaneous irritation, memory is but slightly damaged : the patient 
reports that he has heard everything but was unable to speak or to move. 
Short cataleptic attacks are rare, prolonged cataleptic conditions are much 
more common in the course of hysterical psychoses. 

Hysterical sleeping attacks, which are also called hypnoid attacks, are 
closely allied. Drowsiness commonly appears quite suddenly. If the patient, 
as was recently the case with one of mine, is unexpectedly taken with the 
seizure while he is out walking, he either falls asleep where he is or proceeds 
home in a staggering condition. The eyes are closed during the attack. A 
slight tremor of the eyelids sometimes shows that this closure is not passive 
as is the case in normal sleep. If the head is permitted to droop beyond the 
margin of the bed the eyelids, as a rule, remain closed. There is no reaction 
to calls or cutaneous irritation, but it may happen that a slight touch may 
awaken the patient, while he does not stir when called. Passive motion meets 
with no or but little resistance. Catalepsy is said sometimes to be present; 
I would include these cases with catalepsy as a sharp differentiation is im- 
possible. The pupils are contracted; they react to light but are sluggish. 
The reflexes are the same as in the cataleptic attacks. Food, which is placed 
in the mouth, is sometimes swallowed and occasionally not. The duration — 
as in the case previously mentioned — is from 15-30 minutes, but it may be 
16, 24, 48 hours or even longer. The sleep conditions lasting longer than this 
are accompaniments of hysterical psychoses. The short attacks have also 
been called narcolepsy. 1 

These sleeping attacks as well as natural sleep may be combined with 
sleep wandering (somnambulism). 2 One of my patients arose during his 
sleep, sat upon the window-sill so that his feet were hanging free, and was 
found in this condition. Others wander shorter or longer distances during 
sleep either with their eyes open or closed. The behavior of the patient often 
reveals that he is under the influence of illusions, delusions, or hallucinations. 
Suggestions are almost always effective in these somnambulistic states. The 
condition frequently resembles the delirious phase of the hysterical attack. 
In fact it differs only by its relations to sleep, that is. to a hypnoid condition. 

We may regard the hysterical somnambulistic condition as a variant of 
the hysterical spasmodic attack ; it is certainly possible to present an unbroken 
series between the complete spasmodic seizure and the hysterical somnambulis- 
tic state. The delirious phase may even be regarded as a brief post-paroxysmal 

i The attempt to differentiate these " narcoleptic " attacks from the sleeping at- 
tacks, I believe to be futile. The designation " autohypnosis " for the sleeping attacks 
is improper because it is based upon an incomplete comparison. 

2 The term somnambulism should be strictly limited to conditions which can be 
demonstrated to be in relation to natural sleep, to the hypnoid condition or to hypnosis. 



1084 HYSTERIA 

somnambulistic stage. In regard to the symptoms it does not differ at all 
from the ordinary hysterical somnambulistic state. The differentiation can- 
not be made from the unequal duration, for the duration of the ordinary 
hysterical somnambulistic condition varies within wide limits. There would 
remain then only the precedence of the earlier stages which might characterize 
the delirious stage from the hysterical somnambulistic condition. Little 
importance can be attached to this, for we have seen that any of these stages 
may be absent in the ordinary hysterical attack. If we consider, in addition, 
that in the course of the hysterical somnambulistic phase there are occa- 
sionally complete or incomplete spasmodic attacks, there must be general 
agreement that the hysterical spasmodic seizure and the hysterical somnam- 
bulistic condition are very closely related. 

COURSE AND PROGNOSIS 

At the very beginning of our discussion it is necessary to state that 
hysteria, like many another disease of the nervous system, is delective 1 in 
regard to its symptoms; by this I mean to express the fact that each indi- 
vidual case presents a choice of all of the symptoms which may arise in 
hysteria. This is the reason for the extraordinary multiplicity of the clinical 
pictures which we encounter in the course of hysteria. 

Exceptionally this choice may go so far that at least for a time but a 
single prominent symptom, for example, a topoalgia, may be present and a 
careful examination is necessary to demonstrate other hysterical symptoms. 
Such mono-symptomatic cases are met with especially in the hysteria of 
children and also, as a rule, in the earliest stages of the affection. 

The combination of symptoms due to this manner of selection does not 
remain the same in the further course of the disease, for, in addition to very 
tenacious and monotonous clinical pictures, we also observe an active change 
in the grouping of symptoms. In this respect there is no other disease of 
the nervous system that can compete with hysteria. This variation in the 
clinical picture is often associated with the spasmodic attack or with emo- 
tional conditions. Thus we may note a paralysis or tremor after a spasmodic 
attack which was not present previously. On the other hand it may be 
observed that a previous tenacious symptom may disappear after a seizure. 
The influence of emotional disturbances upon the development of new symp- 
toms and complexus of symptoms has been mentioned so often that further 
discussion is unnecessary. In regard to accident cases it is of importance 
to know that the appearance of new symptoms after trauma or a severe 
emotional condition may show a certain period of latency. Thus after trauma 
the condition of the patient may be normal for several days and even weeks, 
and then merely a slight loss of power may appear which in a few hours 
develops into a well characterized paralysis. 

The development and course of the affection must be regarded as chronic; 
on the other hand the appearance of a single conspicuous symptom is often 
acute and even peracute. The latter is especially true of the appearance of 

i I differentiate between " delective " and selective ; the former designates a choice 
varying from case to case, the latter a constant choice. 



DIFFERENTIAL DIAGNOSIS 1085 

the first manifest phenomena. In rare instances a decided tendency to 
periodicity has been noted in regard to some of the important symptoms. 

If, nevertheless, it is thought desirable to single out a few of the indi- 
vidual pictures on account of their frequency and to give them a special 
designation, the following forms must be mentioned : 

(1) The undeveloped cases; 

(2) Those characterized by some striking feature (such as paralysis) ; 

(3) Those characterized by frequent spasmodic attacks and 

(4) Those in which severe psychical symptoms predominate. 

I must repeat that these clinical pictures are often combined, or that they 
may alternate and that they by no means exhaust the abundance of the 
different varieties. 

The prognosis, 1 in so far as complete and permanent cure is concerned, is 
unfavorable. Only in childhood have I seen a definite and complete cure, 
which in some special instances has been continued to 15 years and longer. 
Sometimes it is possible, in favorable cases, to bring about an intermission. 
The prognosis of the individual hysterical symptom is much better. As a 
rule even the severest hysterical symptom finally yields to suitable treatment 
or to fortunate chance. Permanent "invalidism" in the legal sense, is ex- 
ceedingly rare as the result of the corporeal symptoms of hysteria. Never- 
theless, now and then, some symptoms are met with which do not yield to 
treatment, or, after they have disappeared, return anew. Some of the hyster- 
ical topoalgias belong to this category. 

A fatal issue, apart from the hysterical psychoses and the attempts at 
suicide, is extremely rare. But the vomiting, anorexia, and some of the other 
symptoms may endanger life and aid in causing death. Some authors have 
reported fatal cases, the result of a hysterical spasm of the glottis. Person- 
ally I am not familiar with such a case. 

DIFFERENTIAL DIAGNOSIS 

The importance of the individual symptoms, from the standpoint of dif- 
ferential diagnosis, has already been considered in the symptomatology. We 
are now concerned with the differential diagnosis of special diseases. In this 
connection the following affections must be taken into consideration : 

(a) Epilepsy. — Only the absolute loss of consciousness is decisive between 
the epileptic and the hysterical attack ; and this is present only in epilepsy. 
Caution must be enjoined in taking the patient's statement of loss of con- 
sciousness during the attack. The amnesia of the hysteric frequently simu- 
lates loss of consciousness which, in reality, is not present. In these instances 
those that have seen the seizure must be thoroughly questioned as to whether 
the patient reacts to any form of stimulation, shows no complicated or 
mimical movements, and is, therefore, actually unconscious. 

Often involuntary evacuation of urine, biting the tongue and self inflicted 
injuries are stated as the factors of the epileptic character of the attack, and 
in general this is true. But it must be added that the absence of involuntary 
evacuation, biting the tongue and injuries during the seizure do not exclude 



i Hysterical psychoses are not considered here. 



1086 HYSTERIA 

epilepsy. There are many epileptics in whom these three conditions never 
occur. Therefore only the positive factors and not the negative may be 
utilized in diagnosis. Besides it must not be forgotten that exceptionally 
involuntary evacuations of urine and especially biting the tongue (biting the 
lips somewhat more commonly) also occur in hysterical attacks. In regard 
to self-inflicted injuries only those that occur as the result of the fall come 
under consideration. Self-inflicted wounds in the later course of the seizure 
are quite common in hysteria while they are exceedingly rare as the result 
of the primary fall. 

If the physician has an opportunity of noting an attack or if the report 
of a reliable eye witness can be obtained the nature of the convulsions should 
be carefully investigated. Mimical spasmodic movements are foreign to the 
epileptic attack, coordinated convulsions are very rare, and even should they 
be present they are exceedingly monotonous and simple (rubbing, scratching, 
striking, etc. 1 In the hysterical seizure these mimical and complicated con- 
vulsions are never entirely lacking. This is also the reason why the hysterical 
spasm is much more variable in the same patient than is the epileptic seizure. 
I do not attach great importance to the reaction of the pupil for the reasons 
previously stated. The transitory presence of Babinski's sign, immediately 
after the attack, is in favor of epilepsy. 

Examination during the interval free from attacks often reveals important 
points of support. The characteristic corporeal and psychical factors of 
hysteria, the hysterical stigmata, are never entirely absent during the interval, 
while in epilepsy analogous symptoms are never present. Only pressure- 
points are occasionally found in epilepsy, and exceptionally, especially in 
dementia epileptica, general analgesia. Among the psychical symptoms an 
acquired, progressive defect in intelligence is always in favor of epilepsy ; 
only we must be quite certain that this defect in intelligence is truly acquired 
and that it is progressive. A stabile, congenital lack of intelligence may be 
present in hysteria ; the latter affection not infrequently develops as the result 
of congenital weakness of mind, especially of debility. 

Finally it must be stated that sometimes, in the course of genuine epilepsy, 
hysterical attacks and other hysterical symptoms and, vice versa, in the course 
of genuine hysteria, typical epileptic attacks may occur. A gradual trans- 
formation, for example, an infantile epilepsy into hysteria may also take 
place in exceedingly rare cases. In all of these combined and transformed 
cases we refer to hystero-epilepsy. 

(b) Chorea. — The choreiform movements of hysteria may very closely 
resemble those of chorea minor. Hysterical choreiform movements are more 
often unilateral than those of chorea minor, but this is not sufficient for a 
differential diagnosis as the movements may also be most prominent upon 
one side; I have even met with pure unilateral cases. Increase of the chorei- 
form movements upon intended innervation may often be demonstrated in 
both forms. It is by no means constant in chorea minor. Increase of the 
movements under emotional conditions is common to both affections. Of 
more importance is the influence of open or concealed suggestion (pressure 
upon the pressure-points) in the differential diagnosis: as a rule, this is slight 

1 Post-epileptic somnambulistic conditions are not included here. 



DIFFERENTIAL DIAGNOSIS 1087 

in chorea, in hysterical choreiform movements it is often marked. Of more 
importance is the type of the movement upon accurate observation: in chorea 
in addition to combined movements there are also isolated ones as of a toe, 
finger, etc. ; the movements also take place in much greater variation without 
connection in the most varied parts of the body; in hysterical chorea there 
is a tendency to an almost rhythmical repetition of the same movements and 
especially of combinations of these. In the history, a preceding infectious 
disease, or an acute endocarditis, is in favor of chorea. The association with 
an emotional disturbance is not absolutely in favor of hysteria, as this may 
also be an accidental factor in chorea minor. Imitative development decid- 
edly points to hysteria. The demonstration of other hysterical symptoms is 
naturally of great significance, but it must always be borne in mind that 
chorea is particularly liable to develop in hysterical persons. 

(c) Neurasthenia. — Well developed cases of both diseases cannot be con- 
founded with one another. Apart from the pressure-points, which appear in 
both affections, all of the symptoms, especially if they are well developed, 
may be easily differentiated. Caution must be exercised so as not to confound 
the rare hypochondriac attacks due to fear and hypochondriacal impressions, 
which occasionally arise in severe forms of neurasthenia, with hysteria; for, 
here, sometimes very bizarre movements appear which resemble the hysterical 
attack. On the other hand the differential diagnosis may be exceedingly 
difficult in the undeveloped cases. It can only be stated that regional sensory 
disturbances (but not regional topoalgias), unilateral pressure-points, marked 
sensations of constriction and oppression, and a great variation in the mood 
favor hysteria; pathologic exhaustion and pathologic irritability favor neuras- 
thenia. It must, however, be admitted that there are many transitional forms 
which have been called lnjsteroneurasthenia. Many cases of traumatic neurosis 
belong to this category. 

(d) With Organic Diseases of the Nervous System. — In regard to this 
differential diagnosis I must state that the question should never be put: 
organic disease or hysteria ? but always : organic disease, hysteria, or organic 
disease -f- hysteria ? It is very common for hysterical symptoms to be " super- 
imposed " upon an organic disease of the nervous system. 1 This possibility 
must always be considered in the diagnosis. The demonstration of a few 
symptoms of a hysterical nature (their capability of being influenced by sug- 
gestion) is never sufficient to make a diagnosis of hysteria, but a complete 
examination of the nervous system must be undertaken and each symptom 
investigated as to its hysterical nature. Very often, in this manner, reflex 
pupillary rigidity, chocked disk or optic neuritis, the absence of a tendon 
reflex, etc., will show that in addition to hysteria a severe organic disease 
is present. 

The following organic diseases often bear a resemblance to hysteria : 
Multiple Sclerosis. — Exaggerated tendon reflexes are present in both affec- 
tions. The contractures of multiple sclerosis resemble those of hysteria, but 
develop more gradually. Foot clonus is sometimes simulated in hysteria as 
well as patella clonus. The intention tremor of multiple sclerosis is occa- 

i These hysterical symptoms may have been present prior to the development of 
the organic disease, but the latter is often the causative agent of the former (thus 
in hereditary cases, but by no means exclusively). 



1088 HYSTERIA 

sionally present in hysteria so that confusion is very likely to occur. If there 
is an undoubted Babinski reflex — I understand by this, that upon stroking 
the sole of the foot a dorsal flexion of the great toe, which must not be too 
rapid, without dorsal flexion of the entire foot and of the other toes takes 
place — is decidedly in favor of multiple sclerosis. The examination of the 
eye-ground is of particular importance, for optic neuritis is often present in 
multiple sclerosis; in hysteria the examination is negative; exceptionally a 
pseudo-optic neuritis may produce difficulties. The presence of dissociated 
paralyses of different nerves is very important: this is greatly in favor of 
multiple sclerosis. This is especially true of isolated paralyses of the ocular 
muscles. A history of genuine diplopia or its presence upon examination, 
therefore, favors multiple sclerosis, while in hysteria there is monocular 
diplopia and polyopia but not true diplopia. Feebleness of convergence may 
produce diplopia in hysteria, but this diplopia differs according to Parinaud 
from the organic variety in that it does not increase upon lateral movements. 
Well developed acquired nystagmus is present only in multiple sclerosis al- 
though a resemblance to the symptom may sometimes be noted in hysteria. 

The same differentio-diagnostic considerations are important in gumma 
of brain syphilis. Here we must search for cranial nerve paralyses, pupillary 
rigidity and changes in the eye-ground. The history of a syphilitic infection 
is not sufficient to make a diagnosis of cerebral syphilis. In regard to the 
differential diagnosis from dementia paralytica I shall only remark that in 
dementia paralytica there are sometimes well developed superimposed hyster- 
ical symptoms; thus the paralytic disturbance in speech may act as the agent 
provocateur for hysterical stuttering, etc. 1 To obtain a positive decision, in 
some few cases, between cerebral syphilis and hysteria and between dementia 
paralytica and hysteria, I found it necessary to resort to lumbar puncture: 
both organic diseases — at least at the onset — show a pathologic lymphocytosis. 

The differential diagnosis from brain tumor depends upon the ophthal- 
mascopic examination of every case "no matter how functional the condition 
may appear." This is obviously not sufficient in those cases which do not 
cause chocked disk. But these tumors are characterized by the severity and 
tenacious nature of the headache in addition to heaviness and insomnia. 
Slow pulse is also in favor of tumor and against hysteria. 

Focal diseases of the medullary layer and of the internal capsule may 
present difficulties in diagnosis. In this connection I must reiterate that a 
strict, typical hemianopsia does not occur in hysteria. In regard to hemi- 
plegia and hemianesthesia we have already learned to recognize the. factors 
which permit of a differentiation whether they are organic or of hysteric 
nature. In addition I must add the following: if an apoplexy, followed by 
hemiplegia, occurs in connection with an emotional disturbance, such as 
fright, hysterical hemiplegia should be thought of first, but I must remark 
that occasionally, although very much more rarely, fright may produce 
cerebral hemorrhage and cerebral thrombosis; I have seen a number of un- 
doubted cases of this kind. The paralysis betrays its organic origin by the 

i The development of dementia paralytica in a hysterical person is by no means 
uncommon. Naturally there is no connection between these affections but it may be 
readily noted how the hysterical symptoms are gradually replaced by the organic ones 
and finally substituted for them. 



DIFFERENTIAL DIAGNOSIS 1089 

involvement of the so-called muscles of predilection, exaggerated tendon re- 
flexes, Babinski's sign, modification of the patella tendon reflex, and finally 
by the gradual development of characteristic contracture and the implication 
of the muscles of the mouth supplied by the facial nerve. The sources of 
error and the exceptions have already been indicated. Somewhat more diffi- 
cult is the differentiation of organic from hysterical hemianesthesia. The 
former usually increases distally. The peculiarities of the boundaries of 
hysterical hemianesthesia must also be considered. Hemialgia, so as to make 
the point quite clear, also occurs in organic hemianesthesia, such as is due to 
foci of softening in the posterior areas of the internal capsule. 

Among the affections of the spinal cord, dorsal transverse myelitis is the 
one which is most likely to be mistaken for hysterical paraplegia. The con- 
dition of the tendon reflexes cannot, very often, be utilized, as the comparison 
with the opposite side gives us no points of support and a bilateral increase 
is often present in hysteria. Babinski's reflex is decisive and in favor of 
myelitis. Determining the rrmer limit of the sensory disturbance often gives 
us valuable aid. In lumbar myelitis the absence of the knee jerk and of the 
Achilles tendon reflex is sufficient for a diagnosis. 

Great difficulties may arise in the case of syringomyelia, if, as sometimes 
happens, paralyses and muscle atrophy, which would at once reveal the organic 
nature of the malady, are absent as well as trophic disturbances. A thorough 
investigation of the sensory condition is then necessary. The more the lim- 
itation of the sensory disturbance corresponds to the areas of the spinal cord 
roots or the spinal cord segments, the more likely is syringomyelia. A very 
marked dissociation of sensation — abolition of the pain and temperature 
senses with retained contact sense — is in favor of syringomyelia, but excep- 
tionally very similar dissociation is present in hysteria. 

The characteristic limitation of the sensory disturbance is decisive in the 
case of peripheral neuritis, provided the diagnosis cannot be made from the 
involvement of the motor fibers, the degenerative paralysis, and decrease or 
absence of the tendon reflexes; besides in hysteria the characteristic sen- 
sitiveness upon stretching the nerve trunks (Lasegue's symptom) is lacking. 
We have already considered the differentiation of true neuralgia from hyster- 
ical pseudoneuralgia. 

(e) With Diseases in the Vicinity of the Central Nervous System. — 
Meningitis and its varieties must be considered. Among the symptoms of 
the latter affection the rigidity of the neck, the headache, the vomiting, the 
delirium, and the convulsions, may be imitated by hysteria. The absence 
of fever and an irregularity of the pulse as well as paralyses of the basal 
cranial nerves is sufficient for a differential diagnosis. In addition the 
hysterical delirium differs in being more connected and also by its emo- 
tional admixture. As a final diagnostic resort lumbar puncture may be 
employed. 

Among diseases of the spinal cord care must be exercised in differentiating 
diseases of the vertebra, such as the tuberculous with hysterical rachialgia. 
The pain in the latter is even more severe than in the former. Contact and 
passive motion, which do not involve the vertebrae that are affected, are asso- 
ciated in hysterical rachialgia with active pain. On the other hand in hyster- 
ical rachialgia the characteristic girdle root pain is absent. The regional 



1090 HYSTERIA 

hyperalgias, which often accompany hysterical rachalgia, must not be for- 
gotten. 

(/) With Diseases of Other Organs. — The regional topoalgias give suffi- 
cient cause for mistakes of this kind. First the articular topoalgias must be 
considered. I advocate a minute surgical examination in all of these cases. 
If there be any doubt an X-ray examination is necessary. If there be any 
suspicion of tuberculous joint inflammation careful temperature records are 
necessary. I am in doubt whether there are a greater number of reckless 
operations performed on account of an inconsiderate diagnosis when pure 
hysterical topoalgia is present or whether the necessary operation is omitted 
more frequently from a hasty diagnosis of hysterical topoalgia. For this 
reason alone a minute surgical examination is required. If no surgical affec- 
tion is present the well known characteristics of hysterical topoalgia, as a 
rule, are sufficient for a correct diagnosis. Other hysterical topoalgias may 
simulate gall stone disease, a gastric affection, angina pectoris, a peritonitis, 
etc. In these instances all of the diagnostic aids known to internal medicine 
are to be invoked, and only then should a diagnosis of hysterical topoalgia 
be made by exclusion. I cannot enter upon a minute differential diagnosis 
of these conditions, they will be found in the respective chapters of this work 
dealing with the separate maladies. 

There is often the greatest difficulty in the differentiation of hysteria 
from malingering. The hysteric impression of " not being able to walk " 
cannot be so readily discerned from the " not being willing to walk " of the 
malingerer, as it might appear. It finally depends upon this: whether or 
not in the occurrence of the symptom under consideration a conscious con- 
ception has been active from which advantage is to be expected. Where this 
is the case an attempt at aggravation is the motive. If the conscious con- 
ception which produces the symptom, from which an advantage is expected, 
is the only cause of its presence, the case is one of simple malingering. 
Practically when we are considering the question " simulation or hysteria " 
we must investigate whether the report of the patient is contradicted by the 
actual psychic condition present, for example, whether he maintains, against 
his better knowledge, that he cannot walk, etc. I cannot describe the different 
methods which are in use to obtain this knowledge. Most of those in vogue, 
to unmask simulated disturbances in sight and hearing, cannot be employed 
in this condition. If we determine, in the well known manner, that a person 
who simulates right-sided deafness nevertheless hears upon the right side 
while we make him believe that he is hearing with the left ear, whereas the 
sound wave actually strikes the right ear, this argument would not be ap- 
plicable in hysteria, for here the entire disturbance is dependent upon im- 
pression : a hysteric who suffers from hysterical deafness upon the right side 
must, if he is able to appreciate sound waves in the right ear, hear these, 
although he may believe that only the left ear is capable of appreciating 
sounds. It is necessary to point out that simulation of the entire pathologic 
picture can never be demonstrated from the proof of simulation of a few 
symptoms. Exaggeration and aggravation are so common in hysteria that 
we must always reckon upon a combination of actual and simulated symp- 
toms or with an exaggeration of actual symptoms. We may only speak of 
complete malingering when we are able to demonstrate that each separate 



TREATMENT 1091 

symptom is simulated. Sometimes the exaggeration itself has a pathologic 
character. I need only refer to the pathologic distorted simulation in Gan- 
ser's hysterical somnambulistic condition. 

Let us assume that we have made a diagnosis of "hysteria/' it now be- 
comes necessary to develop a plan of treatment. 

TREATMENT 

The therapy of hysteria is dominated by two main principles, the etiologic 
and the psychotherapeutic. 

The etiologic principle requires counteraction of the causes of hysteria 
which is, at the same time, the method of prophylaxis. This primarily re- 
quires, as the hereditary predisposition as such cannot be eliminated, a proper 
education in the threatened, but not yet affected, child and often enough also 
in the diseased adult, so as to obliterate deleterious influences. Above every- 
thing the child must become accustomed to control its feelings and emotions, 
especially the negative ones. This is in consonance with our general inter- 
pretation of hysteria, of which, according to our description, the abnormal 
action of exaggerated emotional conceptions is characteristic. Translated 
for practical purposes this simply means that the child must not be indulged, 
that its little pains and aches should be ignored, that it must be callous to 
such irritants as slight pain, hunger, cold; it must be taught to subjugate 
its will and convenience to that of others and learn to observe foreign inter- 
ests. Even in married adults there is too great neglect of this requirement, 
and an elimination of these deleterious influences of education, that is, the 
behavior of those in contact with the patient, is a prerequisite, in these cases, 
for therapeutic success. It is self-evident that this process of " hardening " 
must not be overdone, above all, it must be methodic and uniform. It is 
of the greatest importance that the parents set an example to the child in 
the control of their emotions. From this point of view it will be readily 
understood that such children are ordered cool ablutions, etc. The essential 
element is the control of the emotional state, not the hardening of the body. 
Gymnastics, swimming, skating, etc., have a similar purpose: the child must 
learn to control its fears of slight danger. 

In another direction also education is capable of removing an etiologic, 
deleterious factor. It is very significant that not emotional sensations, but 
above all exaggerated, emotional impressions are of pathologic influence in 
hysteria. Pathologically increased activity of the imagination, we have seen, 
is a chief symptom of the hysterical, psychopathic constitution. Here also 
education must step in. A child, that is threatened or already attacked by 
hysteria, should not be permitted to read books that stimulate the imagina- 
tion to any considerable extent, whether these be fairy tales or Indian stories. 
The hysterical adult, for the same reason, should not be permitted to read 
exciting novels. On the other hand, it is very good practice to accustom the 
child to observe phenomena of nature. It may build with blocks, draw, col- 
lect stamps, stones, plants, insects, etc. ; cultivate the garden, have an aquarium 
and so on. These pastimes may also be followed by adults. All spare time 
is to be devoted either to rest or to objective observation. Hand-work is not 
suitable for it allows the patient to lose himself in phantasies and broodings. 



1092 HYSTERIA 

Translating and abstracting is much better employment. Play and associa- 
tion with others is very effective protection against the egocentric limitation 
of the hysterical life of impressions. Cultivation of the habit of nature 
observation upon walks is particularly important, for upon such occasions 
there is most likely but little opportunity for the patient to indulge in useless 
dreamings. The theatre is to be prohibited. It is also obvious that public 
schools, in general, are to be preferred to private instruction, for the former 
gives a much better opportunity for association and the necessary process of 
hardening. 

Among the other etiologic factors of hysteria there are none that require 
special treatment. Therefore we may be brief, and but a few points need 
be discussed. If there is a severe, nutritive disturbance Weir Mitchell's 
treatment may be employed. Should a chronic intoxication be an etiologic 
factor this must naturally be combated. The question will often arise in 
the female whether the treatment of a genital affection is not indicated from 
the stand-point of etiologic therapy. In the discussion of the etiology it 
was expressly stated that gynecologic affections are not of essential importance 
as factors in the etiology. This coincides with the experience that only very 
rarely does gynecologic treatment produce an essential or lasting improve- 
ment not to speak of a cure in hysteria. Even in the few instances where 
improvement has followed operation it is probable that the operation merely 
had a suggestive effect. On the other hand, there are many cases in which 
gynecologic treatment, especially if long continued (such as internal mas- 
sage), has materially increased the hysterical symptoms. Urgent indications 
for gynecologic management must of course be fulfilled in spite of the 
existence of hysteria, but hysteria neither indicates castration nor any other 
gynecologic treatment; on the contrary, caution is necessary and no gyne- 
cologic measures that are not urgent should be attempted. Only when it has 
been determined, after rigid investigation, that the gynecologic symptoms 
are regularly and markedly aggravated from the presence of some genital 
malady (such as retroflexio uteri) is it advisable to treat this malady even 
without urgent indications. Not with the expectation of curing the hysteria, 
but only with the view of relieving a factor which might hinder the treat- 
ment. If at all possible a rapid method should be employed, perhaps even 
operation (such as ventrofixation of the uterus), for the irritative effects of 
chronic treatment are not without serious consequences. There should be 
no unnecessary gynecologic examinations, particularly in the case of virgins, 
for this very often brings about a decided aggravation of the symptoms. 

The question often arises whether emotional conditions, brought about by 
occupation and the affairs of daily life having a tendency to keep up the 
malady, cannot be removed. Sexual relations, dissatisfaction in the occu- 
pation, marital difficulties, etc., come under this head. The "process of 
hardening," to which we referred previously, is too late for these cases. Here 
the physician must decide each individual case according to its merits, and 
after a minute investigation and after having exhausted all other methods 
of treatment must explain to the patient and his friends the chances of a 
change in occupation, etc. Only from my own experience I should advise 
against a too sanguine opinion in this respect. 

In this connection the very important question arises whether hysterical 



TREATMENT 1093 

persons "may" marry. It is an undoubted fact that marriage often has 
an unfavorable effect, that the hysterical symptoms increase and that the 
hystero-psychopathic constitution leads to the greatest marital misfortunes. 
It is rare that a favorable influence is noted. From this condition of affairs 
it is not justifiable to advise all hysterical persons against marriage. In 
fact the physician is not to " advise," but to state quite objectively the dan- 
gers of marriage for the patient and for the possible offspring. In weighing 
these dangers not merely the severity of the symptoms but also the entire 
external situation in the contemplated marriage, the personality of the hus- 
band, etc., are to be taken into consideration. 

If the outbreak of the manifest, severe symptoms is associated with a 
special emotional shock, such as relates to the sexual sphere, which acts in 
the sense of a latent, exaggerated, memory picture, the patient should be 
permitted, at the first examination, to explain the entire emotional cause as 
fully as she desires. This is sometimes an absolute requirement for a cure. 
By continued questioning and a repetition of the same answers, as advised 
by Freud and Breuer, so that all of these latent memories are refreshed, 
very little good can be gained ; I have repeatedly observed that the " hyper- 
imagination " of the patient constantly enlarges the original experience, 
sometimes even in the most dangerous manner, without decreasing the emo- 
tional emphasis of the memory and without improving the other hysterical 
symptoms. 

After these etiologic therapeutic requirements have been fulfilled and all 
deleterious factors have been removed in so far as is possible, treatment in 
a positive sense is to be instituted. These positive' measures of treatment 
are almost exclusively psychotherapeutic. In our previous discussion, in re- 
gard to the removal of deleterious factors in the rearing of the child, we 
have discussed a few of these curative factors. The first practical question 
which must be put in every case is whether the patient may remain in his 
previous surroundings. Very often the reply to this question must be in the 
negative. The patient must be placed under favorable psychical conditions. 
For this purpose a visit to trustworthy relatives or friends, or — in the case 
of youthful hysterics — a suitable pension. In severe cases the patient should 
be sent to an institution, so that the psychotherapy may daily and hourly be 
under the direction of a specialist in nervous diseases. Such institutions are 
private and public hospitals for nervous disease, psychiatric and nervous 
clinics; but general hospitals are available only in so far as the directing 
physician is familiar with nervous diseases and psychotherapy. In rare cases, 
under especially favorable circumstances, isolation may be attempted at home, 
but my experience in this direction is not very favorable. In many instances 
the change of residence cannot be considered for pecuniary reasons. Secondly, 
the question must be considered whether the attending physician should carry 
out the treatment himself. The answer to this question depends upon whether 
the physician is conversant with nervous disease and psychotherapy and 
whether he possesses sufficient authority in the special case. It is undoubted 
that the family physician often has less influence, because the patient is 
accustomed to him, than a strange physician. Very often incidental qualities 
of the physician are of the greatest importance in obtaining this psychical 
influence over the patient. It would be a great mistake to attempt to carry 



1094 HYSTERIA 

the treatment on in a severe ease in the absence of this personal influence. 
It is quite remarkable how often a change in physician brings about sur- 
prising improvement in the hysteric'. The " cedo meliori " in such cases is 
nothing of which the physician need be ashamed. Thirdly, in severe cases 
the nurse is of the greatest importance, for the personality of the latter is 
often of vital importance upon the result of treatment. Naturally the indi- 
vidual circumstances are decisive in the choice of a nurse, but in general it 
may be stated that above all absolute conscientiousness in carrying out the 
orders of the physician is the first requisite. Nurses who allow themselves to 
be controlled by the patient and who become intimate with their charges are 
usually not to be trusted. 

After these practical, preliminary questions of psychotherapy have been 
settled a proper manner of living is ordered for the patient in which all of 
the etiologic indications are considered so far as possible. In severe cases 
it is best to give a written, hourly plan ; regular hours for bodily and mental 
occupation and for rest. In the choice of employment the principles devel- 
oped above are decisive. Yery often under this plan of treatment the malady 
improves greatly without other curative measures. But it is often necessary 
to treat special symptoms, and this treatment is largely suggestive. Whether 
the vehicle of suggestion be a drug, a mechanotherapeutic, electrotherapeutic, 
or hydrotherapeutic procedure, it remains the same in principle. It depends 
merely upon the suggestive factor and this again is entirely dependent upon 
the suggestive authority of the physician who orders the drug or the process. 
It is self-evident that a vehicle is chosen that has not already been employed 
unsuccessfully in the special case. The more educated the patient the more 
it is necessary to employ concealed suggestion. Naturally a pill may be or- 
dered or the electric current or massage employed and the physician declare 
in an authoritative tone : " this will cure the paralysis " ; however, such a 
crude suggestion will not be effective in the case of an educated person. 
Therefore, a more cautious method must be utilized; for example, without 
previous preparation the paralyzed muscles are caused to contract by the 
electric current, and then very casually the action of the current should be 
emphasized. If there is no actual paralysis but merely paresis, skillful exer- 
cise treatment, in the sense of suggestion, should be employed. It is of ad- 
vantage to test the muscular power by the aid of the dynamometer from 
time to time without concealing the differences from the patient. In paresis 
of the legs and abasia " walking-frames " may be employed. I have often 
used these exercises with success even in absolute paralysis. In such instances 
I have previously stated to the patient : " Naturally your exertions will not 
show immediate motion, but it is of great advantage if the paralyzed muscles 
are stimulated at all; they gradually become strengthened and some day 
motion in the part will appear." Passive gymnastics and resistance gym- 
nastics are often serviceable in the sense of suggestive therapy. Contractures 
with and without accompanying paralysis often present great difficulties in 
suggestive treatment. Here passive gymnastics as well as electricity and 
massage of the antagonists of the contracted muscles is of use. Prolonged 
baths with exercises (in the bath) are sometimes valuable. Forced over- 
powering of the contracture and immobilization in a new position should 
not be practiced as bandages of this kind often produce contractures in 



TREATMENT 1095 

hysterical persons and we merely risk a new contracture in another position. 
The artificial production of a hysterical spasmodic attack — in the hope that 
after the attack the contracture may disappear — I utilize only in the most 
desperate cases. 

Inhibitive exercises are of use in combating the various forms of spasm. 
It would be quite superfluous to indicate the suggestive remedy for each 
individual hysterical symptom. In the text-books and special monographs 
countless drugs, electric, hydriatic, and other measures will be found. The 
vehicle of suggestion in itself is never decisive. I shall explain this by an 
example of hysterical vomiting. Sometimes the use of a drug will relieve 
the condition. Whether this be sugar, chloral, bismuth or an ice pill is 
immaterial if success is attained. If the medicine fails relief may be obtained 
by suitable diet. If this also proves useless, massage, the thermophore or 
faradization of the gastric region may be tried. Gastric lavage used once or 
several times is sometimes effective. Pretended operations, which have occa- 
sionally been used for this or that hysterical symptom, had better be avoided. 
In some very tenacious cases I have prohibited food until 1 o'clock in the 
afternoon, and then, at 1 o'clock, when a — very scant — meal was given the 
patient it was not vomited. In another patient the vomiting ceased when I 
had her fed in the bath. 

In addition to the suggestive therapeutic methods that have been described 
there is also the so-called "harsh" method. This consists in the physician 
suddenly giving an energetic command to the patient which relieves the symp- 
tom at once. For instance, the abasic patient is commanded, in a harsh 
tone, to walk. On the whole I have had very little success with this method. 
It is perhaps most suited to the hysteria of childhood. 

Another method, in a certain sense the direct contrast to the previously 
mentioned one, is more effective: deliberately ignoring the symptom that is 
to be treated, a "purposeful neglect." I utilize this method by first exam- 
ining the patient very carefully, then give a plan of treatment for each hour 
and then ignoring all of the symptoms for weeks. At the onset I usually 
state to the patient " the treatment must now continue for some weeks with- 
out change in the manner just outlined, in spite of any of the symptoms; 
rapid improvement is not to be expected, but only this method will prove of 
benefit; frequent examinations and complaints are, therefore, without pur- 
pose." Exceptionally I may add that the patient should aid in ignoring 
the symptoms until the treatment begins to be effective. It is rarely advis- 
able to go further than this and to reject all forms of treatment, at most 
to give a plan for each hour or directions as to employment and the manner 
of life, ignoring all of the other details. It is self-evident that this method 
must also be followed by all of the relatives, friends and nurses and — in the 
hospital — by the other patients, otherwise there will be absolute failure. In 
the hospital I generally isolate these patients. Dejerine has even developed 
this " isolation method " still further. 1 This process of ignoring must also 
be required of those about the patient in cases in which the physician em- 
ploys the suggestive method and not the medical method of ignoring. Exag- 

i Here other curative factors also come into consideration, such as avoiding irri- 
tation, etc. 



1096 HYSTERIA 

gerated sympathy and care on the part of parents often serves to increase 
the pathologic, hysteric impressions. We have, therefore, seen that removal 
of the patient to other surroundings is a necessary measure for cure. Now 
the method has been described in the treatment of those cases in which this 
requirement cannot be fulfilled; we must then require of those about the 
patient that the process of ignoring be strictly maintained. This need not 
bear the character of lack of sympathy: brief, not too frequent, sympathetic, 
but quiet inquiry of the patient's condition may continue; I often permit 
the relatives to say to the patient about as follows : " the physician has as- 
sured us that there is no danger, but he has said that we must be prepared 
for a long illness and that it will take some time before the first signs of 
improvement appear; he has, therefore, forbidden us to inquire daily in 
regard to your condition; he will inform us himself." It is understood that 
this treatment of ignoring on the part of the physician and the friends must 
be continued even if there be at first an increase of the symptoms, spasmodic 
attacks, etc., after the method has been tried for a short time. 

This method unquestionably very often bears good fruit. The question, 
therefore, arises when should the suggestive method be used and when the 
method of ignoring? If there is actual danger of suicide or a very con- 
spicuous tendency to hyperphantastic illusions there is an absolute contra- 
indication to the latter method. In fact, in the case of a very pronounced 
hysterical, psychopathic constitution or with a fully developed hysterical 
psychosis, we must be exceedingly cautious with treatment by the method of 
ignoring; it must be either dispensed with entirely or at most greatly modi- 
fied. But the treatment may be employed in the milder form, previously 
described, in all cases in which the suggestive method does not produce im- 
mediate results. In these milder forms the method of ignoring is constantly 
associated with suggestive factors, and the plan of treatment in regard to 
hourly employment is persisted in, which on its part depends entirely upon 
suggestive effects. The only difference consists in this, that the suggestive 
effects of the hourly plan are not constantly urged, but on the contrary are 
combined with the psychical influence of the purposeful ignoring. I believe, 
therefore, that the methods do not exclude one another, but according to the 
varying conditions in the individual case must be combined. 

Up to this time I have purposely avoided the mention of the special treat- 
ment of the hysterical, spasmodic attack. Here also all of the therapeutic 
factors which we have mentioned as valuable for the permanent symptoms 
are in place. Exceptionally the attack may be surpassed by gruffness. A 
suitable suggestion has a better effect. This, as we have seen previously, 
may be connected with one or the other hysterofrenic pressure-points. Cool 
ablutions or the faradic brush may be tried in the same sense; but here the 
gruffness is more important than the suggestive factor. These measures must 
never have the appearance of punishment. In most instances ignoring the 
attack is at the same time the best form of treatment. We only interfere 
if in the jactitory or delirious stage the patient threatens to injure himself, 
and after having reassured the friends as to the harmlessness of the attack, 
they are sent into another room. It is unnecessary to administer drugs. 
Hypodermics of morphin are absolutely contraindicated. 

In case these general measures in treatment are insufficient to bring about 



TREATMENT 1097 

the disappearance of the attacks, I believe it to be good practice to relieve 
the emotional irritability, which favors the appearance of attacks, by suitable 
drugs. The bromids are especially useful in this respect, given in small 
doses. Large doses, such as are administered in epilepsy, are not merely 
superfluous, 1 but are likely to produce bad results, for hysterical persons not 
infrequently react to large doses of the bromid with delirium. Doses of 1-2 
grams of sodium bromid are given to reduce the emotional irritability, which 
is very likely to control the attacks and thus aid the other curative measures. 
Morphin and other narcotics are absolutely prohibited, even in a prophylactic 
sense, for hysterical persons are exceedingly apt to become habitues. 2 

Finally, at the conclusion of our therapeutic considerations it will be 
asked whether hypnotism, that is suggestion during hypnosis, is to be em- 
ployed in treatment, as is maintained by so many professional hypnotizers 
and unfortunately also by some physicians. 3 I must state, briefly, that hyp- 
notism should only be considered as an vltimum refugium. Hypnotism, no 
matter what the professional hypnotizers may state, is often directly harmful. 
The special directions which are supposed to render hypnotism harmless are 
to be regarded as humbug. In children hypnotism is not to be permitted 
under any circumstances. Only after all of the other methods of treatment 
with which we have become familiar, after conscious employment have failed, 
may hypnotic suggestion be considered; it is then urgently necessary to state 
to the friends that the method is by no means harmless, that mental disturb- 
ance or new symptoms may arise in connection with the hypnotic treatment. 
The more I see of the treatment of hysteria the more I am convinced that 
hypnotism may be dispensed with in the treatment of the affection. Hyp- 
notism does not bring about what must be considered as the chief aim in the 
treatment of the malady corresponding to the underlying condition of the 
hysterical change: restitution of the psychic equilibrium by the removal of 
the tendency to pathologic, active, hyper emotional impressions. 

1 It is characteristic of hysteria, in contrast to epilepsy, that large doses of the 
bromids do not decrease the number of attacks, at least not more so than do small doses. 

2 For the same reason I prohibit the use of alcohol entirely. 

3 These persons always maintain that " their " hypnotism is never harmful and 
has never injured anyone. 



TRAUMATIC NEUROSES 
By P. SCHUSTER, Berlin 

I. HISTORY 

At the beginning of this article a definition of the term traumatic neu- 
roses may, as a matter of course, be expected. But, if we review the historic 
data which mark the development of our pathologic conception of traumatic 
neuroses, it soon becomes evident that a definition cannot be given in a few 
words, therefore we at once turn to the historic facts. A study of the history 
of traumatic neuroses cannot be omitted, being both interesting and in- 
structive; interesting because the development of the pathologic picture of 
traumatic neuroses largely represents the total development of neurological 
science within the last decades; instructive because the development of differ- 
ential diagnosis in almost every individual case is a miniature reflection of 
all development of pathologic conceptions. Therefore in the diagnostic on- 
togenesis of any case the phylogenesis of the pathologic conception is always 
kept in view. 

In a work, " Upon Injuries of the Central Portion of the Nervous Sys- 
tem," etc., published in London in 1866, Erichsen first called the attention 
of physicians to certain pathologic conditions following injury, especially 
after railroad accidents. Erichsen regarded the symptom-pictures as organic, 
and believed the seat of the pathologic processes to be located in the spinal 
cord. A few years later he expressed the same view in his book " On Con- 
cussion of the Spine." To Erichsen, therefore, the designation " railway 
spine " is to be attributed. 

It was quite natural that those physicians, who, following Erichsen and 
stimulated by his publications, made a specialty of diseases occurring after 
accidents, should share and retain the views of the originator. Among his 
followers in Germany were Leyden, Erb, Bernhardt, Westphal, and Rigler 
(Ueber die Folgen von Verletzungen auf Eisenbahnen, Berlin, 1879), who 
devoted themselves to the study of the newly described pathologic picture, 
and accepted Erichsen's views. 

Modi's report in 1881 led to the adoption of a more advanced theory in 
place of the original pathological conception. This report, which deals with 
" psychical disturbances following railroad accidents," was the first to intro- 
duce a psychical element into the pathology of traumatic affections. Another 
step in the direction indicated was taken by Wilks (Guifs Hospital Reports, 
1883) and Walton (Arch, of Med., 1883), who described hemianesthesia as 
a symptom of trauma, as a rule functionally produced. In the two articles 
upon sensory disturbances which he published in 1884 (Berliner klin* 
1098 



HISTORY 1099 

]Yochcnschr. and Arch. f. Psychiatric) Oppenheim attempted to base the 
pathologic picture upon the sensory disturbances observed at the onset. In 
the latter of these articles Oppenheim (to whom Germany is indebted for 
the great advance in the understanding of traumatic nervous disease) abso- 
lutely rejected Erichsen's view of the spinal nature of the pathologic picture, 
and maintained its cerebral localization; he also brought out strongly the 
many functional features of the pathologic picture. But in 1886 Charcot 
took the most important and final step in advancing our knowledge of the 
disease by stating that the entire pathologic picture was functional and 
psychical. In 1883 Page, an English author, in the first edition of his book 
" Injuries of the Spine and Spinal Cord and Nervous Shock/' expressed 
views which extraordinarily resemble those of Charcot. Although Charcot's 
idea of the nature of traumatic diseases to-day unquestionably molds the 
views of most neurologists, yet the history of the development of our path- 
ologic conception was not completed with Charcot's teaching. Subsequently 
the view of the functional nature of the disease was generally adopted, but 
there was a want of unanimity concerning questions of secondary importance. 
An originally calm and purely scientific consideration of the question gave 
way to a remarkably warm polemic, which was not always confined to the 
subject. The discussion began about 1890, and the most important point 
was no longer the general medical one, but the special neurologic cause of the 
pathologic symptoms. 

Charcot in his " New Lectures upon Diseases of the Nervous System " 
considered the nervous symptoms arising after trauma as purely hysterical. 
He therefore did not assign to these symptoms any special nosologic im- 
portance. Oppenheim's celebrated treatise, which appeared in 1889, was 
based upon a diametrically opposite view. In this he absolutely maintained 
the functional nature of the pathologic picture, but he objected to the opinion 
that the " traumatic neurosis " — as he called the clinical condition — should 
be regarded as hysteria nowise different from other forms of hysteria except 
in its traumatic origin. Oppenheim's theory that the traumatic neurosis 
was a peculiar neurosis rapidly found acceptance among general practitioners, 
but there was much opposition on the part of our most celebrated neu- 
rologists. Schultze, Jolly, Eisenlohr, Mendel, and others denied the spe- 
cificity of traumatic neurosis as a disease sui generis, and claimed that, 
instead of the schematic designation " traumatic neurosis " for the existing 
symptom-complex, the designations traumatic hysteria, traumatic neuras- 
thenia, or traumatic hypochondria, etc., according to the type, should be 
substituted. Oppenheim did not absolutely deny that there was truth in the 
objections of his opponents, but he strongly emphasized that the peculiarity 
of his pathologic picture of traumatic neurosis was the peculiar admixture of 
symptoms which originated entirely from this neurosis. On this point com- 
plete unanimity of opinion has never been attained. I believe, however, that 
the views enumerated above are not so diametrically opposed as discussion 
would sometimes make them appear to be. Undoubtedly we must agree with 
Oppenheim that many cases of the affection may be differentiated from non- 
traumatic cases of hysteria, hypochondriasis, etc., by certain common symp- 
toms, but these differentiating factors are non-essential, and they depend less 
upon the intimate nature of the neuroses than on external circumstances. 



1100 TRAUMATIC NEUROSES 

To illustrate this, we may briefly refer to accident insurance under the control 
of the State. The immediate and mediate psychical effects of the filing of 
claims for damages on the part of the injured, and the practical insistence 
on these claims, is primarily, although perhaps not wholly, the factor which 
gives to the clinical physiognomy of accident neuroses their definite stamp. 
One of the most important results of these psychical effects is the " concep- 
tion of indemnity" (for damages) which Striimpell brought prominently 
into the discussion. Besides the question of the specificity of traumatic 
neuroses, which is of more scientific than practical importance, in the last 
15 years practical and significant issues have been discussed; namely, the 
objectivity of the pathologic phenomena and the question of malingering. 
In the discussion of the objectivity of symptoms, sensory disturbances have 
filled an undeservedly wide space. 

In regard to simulation, which some observers regarded as unlikely or 
rare, and others as extremely common, in the course of time we have arrived 
at unanimity. To-day nearly all authors maintain the importance and fre- 
quency of exaggeration, while they admit that a close simulation of the entire 
pathologic picture is rare. 

The conviction is steadily gaining ground that the psychical genesis of 
most of the symptoms .explains why there are so few strictly objective phe- 
nomena. Among those independent of the psychical sphere are the numerous 
symptoms on the part of the vessels, conditions which — strictly speaking — 
are not actually symptoms but complications of traumatic neurosis. In 1889 
Sperling and Kronthal were the first to call attention to arteriosclerotic 
phenomena in the pathological picture of traumatic neuroses, and to demon- 
strate them by anatomical proofs. 

Friedmann (1891-93) referred a number of symptoms (marked vertigo, 
etc.) to vasomotor disturbance in the interior of the skull, and proved that 
these conditions were due to changes in the small cranial vessels and their 
surroundings. Subsequent investigations upon an experimental basis were 
made by other authors, and these made it appear likely that other symptoms 
of traumatic neuroses which are not observed in non-traumatic hysteria, 
neurasthenia, or hypochondriasis, may be regarded as organic. By an animal 
experiment performed in 1896 Schmaus showed that shock to the vertebral 
column produced small foci of degeneration in the spinal cord and changes 
in the axis cylinders. There is nothing to prevent the same demonstration 
in human pathology. It cannot have escaped attention that in applying 
Schmaus's experiment to human pathology, the same conditions are produced 
as exist in so-called commotio cerebri and commotio medullce spinalis. There- 
fore Schmaus's experiment has illuminated the clinical and pathologico- 
anatomical border-land between cerebral shock and traumatic neuroses. 

In the discussions of the Wiesbaden Congress of Internal Medicine in 
1893 there was an apparent tendency to regard certain severe traumatic 
neuroses as also due to organic change (which might be assumed from 
Schmaus's experiments) ; subsequently the same inclination was noted in 
other authors, especially Striimpell, Vibert, and Stepp. 

After this, opinion apparently drifted back to where it stood in 1868. 
In reality our comprehension of the nature of traumatic neuroses has made 
great advance in the last thirty years, as I have previously attempted to 



ETIOLOGY AND PATHOGENESIS 1101 

prove from the valuable historical data referred to. It is true our pathologic 
conception is not yet perfect. It may not be irrelevant to mention Hoche's 
opinion that the pathology of rare electric injuries may give us a further 
insight into the nature of traumatic neuroses. 

On reviewing the history of these affections, we will more minutely define 
our pathologic conception, and will state that by the designation traumatic 
neuroses we mean pathologic pictures which are either cases of pure hysteria, 
neurasthenia, or hypochondriasis due to trauma, or cases which, besides show- 
ing these neuroses, also exhibit slight organic changes, or, finally, cases which 
reveal the same combination of symptoms of hysteria, neurasthenia, and 
-hypochondriasis which is peculiar to some of the non-traumatic neuroses. 

II. ETIOLOGY AND PATHOGENESIS 

In discussing the etiology and pathogenesis of traumatic neuroses we shall 
consider only the purely functional pathologic . picture. The etiology of the 
phenomena due to organic changes (changes in the circulatory system, hemor- 
rhages into the central organs and their membranes, destruction of nerve 
substance, etc.) does not come within the scope of this article. 

As in all other diseases, in the pathogenesis of traumatic neuroses there 
are a number of causative factors, and all those predisposing agents which 
play a role in the development of other nervous diseases must here be con- 
sidered. Chief among them are chronic abuse of alcohol, chronic metallic 
poisoning (above all, lead poisoning), and a number of occupation injuries 
such as those due to working in intense heat, near noisy machinery, and 
those in which the duties are combined with great responsibility or in which 
there is rigid discipline. In addition there are a few other predisposing 
factors which are especially significant in the cases with a purely psychical 
pathogenesis; namely, the mental or intellectual inferiority of the injured, as 
well as the baneful influence of those about the patient, and the evil effects of 
instituting a claim for damages. 

As is evident from its name, the most significant factor in the etiology 
of traumatic neuroses is trauma; the more serious the accident, and the 
more extensively the central nervous organs or their coverings are implicated, 
the sooner will traumatic neurosis develop, and the more severe will the 
symptoms usually be. I emphasize " usually." For the etiology of traumatic 
neuroses depends not so much upon the objective severity of the accident as 
upon its subjective consideration by the injured person. This statement is 
based upon the fact, repeatedly emphasized, that the development of traumatic 
neurosis is generally psychogenous, but it by no means indicates that the 
pathogenesis is the same or even similar in all cases. 

Sometimes the disease develops acutely after emotions which appear and 
disappear suddenly, such as fright, fear, anxiety, and, more rarely, anger. 
These emotional conditions either accompany the trauma or immediately 
precede it. Etiologically they become most active when they are simultane- 
ously experienced by many persons, as in great theatre fires, in railroad 
accidents, in earthquakes, in school panics, etc., in which circumstances severe 
traumatic neuroses frequently arise. 

That after such violent emotions psychical shock and not bodily injury 



1102 TRAUMATIC NEUROSES 






is most active, and that not objective trauma but its subjective reflection in 
the psychical life of the injured one causes the development of the neurosis, 
is proven by hundreds of experiences. For instance, I observed the motor- 
man of an electric street car who became seriously* ill after being struck on 
the head by the conducting wire, which, it transpired later, belonged to a 
" live " wire, but no current was then on. We may thus understand that the 
etiology does not wholly depend upon actual external trauma, for this may 
apparently be absent, but that a purely psychical trauma, like psychical shock, 
may sometimes be sufficient to produce a traumatic neurosis. In addition 
to acute traumatic neurosis from psychical causes, there is also a chronic 
genesis for this affection, which is undoubtedly more common. It may appear 
alone, or may be combined with the former mode of development. 

When the disease develops gradually and completely, the beginning of the 
process — namely, the trauma — is acute. At the onset, there may be neither 
emotion nor psychical irritation. Pain, more or less impairment of function, 
and the inconvenience thereby produced, now cause the mind of the injured 
person continually to dwell upon the local injury, the accident itself, and 
everything connected with it. Sleepless nights following the accident, as 
well as the first days of incapacity for work, are spent by the injured person 
in meditating upon his position. As recovery is not so rapid as he expected 
he gradually becomes anxious about the future. At this period, in which a 
favorable psychical influence is usually lacking, relatives and friends come 
forward with diagnostic and prognostic utterances, and, with the love of the 
uneducated for sensational tales of injury, identical and similar cases are 
related to the injured person in pessimistic strain. Each individual case 
" in which externally nothing was to be seen," but in which there must have 
been " severe internal injuries," is magnified until finally the injured person 
demands indemnity for his injury. 

The moment the question of damages arises, the wife of the injured 
man usually seems to exert upon him an extraordinary and baneful influence. 
She has listened to conversations between her husband and his friends and 
fellow-workmen, has enlarged her understanding of the case by discussing it 
with her neighbors, and now invariably persuades her husband to demand 
indemnity for the injury he has sustained. As a rule, neither the wife nor 
the injured man has in mind compensation merely for the time during 
which it was impossible for him to work. On the contrary, a sense of justice 
impels them to seek redress. 

The time arrives when the physician in attendance pronounces the man 
well and advises him to return to his work. The injured goes to his place 
of employment after being cautioned by his friends and relatives to take 
care of himself and not to attempt to do too much. As a rule, on the same 
day, or, at the latest, after a few days, he returns saying : " It is impossible, 
I cannot work." He seeks lighter employment — as a rule without success; 
if he obtains it, he soon abandons it. He does not attempt to overcome a 
slight disability by continuing his work, but clings to his own idea that 
he is unable to work. He feels himself justified when he declares that he 
is absolutely unable to work; therefore he demands damages. Subsequent 
professional examinations, the inquiries and communications of his cooper- 
ative associations and of the courts (which, even under normal circumstances, 



ETIOLOGY AND PATHOGENESIS 1103 

are somewhat exciting to the workman) highly inflame the mind of the 
injured man, and he and his adherents regard them merely as attempts to 
deprive him of his just deserts. His mind becomes embittered, he is quarrel- 
some, he devotes special attention to the results of his accident, and nourishes 
these as proof of his rights. In describing his case in the courts or before 
his labor union, all the details are now grossly exaggerated. He speaks of 
" dreadful pains/' " absolute weakness of memory, and of mania," " whole 
nights without sleep/' "great nervous depression/' etc. As soon as he has 
made these statements in court or lodge, either verbally or in writing, he 
becomes absolutely convinced of their truthfulness. 

The strongest confirmation of his views is the patient's distress because 
he cannot work, distress not due to consideration and thought, but to want 
of money which now makes itself felt. If the unfortunate man, because not 
working, has suffered from hunger (i. e., if he has once suffered from the 
idea which possesses him), the constant thought of his disease and the sense 
of his inability to work cannot be removed by argument or by persuasion; 
nothing will dispossess him of this idea, not even the granting of the full 
damages claimed. A pathologic mental state has gradually supervened, a 
distressing form of hypochondriasis. 

That this picture may not be too one-sided and distorted, and to avoid 
injustice, I must remark that the injured person sometimes more strongly 
resists the development of the disease, and from a sense of duty and native 
energy he sometimes masters the threatening affection. But these cases are 
rare. 

In the opinion of some authors, the acute and chronic traumatic neuroses 
show no other mode of development than the psychical, which has just been 
described. As opposed to this, attention must be called to special forms of 
traumatic neurosis without discussing their ps}'chical or non-psychical patho- 
genesis. Cases occur in which the injuries are purely peripheral and either 
severe or moderately severe without general shock to the body or to the 
central organs, and in which no emotion of any kind, especially fright and 
psychical shock, had appeared prior to the accident, and in which the slowly 
acting psychical deleterious change just described could not be demonstrated. 

In such cases (in which, according to existing views, there is no justi- 
fication for speaking of a neurosis due to autosuggestion or to any similar 
psychical agency), motor, sensory, and even trophic changes are often ob- 
served which are strictly limited to the region of the trauma. In these cases 
the injury or shock to which the peripheral nervous apparatus has been sub- 
jected almost invariably appears to have been propagated by the nerve tract 
to the spinal and cerebral centers, or has reflexly produced a pathologic 
change in the centers of the affected peripheral tracts. Cases of the kind, 
which closely resemble typical hysteria, are commonly observed after electric 
injuries. Whether, as suggested above, their pathogenesis is chiefly a purely 
somatic process of unknown " molecular " nature, or whether in spite of the 
apparent absence of any psychical shock psychical processes are active, can- 
not now be determined. At all events the condition must be attributed to a 
psychical genesis if the accident caused absolutely no conscious psychical 
shock. For an external and subsequent psychical effect is possible even when 
there has been no conscious emotion at the time of the accident. We are 
71 



1104 TRAUMATIC NEUROSES 

accustomed to think of psychical effects only when the patient has suffered 
from fright, shock, or other emotion. But experience with hypnotism, with 
conditions during sleep, and also in states of subconsciousness, has shown that 
an external factor like trauma may under some circumstances subsequently 
produce psj^chical effects of most complicated nature and extent in cases in 
which there is absolutely no question of a conscious psychical impression of 
the trauma or of a conscious beginning of the psychical process it produces. 
Here I must call attention to another condition which is not only most 
significant in the psychogenesis of the cases last described, but in the psycho- 
genesis of all traumatic neuroses, and in my opinion is not sufficiently ap- 
preciated. 

As shown by superficial observation every sensory impression produces 
not only a quantitative but also a qualitative effect, which varies even in the 
same individual. This effect may at different times be differently experi- 
enced, and may lead to absolutely unlike emotions. For the effect does not 
depend upon the nature of the stimulation, but is determined to some extent 
by the nature of the area stimulated; i. e., it is determined by the existing 
functional condition of the brain. In other words, the perception and dis- 
tribution of every sensory irritation depend to a great extent upon the condi- 
tion of the psychical functions at the time the irritation was conveyed. 
Obviously it makes a difference whether the irritation is expected and thus 
is met by a brain prepared for it, or whether it is received unexpectedly by 
an absolutely unprepared brain. The condition of " expectation " or " non- 
expectation," as stated, furnishes a crude illustration of the extremely varying 
psychical conditions which may exist at the time of the trauma. Just as 
the prick of a needle may be differently experienced, according to whether the 
attention of the person is withdrawn or not, and whether or not the prick is 
expected, so will the effect upon the mind of trauma vary, according to 
whether it is expected and dreaded, or comes as a complete surprise. 

Now it is more than likely that not only the conscious psychical state of 
the person, but perhaps still more so those psychical processes which run 
their course with a wave of consciousness, has an important influence upon 
the further distribution and the ultimate action of those irritations which 
affect the sensory sphere. There are laws for the development of sensory 
irritations in different psychical fundamental conditions, but such laws are 
unknown for conditions within the wave of consciousness, as well as for 
unconscious psychical states. We must, therefore, not be greatly surprised 
if in one case trauma leaves no visible signs, in spite of the fact that a marked 
psychical impression is made upon the injured, while in another case sensory 
and motor changes appear in an affected member notwithstanding the fact 
that the injured person has experienced neither fright, fear, nor actual pain. 
It is to be hoped that the foregoing will somewhat broaden our conception of 
what is generally understood by "psychical genesis." 

We have thus far considered the traumatic cases which, without recog- 
nizable psychical change, show symptoms resembling hysteria. In view of 
the preceding survey we do not maintain that in all of these cases the patho- 
genesis is absolutely psychical and not somatic. But I believe I have made 
this clear — that in those cases which apparently are not psychically affected 
(nevertheless interesting) there may be a psychical pathogenesis. Moreover, 



SYMPTOMATOLOGY 1 105 

if we enlarge the circle of somatic pathogenesis as we do the psychical, we 
must expect that these two circles will finally meet, and somatic genesis be 
merged in the psychical. 

III. SYMPTOMATOLOGY 

As the clinical picture of the affection under discussion is not uniform, 
but as we are dealing with a number of related neuroses, the symptomatology 
must be so presented that the majority of the phenomena appearing in the 
neuroses, as well as the most common ones, and those which deviate from 
type, will be considered. 

General Impression and Mental Symptoms. — In so far as the general im- 
pression is apparent in the constitution, the bony structure, the muscles, and 
the subcutaneous fatty tissue, traumatic neuroses reveal nothing peculiar. 
In many cases the facial expression and the general posture of the injured 
person is so characteristic that these alone tell the tale of suffering. There 
is a slight, but persistent, longitudinal wrinkling of the forehead, the eye- 
brows are highly arched, and the angle of the mouth is drawn downward. 
The expression of these patients changes but little while speaking; on the 
contrary they look dissatisfied, anxious, careworn, and often frightened. The 
posture of the body and its movements correspond to the physiognomy. The 
head and trunk are often bent forward (except in those cases known as 
" spinal cord neuroses " in which there is an abnormal extension of the 
body) ; all movements of the trunk, all changes of position are sluggish, 
and are made as if the patient were tired, dissatisfied, or as if under constant 
protest. 

The mental condition is frequently what might be expected from the ap- 
pearance of the patients, all of whom feel much more ill than they actually 
are, and believe themselves unfit for any kind of work. As a rule, they 
detail no special hypochondriacal symptoms, but only hypochondriacal gen- 
eral states, declaring that " the entire nervous system is shattered and ruined, 
the whole body is useless, cure or even improvement is absolutely impossible." 
In consequence of this underlying hypochondriasis and the struggle for dam- 
ages, most patients become irritable and embittered, and this engenders a 
nervous condition, feelings of anger, opposition to the physician, sometimes 
even thoughts of suicide. The injured talk themselves into such a state 
that they become utterly unreasonable and unmanageable. The physician, 
who has their welfare at heart, explains to them their condition and why 
the obtaining of heavy damages under present circumstances is impossible, 
tells them that light work will be beneficial, that this is recommended also 
for nervous patients who have not suffered from accident, etc.; the patient 
not only obviously but in reality fails absolutely to understand this well 
meant and conscientious explanation. This is largely to be attributed to 
the persistent psychical depression resulting from the condition, partly, how- 
ever, to the influence of friends and fellow workmen, from whose ranks most 
of these patients are recruited, and who are incapable of understanding this 
objective logic. 

As to intellection, the patients often complain of " defective memory," 
" incapacity for thought/' etc., and often attempt to simulate mental weak- 



1106 TRAUMATIC NEUROSES 

ness; usually, however, there is merely a limited power of psychical concen- 
tration, due to the hypochondriasis, as well as to want of effort without 
actual loss of memory. In the serious cases which must be included with 
severe forms of hysteria or melancholia, there may actually be a marked 
psychical inhibition which gives us the impression of dementia. 

That these patients manifest great loss of energy and an inherent " weak- 
ness of will " need not be specially emphasized when we remember that 
traumatic neuroses are really hysteria, neurasthenia, or hypochondriasis, or 
combinations of these affections. Their conversation often voices their mental 
depression. Their speech is soft, monotonous, or whining, and they become 
animated only when discussing the accident or the question of indemnity. 
In some markedly hysterical cases I have repeatedly noticed speech which 
closely simulated stuttering, and in exceptional instances have observed the 
appearance of the falsetto voice. 

Constitutional Condition and the Vegetative Functions. — As already 
stated, persons of most varied constitution are liable to traumatic neurosis. 
It cannot be stated that the poorly nourished or those with weak frame are 
more readily attacked than the robust and well fed. Varying degrees of 
muscular development, all types of complexion, and differences in the gen- 
eral nutrition, will therefore be observed in these patients. When the disease 
is prolonged, the general constitution it naturally more or less affected. Cer- 
tainly this is true of some forms of traumatic neurosis; combined with 
psychical depression it gradually produces a constitutional invalidism. We 
often observe patients who suffer so greatly from excitement and psychical 
unrest that they emaciate; but true cachexia is hardly ever seen. It has 
always seemed to me remarkable that, in contrast to the foregoing observa- 
tion, many of our patients who complain of numerous corporeal and psychical 
ills, maintain their weight and occasionally even grow stout. Perhaps alco- 
holism, evident in so many of these patients, here plays a role. 

The appetite is more or less capricious. The patients eat enough, and 
their metabolism is usually in equilibrium, but — as they often tell us — 
" there is no pleasure in eating." This loss of appetite is probably in part 
due to their inactivity and refusal or inability to work. The function of the 
bowels is not greatly disturbed; when there is constipation this is often due 
to a spastic state of the muscles of the colon. 

In general remarks upon the nutrition, the appetite, and the posture of 
the body, I have referred only to certain groups of traumatic neuroses; but 
what is said of sleep will apply to nearly all cases. The majority of these 
patients are poor sleepers, they complain of retarded falling to sleep, as well 
as of its being restless and frequently interrupted. If we carefully question 
them we often find these complaints to be justified, although the absolute 
insomnia often emphasized does not exist. Yet the reports of absolute in- 
ability to sleep are, as a rule, not consciously false; for the patients do not 
sleep soundly, therefore while asleep are still alive to certain sensory im- 
pressions. Moreover, in health sleep is followed by a sensation of rest and 
rejuvenation which is absent in these cases, hence the patients feel as if 
they had not slept at all, but were constantly awake and " had heard every 
hour strike." Nocturnal rest is frequently disturbed by dreams, rilled with 
unpleasant sensations of fear (of persecution, falling, corpses, or disputes). 



SYMPTOMATOLOGY 1 107 

Dreams in which the accident is lived over again are rare, while those of a 
pleasant nature have never been reported to me. 

Motion. — It is not remarkable that the muscular system which directly 
depends upon the nervous system should manifest great disturbance. True 
degenerative muscular atrophy does not follow traumatic neuroses; but we 
frequently note atrophy of the extremities — the so-called inactivity and dis- 
use atrophy — the extremities being kept immotile by the patient from pain 
or other cause. Insufficient muscular nutrition is revealed by idiomuscular 
contraction. This shows itself by the appearance of a small transverse 
prominence whenever the muscle is sharply tapped with the percussion ham- 
mer. In these patients true fibrillary muscular contractions are more rare, 
while muscular waves which are closely allied to fibrillary muscular contrac- 
tions are somewhat common. 

Among the numerous symptoms on the part of the muscular system we 
observe all grades of tremor. The very fine tremor seen in Graves' disease, 
a coarse tremor, and the physiological trembling from cold which resembles 
exhaustion tremor are noted; even the grotesque tremor which visibly in- 
creases with intended movements, and which is best designated as shaking 
tremor. The most common localization of this is as follows: Tremor of the 
eyelid on closure, tremor of the muscles of the face during speech, tremor 
of the extended hands, tremor of the legs when lifted high, and tremor of 
the muscles of the trunk. 

Passive movement in many cases is impeded. Not that actual muscular 
spasms such as occasionally appear in hysteria — for instance, in pseudo- 
spastic paresis with tremor (Nonne) — are so common in these patients, but 
peculiar muscular contractions are readily produced in a painful extremity 
or in its vicinity, and these at first are perhaps voluntary, but in the course 
of time, if the pain continues or if the patient believes it to his advantage 
to make the affected extremity appear immotile, these gradually become 
uncontrollable and permanent. Among such contractions belong the muscu- 
lar rigidity which follows injury to the joints, the so-called Ehret's con- 
tracture after injury to the foot, and curvature of the vertebral column and 
changes in the position of the pelvis accompanied by pain in the region 
of the sciatic nerve after injuries to the legs and back. 

Here I must briefly describe a group of injuries to the back which have 
been referred to as " spinal cord neuroses." In these cases the back, espe- 
cially the lumbar portion of the vertebral column, is persistently extended 
far beyond the physiologic limit, and the erector trunci muscles project at 
either side of the lumbar vertebral column like two contracted w^ooden bands. 

In describing pathologic muscular tension we have so far considered 
merely the conditions resembling contracture. Excessive contractions may 
readily be recognized, but they are quite rare. The simple and more common 
muscular tension which is often detected by passive movement of the injured 
extremity is more difficult to diagnosticate. Here a thorough understanding 
of the condition, practice, and care, are necessary in order to differentiate 
an actual spastic, reflex, or involuntary tension from a voluntary one inten- 
tionally produced. Of course, in the conditions under discussion it is pre- 
supposed that there is no evidence of local organic change, especially of 
disease of the joint. But even if this be present the differential diagnosis 



1108 TRAUMATIC NEUROSES 

is sometimes extremely difficult because hysterical arthralgias may so closely 
resemble organic joint affections — certainly in regard to fixation and position 
of the member and in the pain — as to make mistakes extremely likely. 

Hypotonia of the muscles, actual decrease and flaccidity of the muscular 
tonus, is seldom observed in these patients. The flaccidity of the muscles 
after injury to the joints is generally due to inactivity. 

We must now consider active motility in these patients, and what has 
been said in regard to the difficulty in the diagnosis of muscular tension 
may here be repeated. Whether there is complete and real paralysis of an 
extremity — for the condition may be due to hysteria — cannot always be de- 
termined by a single examination, but after repeated investigations this 
should not be difficult. Whether, however, there is merely a diminution in 
the power of certain movements, or of an entire extremity, or — which often 
happens — this is simulated by the injured person, is one of the most difficult 
problems which the neurologist is called upon to decide. True " adynamia " 
or " hypodynamia " is more easily diagnosticated when it implicates the en- 
tire motor apparatus of the body than when the power of only a single member 
is diminished. 

General adynamia, which is more conspicuously shown by the abnormally 
great exhaustion than by the slight production of power, accompanies hysteria 
as well as neurasthenia and hypochondriasis. Local hypodynamia is decid- 
edly more common in neurasthenia and hypochondriasis than in pure hysteria. 
It is to some extent characteristic of general adynamia that in the upper 
extremities the grasp of the hand in particular appears weak while the 
shoulder- and elbow-joints are still powerful. Inversely, in the lower ex- 
tremities the movements of the foot are quite forcible while those of the 
hip- and knee-joints are hypodynamic. Hypodynamic conditions in the 
course of the cranial nerves are not rare in these patients. They are revealed 
by an inability to wrinkle the forehead, to open the eyes widely, to rotate 
them outwardly, and to protrude the tongue. The movements of the eyes 
and, even more so, of the tongue are occasionally impeded by hysterical 
spasms. 

In the same frequency with which the power of motion is decreased co- 
ordination is disturbed. Undoubtedly ataxic movements take place, but only 
rarely and in certain forms of hysteria. A symptom which is usually re- 
garded as a sign of coordinative disturbance is that on closing the eyes and 
placing the feet close together the patients sway or even fall. Sometimes 
this swaying occurs when the feet are in juxtaposition with open eyes. This 
is not the true Romberg's symptom, but is a psychical phenomenon, for sway- 
ing with closed eyes and feet close together is also noted in amaurosis. 

The disturbances of the gait in traumatic neuroses are many and various. 
Some of the patients when walking exhibit only such sluggishness and flac- 
cidity as corresponds to the general motor weakness; others, and this is by 
far more common, simply drag the injured leg and try to spare it. The 
leg is then slowly abducted and extended at the knee-joint, probably at first 
voluntarily although often unconsciously, in the subsequent course of the 
disease from habit and unconsciously; these disturbances in the gait are usu- 
ally observed in neurasthenics and hypochondriacs. 

More manifold and interesting are hysterical abnormalities of gait. Here 



SYMPTOMATOLOGY 1 109 

the hemiplegic form, the sweeping gait (Todd) with a mere dragging of 
the leg which hangs as if dead, as well as hundreds of variations of the 
hysterical dysbasic gait, with spastic or ataxic features, or wdth these com- 
bined. Frequently a cane or crutch is used in walking when this is unneces- 
sary, and this is done merely to awaken sympathy or because the patient 
desires to appear as ill as possible. The staggering gait is most common with 
malingerers, and — as is quite evident to the injured person — this resembles 
the gait of one under the influence of alcohol. 

Just as in walking, motor sluggishness and flaccidity are often apparent 
in other movements of the trunk — in sitting down and standing erect, in 
rising from the recumbent posture, as well as in bending forward and again 
assuming the erect posture. Some patients groan and complain of vertigo 
when they perform this last movement; others, who suffer from so-called 
spinal cord neuroses, cannot stoop at all but let themselves down by flexing 
the knee while holding the back stiff. 

Sensory Functions and Those of the Special Senses. — Disturbance in the 
function of the special senses is noted in all forms of traumatic neurosis. 
Usually there are subjective phenomena : specks before the eyes, blurring of 
the vision, photophobia, tinnitus aurium, sensitiveness to noise, a bad taste, 
etc. Compared with the frequency of these subjective disturbances, actual 
diminution of sensory function is extremely rare, and is almost peculiar to 
hysteria. 

In the eyes this impairment of function does not appear as a decrease 
in the central acuity of vision but as a concentric limitation of the optical 
field. The concentrically limited field (a typical sign of hysteria) is usually 
bilateral. It betrays its psychical genesis by the fact that it remains nar- 
rowed, unchanged, and uniform, whether its projection is in the immediate 
vicinity of the eye or far removed from it (tubular form of the field of 
vision). If we carefully examine the optical field in these patients without 
asking them any suggestive questions, and do not depend merely upon the 
results of a single examination, we will conclude that the frequency of a lim- 
itation in the field of vision in traumatic neurosis is greatly exaggerated. 

Much less familiar to us, and more obscure than in the eye, are the con- 
ditions found in the ear. Here, chiefly after injuries to the head, and with 
an absolutely negative finding by the speculum, we detect a unilateral or 
bilateral defect in hearing especially impairing the perception of the high 
tones of the tuning-fork. Whether this defect, designated by otologists as 
" nervous difficulty in hearing " or " labyrinthine shock/' is to be regarded 
as purely neurotic or the pathologic conception of a neurosis must at this 
point be abandoned is not yet certain. True hysterical deafness or difficulty 
in hearing is much more rare than " commotio labyrintki." Although it 
occasionally disappears suddenly or passes to the other side which has thus 
far been normal — it is, as a rule, extremely tenacious, and the prognosis is 
grave. The diagnosis of a hysterical defect in hearing is extremely per- 
plexing; but, in addition to sudden changes in the power of hearing, it is 
facilitated by the fact that there may be other disturbances of sensation in 
the parts surrounding the ear or in the ear itself. 

A decrease in the senses of smell and taste (a common complaint of these 
accident patients is that " everything tastes alike ") can neither be confirmed 



1110 TRAUMATIC NEUROSES 

nor absolutely disproved since we are unable to test these senses. In hysteria 
there may be complete anosmia or ageusia. 

Unilateral decrease or even abolition of all the special senses is a well- 
known hysterical stigma, and is usually combined with a loss or decrease of 
the tactile and pain senses on one side of the body. Occasionally the sensa- 
tion of the deeper parts is involved in this hemianesthesia, so that the patient 
is unconscious of a passive change in the position of his members, and even 
the stereognostic sense is lost. 

Among the sensory disturbances of a positive hysterical nature which are 
observed in these patients, general anesthesia affecting all of the sensory 
qualities of the skin must be mentioned; this is occasionally accompanied by 
a decrease of the pain sense in the muscles, so that muscular contractions 
produced by a powerful faradic current are not painful. Moreover, we not 
infrequently observe a decrease and loss of the pain sense with an unimpaired 
contact sense, therefore a dissociated sensory disturbance; the temperature 
sense is rarely disturbed. Partial sensory disturbance limited to a certain 
area of the body, an extremity or a portion of one, is more common than 
disturbance extending throughout the body. On investigating sensation in 
a member that has been injured, the patients feel, as a rule, the prick of a 
needle as dull or blunt, or perhaps not at all. We would greatly err in 
regard to these sensory disturbances if we should at once consider them as 
hysterical. Quite a large proportion of these patients undoubtedly and con- 
sciously make false reports; in others these are due to autosuggestion. The 
injured person cannot believe that in a member which is so painful and so 
useless, one function, that of cutaneous sensation, remains unimpaired. If 
he is simulating, and we express a suspicion or utter a warning, the apparent 
sensory disturbance soon disappears while true hysterical sensory disturbance 
will persist. In hysterical monoplegia localized anesthesia is most apparent. 
Hysterical anesthesias are not always — as is stated — sharply demarcated from 
their surroundings, but they often gradually merge with areas in which 
sensation is normal. I do not doubt that some of the sensory disturbances 
noted in our patients are due to frequent, consequently somewhat careless, 
examinations, and are thus cultivated. Therefore too great value should not 
be attached to them, and it is unnecessary to resort to all kinds of expedients 
in order to learn whether or not there is a sensory disturbance. The diag- 
nosis may often be made without including the sensory conditions, and in 
deciding whether or not the patient is able to work existing anesthesia plays 
but an insignificant role. 

Much more essential in regard to capacity for work is hyperesthesia. This 
may appear at the point of trauma, also in other portions of the body which 
are not injured, in the head, the back, etc. The preceding statements in 
regard to the reality of anesthesia are specially applicable to hyperesthesia. 
The mental condition of traumatic neurotics is usually such that the patients 
complain of great pain on pressure at the point at which the trauma oc- 
curred, and even of sensitiveness on superficial contact. In the injured 
extremities passive motion of the joints, sometimes even the mere contact 
of the limbs, is said to cause pain. Whether this hyperesthesia is simulated 
or not can be decided only by successive investigations. If by diverting the 
patient's attention we can repeatedly make firm pressure upon the area said 



SYMPTOMATOLOGY 1111 

to be hyperesthetic without his manifesting pain, while on direct questioning he 
reports extreme pain, it is usually a case of malingering. If, in a single exam- 
ination, there is a contradiction between the condition with deflected attention 
and that upon direct questioning we must not at once assume simulation. 

Hyperesthesia of the head and back also follows injury of these areas, 
but may occur even when these parts are uninjured; if in the head, it is 
particularly noted over the forehead and the crown; if in the back, it is 
usually in the vertebral column. The lower portions of the vertebral column 
are generally most sensitive. Spinal hyperesthesia is peculiar in that the 
areas most sensitive to pressure or to percussion vary. If patients with this 
symptom are examined several times in succession, and at each examination 
we mark the vertebra most sensitive to pressure, we may find that a different 
vertebra is each time designated as the most painful one. This variation 
in the report by no means indicates malingering. I have often seen the same 
condition in patients with neurasthenia not due to injury. Other hyper- 
esthetic areas are the following: the arch of the ribs, the hypogastrium 
(ovarian pressure points), and the superficially situated nerve trunks, the 
last being most marked in women. We must be cautious in assuming a 
special sensitiveness of the nerve trunks to pressure. Many a reported 
pressure point would be omitted from the history of the patient if the in- 
vestigator had by repeated examinations informed himself concerning sen- 
sitiveness to pressure in the areas surrounding the so-called pressure point. 
For there are numerous cases in which pressure at any point of the body 
(in so far as pressure of the soft parts against the periosteum and the bone 
is possible) is asserted to be painful. 

Spontaneous hyperestliesia and parestliesia are more common than hyper- 
esthesia to contact and pressure. Headache, a sense of pressure or drawing 
pains in the head, stitehes in the head, reeling sensations, vertigo either 
persistent or paroxysmal, and both upon directing the glance upward and 
in bending forward, formication at the top of the head, a sensation of heat 
in the head, a feeling as though the brain " would burst/' or as if it moved 
in the head, or as though the head would burst, are a few of the common 
complaints. Among thousands of paresthetic sensations in the extremities 
only a few will be mentioned: a general feeling of paralysis, "the legs feel 
as if dead," heaviness of the members, formication in the hands and feet, 
a sensation as if the tendons were too short or that " everything is tense," 
as if the bones would break, etc. In the trunk we note pains in the back, 
in the lumbar region, between the shoulder-blades, in the side, pressure in 
the cardiac region, and feelings of constriction. The so-called globus hyster- 
icus is exceedingly rare among the hyperesthesias of these patients. 

Reflexes. — The most important of the tendon reflexes, the patella tendon 
reflex, is always increased in traumatic neuroses, whether they are due to 
hysteria, hypochondriasis, or neurasthenia. In the cases of most decided 
increase, a single light tap over the quadriceps tendon will repeatedly cause 
the extension of both legs in rapid succession. This extension is often ac- 
companied by a general contraction of the body, which is occasionally pro- 
duced when the quadriceps tendon itself is not touched but a neighboring 
point — for instance, the border of the tibia. The Achilles tendon reflex is not 
so invariablv increased as the patella reflex. 
72 



1112 TRAUMATIC NEUROSES 

True patella clonus and foot clonus occur in the hysterical forms. A 
condition resembling true clonus, in which the clonic movements cease with 
the cessation of the psychical stimulation of the patient or as soon as his 
attention is diverted from the clonus, will often be observed in the neuras- 
thenic and hypochondriac forms: True clonus which accompanies organic 
diseases is very persistent. The pseudo-clonus just described is an inconstant 
sign. 

The triceps reflex, the jaw reflex, and other tendon reflexes play no role 
in the symptomatology. But attention must be given to the cutaneous and 
mucous membrane reflexes. 

It is well to bear in mind that the action of motions of resistance which 
are closely related to those cutaneous reflexes following painful cutaneous 
irritation do not always agree with the patient's reports of his cutaneous 
sensation. But it does not absolutely prove simulation on the part of the 
injured if a member which has been reported as analgesic is withdrawn on 
being unexpectedly pricked with a pin. The apparent absence of a cutaneous 
reflex (for example, the abdominal reflex) with normal sensation usually 
depends upon an improper position of the patient and consequent muscular 
tension. In connection with cutaneous reflexes I must devote a few words 
to the so-called goose-flesh reflex. After stroking the skin of the chest or 
back with the handle of a percussion hammer we not infrequently perceive 
that the papillae of the skin in the irritated area come into prominence from 
reflex action of the erectores pilorum. 

Of the mucous membrane reflexes only those innervated by the cranial 
nerves need be considered. In rare cases the conjunctival reflex is absent 
when there is hysterical anesthesia, as well as the reflex of the nasal mucous 
membrane, and those which originate from the posterior pharyngeal wall 
and the region of the soft palate. The last two reflexes may be absent with- 
out simultaneous sensory disturbance, and in some of the cases this depends 
upon chronic irritation of the mucous membrane such as is observed in 
smokers or alcoholics. Of course, the absence of the retching reflex is not 
the rule in chronic alcoholism; but, on the contrary, a decided increase of 
the reflexes originating in the pharyngeal mucous membrane. 

The internal reflexes, the pupillary light reflex, the reflexes for the evac- 
uation of urine and feces, and the sexual reflexes, are generally undisturbed 
in these patients, although even here some deviations and peculiarities are 
noted. In hysteria and neurasthenia a rapid and intense pupillary reaction 
to light will sometimes appear, and the so-called hippus of the pupil is often 
observed. This hippus is shown by the fact that, without any variation in 
the brightness of the light, changes in the width of the pupil take place in 
rapid succession. 

Rigidity to light does not appear in traumatic neuroses, but the reaction 
is sometimes sluggish and retarded. Strictly speaking, sluggish pupillary 
reaction to light is sufficient to relegate those cases in which it occurs to a 
realm beyond these neuroses. The cases that I have in mind were usually 
those with severe injuries to the head, with profound psychical depression, 
and general mental dulness. 

Among disturbances of the bladder and rectum I must mention inability 
to void urine when the attention is directed to it, also spastic constipation 



SYMPTOMATOLOGY 1113 

due to similar processes in the smooth muscles of the intestine; these condi- 
tions are found in all traumatic neuroses. Abnormally frequent micturition, 
pollakiuria, is also common. Very rare but of undoubted occurrence is the 
occasional involuntary discharge of urine. This is sometimes found in 
hysterical astasia abasia. The sexual reflexes of erection and ejaculation also 
show severe disturbance. In certain cases the power of erection absolutely 
ceases. 

Vasomotor and Trophic Disturbances. — Derangement of the circulation is 
very common. Conspicuous reddening or pallor of the face, livid discolora- 
tion with coolness of the hands, more rarely of the feet, bluish discoloration 
of the knees, and extreme redness of the face on stooping are often observed. 
A number of vasomotor disturbances are not confined to a definite portion 
of the body but are found over its entire surface. x\mong these is a peculiar 
bluish red mottling of the skin as well as irregular map-like red flakes upon 
the chest and back. Some very interesting vasomotor phenomena may be 
evoked. If the physician makes firm pressure upon the skin with the whole 
hand — for example, on the chest of the patient — after removing the hand 
a white impression of it with redness of the surrounding areas will be seen 
for a few seconds. Furthermore, if we stroke the skin firmly with a hard, 
somewhat pointed object — for example, the tip of the percussion hammer — 
a red line appears in these areas, which often swells for a few minutes and 
becomes elevated, then turns pale, and remains so for some time (dermo- 
graphia). Instead of a sharply demarcated line, in some cases irregular red 
flakes appear in the parts that have been stroked. 

A phenomenon which may be noticed during the test of sensation is the 
appearance of small nodules in the areas subjected to the needle; but these 
also are a form of dermographia. Colorless or pale edema is noted as simple 
stasis edema, occasionally in organic as well as in functional paralysis of the 
extremities. 

Besides this stasis edema we must mention so-called cedeme bleu, which 
accompanies hysterical paralyses, and the so-called " flying edema" The 
latter appears to be closely related to hysterical conditions. It reveals itself 
by edematous swelling which is most marked in the face, which appears sud- 
denly around the eyes, remains for hours, and then disappears without leaving 
any disturbance. 

The hair often shows trophic disturbance. The .hair and beard suddenly 
or very rapidly turn gray; this may affect strands or uniformly implicate 
the entire hairy scalp. Besides grayness of the hair we must consider alopecia 
which follows the distribution of the fifth nerve, while the region of the 
occipital nerve is usually exempt. 

Frequently there is a derangement of the secretion of sweat; the secretion 
is usually profuse. In spite of the fact that the patients do not feel warm 
but are often even cold, there is copious perspiration in the hands and axilla. 
The opposite condition — an abnormally slight secretion of sweat and conse- 
quently unnatural dryness of the skin — is much more rare. 

The structures beneath the skin undergo little, if any, atrophy. Muscular 
atrophy is not peculiar to the nervous pathologic process as such; on the 
contrary atrophy may be of the so-called inactivity or reflex form (in arthritic 
diseases). Consequently it may be found in entire extremities, or in portions 



1114 TRAUMATIC NEUROSES 

which are the seat of pain, in joints which are stiff or which for any other 
reason are but little used. 

Under the trophic and vasomotor disturbances I must again call attention 
to certain difficulties from which the patients suffer. Congestion of the head, 
vertigo, headache which appears and disappears suddenly, therefore symp- 
toms which are common in these cases, cannot always be regarded as purely 
functional, but are in all probability often the expression of organic vasomotor 
disturbance in the interior of the skull. Friedmann, who has closely studied 
these disorders which do not strictly belong among the neuroses, found them 
to be accompanied by marked dilatation of the cerebral vessels, extravasation 
into the surroundings of the smaller vessels, hyaline degeneration of the vas- 
cular walls, and similar changes. If such symptoms appear after severe 
injuries to the head the changes in the cerebral vessels which Friedmann 
observed should be borne in mind. 

Spasmodic Conditions. — Although spasm is not an invariable symptom 
yet it is by no means rare. As a rule, local or general spasm justifies us in 
regarding the case as hysteria. Local spasms are never accompanied by loss 
of consciousness. They may be tonic, clonic, or even of a mixed nature, and 
usually implicate the limb or side of the body which is affected by trauma. 
Local hysterical spasm is more common, and may occur many times during 
the day. It is caused by certain external or psychical irritations, and in 
many cases may be evoked voluntarily by the patients. Moreover they often 
possess the power to suppress such an attack at its onset, but for obvious 
reasons make no use of it. General convulsions — such as those of hysteria — 
are manifold. Consciousness is little or but slightly disturbed, or affected by 
hallucinations or illusions. Complete unconsciousness like that of epilepsy 
is never seen in the spasms of traumatic neurosis, and if during the attack 
the patient does not respond to calling, and seems insensible to all about 
him, nevertheless we may easily prove that sensory irritations are both ex- 
perienced and appreciated. If we suddenly grasp the patient who is con- 
vulsed, or press the fist deeply into the hypogastric region, dash cold water 
into the face, or the like, the character of the spasm as well as its intensity 
will change, which sufficiently demonstrates that the patient was conscious 
of the irritation. 

The character of the contractions varies as greatly as do the voluntary 
movements; for in its nature a hysterical spasm resembles voluntary move- 
ment. Entire movement complexes are executed, and only very rarely do we 
see simple flexion and extension of the member or similar motions which are 
in the lowest plane of coordination. The movements most often observed in 
these spasmodic conditions are a tight closure of the eyelids, drumming move- 
ments with the fore-arm, oscillations of the head, rotation of the body upon 
its longitudinal axis, pulling off the clothes, and tearing out of hair. Of 
tonic movements the well-known arc en cercle or its more common abor- 
tive form, mere extension of the back, is common. No mention need be 
made of the other characteristics of hysterical spasm, except that there 
is sometimes a sluggish reaction to light, more rarely even the absence of 
pupillary reaction to light. As a rule the spasm is preceded by certain 
prodromes: severe pain in a certain area, a sensation of fear, spasmodic 
crying and the like. After the spasm has ceased the patient often feels 



SYMPTOMATOLOGY 1115 

perfectly well; sometimes, however, there is exhaustion which lasts several 
hours. 

In addition to pure hysterical spasms there may be other spasmodic at- 
tacks which present some of the characteristics of epilepsy, but these cases 
are usually alcoholic. Here the picture of the neurosis is not pure, but 
comprises also organic symptoms. Besides attacks of spasm there is "ver- 
tigo " which plays a great role in the complaints of the injured. This usu- 
ally embraces a variety of subjective phenomena which the patient groups 
under the name of vertigo. But there may be actual objective attacks of 
vertigo, in which the patients suddenly experience a sensation of dizziness, 
lose their equilibrium, and sway. If these occur during rest and without 
external cause, they are usually due to severe injuries of the head combined 
with circulatory disturbances of the kind described by Friedmann, such as 
arteriosclerotic changes, or there are organic elements in the pathologic 
picture. If by stooping or rising suddenly or by passive movements of the 
head it is possible to produce such a variation in the intracranial circulation 
as to cause swaying or similar objective signs of vertigo, organic complica- 
tions are not necessarily present, but we may have a purely pathologic picture 
unaltered by organic changes. 

Symptoms on the Part of the Internal Organs. — In many cases there may 
be symptoms attributable to the internal organs, although usually of merely 
functional nature. 

First, the heart must be mentioned. Persistent increase of cardiac activity 
and abnormal irritability arc not rare. While the normal pulse after slight 
exertion is accelerated only a few beats per minute, and then returns to its 
original pace, in these patients after slight exertion the pulse is often greatly 
accelerated (20 to 30 beats per minute), and this rapid action persists for 
several minutes; or we observe that even during rest the pulse may become 
accelerated, and be much more rapid than on bodily motion. Simultaneously 
the apex beat is often heaving without a positive hypertrophy of the left ven- 
tricle being discernible. In some cases there is a conspicuous acceleration of 
the heart beat if we make pressure upon any existing pain points (Mannkopf- 
Rumpfs sign). This, however, is so inconstant that we cannot regard its 
absence as a reliable indication — as some authors do — of malingering. 

Irregularity or intermittency of the pulse, signs which appear without 
organic disease of the heart and which are not rare in neurasthenia, may 
also be noted in neuroses of traumatic etiology, although somewhat less often. 

A common and important symptom on the part of the circulatory ap- 
paratus is arteriosclerosis, which is of organic nature and appears to be 
genetically connected with cardiac activity. The occurrence of arteriosclerosis 
in traumatic neuroses without simultaneous and chronic alcoholism, syphilis, 
or any of the other well-known vascular poisons, was at first doubted, but 
must now be regarded as a fact. I believe the genesis of arteriosclerosis and 
its connection with neuroses must be explained by the fact that the principal 
damage to the vascular wall consists in the extremely frequent and intense 
variation of its vascular lumen. This abnormal distention of the arteries 
may be primary and due to a damage of the general vasomotor center, or 
it may be secondary and attributable to a pathological increase of emotional 
conditions of various kinds or to similar psychical processes. Both modes 



1116 TRAUMATIC NEUROSES 

of development are possible in traumatic neuroses. It is obvious that the 
normal physiologic stimulation of the interior of the vessels by the circu- 
lating blood must be changed by the abnormal variation in the caliber of 
the arteries. The reaction of the vascular wall to this peculiar internal 
irritation is expressed by a loss of elasticity in the vessel followed by a 
thickening of its walls, and finally by a calcareous deposit; briefly, the phe- 
nomena of beginning and complete arteriosclerosis. As already indicated, 
arteriosclerosis is an exceedingly important, and the most frequent, organic 
accompaniment of traumatic neuroses. 

The respiratory organs, especially the lungs, less often show changes. 
Sometimes the respiratory type is altered, an acceleration being observed 
which may be quite excessive. It may be paroxysmal or persistent. Of course, 
changes in the lungs and respiratory passages are not the cause of the in- 
creased respiration in traumatic neurosis. Hysterical cough is as rare as 
hysterical tachypnea; it is usually distinguished by its peculiar barking char- 
acter, and here another symptom may be mentioned — hysterical hemoptysis. 
In most cases this is due to hemorrhage from the mucous membrane of the 
mouth, the gums, or the pharynx, produced by the hysterical mental state of 
the patient, or purely from malingering. In isolated cases there are said to 
be spontaneous hysterical hemorrhages from the respiratory organs. 

Of the abdominal organs only the kidneys show disturbance in function. 
Here must be mentioned the excretion of enormous amounts of urine and 
alimentary glycosuria — i. e., a temporary excretion of sugar after the in- 
gestion of large quantities of carbohydrates. 

IV. DIAGNOSIS; SIMULATION 

In the diagnosis of traumatic neurosis we must decide three questions: 
1. Are we dealing with a neurosis? 2. Is the neurosis traumatic — i. e., can 
it be traced to an accident? 3. What form of traumatic neurosis is present? 

The first question — whether a neurosis is present — is generally more easily 
answered than the other two. It is true only the most important neuroses 
form such characteristic pictures that non-recognition is impossible and that 
the diagnosis can be made at sight. Difficulties arise only in the less fully 
developed forms, the so-called formes frustes (undeveloped forms) of general 
neuroses, and in certain local mono-symptomatic neuroses, such as functional 
monoplegia and diplegia, paraplegia of the legs, etc. 

In the differential diagnosis of pathologic conditions manifested by gen- 
eral complaints and general phenomena, we must first consider the following 
organic diseases: Progressive paralysis, obscure focal diseases of the brain, 
multiple sclerosis, general arteriosclerosis, certain chronic intoxications, such 
as saturnism and alcoholism, also a series of systemic diseases such as chronic 
disease of the kidney, diabetes, etc. 

A diagnostic danger pointed out by many authors is that indefinite path- 
ologic conditions of nervous nature are liable to be regarded as "traumatic 
neuroses " simply because of their appearance after trauma ; here only the 
most careful investigation will preserve us from error. It must be borne in 
mind that the presence of one single symptom due to organic cause vitiates 
the diagnosis of neurosis. If we observe sluggish pupils, rigidity of the 



DIAGNOSIS; SIMULATION 1117 

pupils, pallor of the optic disc, absence of the patella reflex, degenerative 
muscular atrophy, a lead line, sugar or albumin in the urine, extreme ar- 
teriosclerosis, or similar conditions, the most obvious inference is that no 
neurosis is present. Of course traumatic neuroses are somewhat more apt 
to be accompanied by organic disease than the non-traumatic — as I tried to 
explain when discussing the s}miptomatology — and only when the compli- 
cated organic symptoms are subordinate to the entire pathologic picture may 
we conclude that a neurosis is present. This is the case when there is slight 
arteriosclerosis after injury in a person who shows chiefly the general ob- 
jective and subjective symptoms of the neurosis or similar conditions. 

If the organic symptom present is pupillary rigidity or something of like 
importance, we must have other and cogent reasons for believing a neurosis 
to be present before we can make such diagnosis, or can believe that symp- 
toms of two different natures, one of a neurosis and one of organic disease, 
coexist. In practice this generally occurs when the organic sequels of chronic 
alcoholism (disturbance of pupillary reaction or optic atrophy) or of an old 
syphilitic infection are joined to neurotic symptoms. 

Among the organic diseases of the nervous system which must be con- 
sidered in the differential diagnosis are a few which, especially at the onset 
of the affection, may completely simulate a neurosis. Chief among these are 
progressive paralysis and brain tumor. A case of the latter proved most 
instructive to me. 

The patient was a man with a severe injury of the head. He presented marked 
symptoms of a neurosis, but under any psychical influence there was such a conspicu- 
ous variation in his condition and complaints (uncertainty in gait) that we seriously 
considered a diagnosis of hysteria, and thought certain symptoms to be feigned. While 
under observation, however, objective signs rapidly appeared which permitted the diag- 
nosis of brain tumor, and autopsy revealed a tumor in the anterior portion of the 
brain. 

In a pathologic condition like that just described the decision whether 
the affection is functional or organic may not always at first be possible; 
nevertheless, after an examination, especially after several such or after 
clinical observation, it can be made with great certainty. But there are con- 
ditions in which such a diagnosis, or even a differential diagnosis, cannot 
be made ; I refer to the cases in which, following severe injuries to the head, 
there is mental depression, inhibition, general psychical dulness, vertigo, 
headache, general adynamia, etc. This symptom-complex, as Friedmann 
demonstrated, is often due to the multiple but slight organic changes in the 
cerebral vascular apparatus and its surroundings. It is no doubt true that 
Friedmann's findings are observed in only a few of the cases with this clin- 
ical picture, and that others with exactly the same clinical phenomena are 
purely functional. But Friedmann's investigations and similar ones make 
it appear likely that in the future many other symptom-pictures following 
trauma, which have so far been regarded as functional, will be proven to be 
■due to an underlying organic condition. 

Assuming that examination has revealed no organic change in our pa- 
tient, even this does not certainly prove a neurosis, and our object has been 
only partially attained. For in the preceding consideration we always as- 
sumed the presence of distinctly objective pathologic symptoms. If the 



1118 TRAUMATIC NEUROSES 

injured person presents no noteworthy symptoms but, on the other hand, 
there are many subjective phenomena and complaints, purely in theory the 
existence of a neurosis becomes likely. In practice, as a rule, another factor 
is added which doubles or trebles the diagnostic difficulties : simulation. Here 
I believe it wise to discontinue our discussion of the diagnosis, and briefly 
consider simulation. 

The term simulation should be employed only when the person examined 
consciously and purposely attempts to deceive us, representing pathologic 
signs to be present which are not so, or when he wilfully exaggerates existing 
conditions. Therefore the attempt to deceive is an integral constituent of 
our conception of simulation. This is decidedly modified when we consider 
what physicians commonly call feigning. Primarily we must differentiate 
those cases in which a pathologic state of mind or of sensation leads to false 
reports, and among these we include most of the prominent forms of hypo- 
chondriasis and many cases of hysteria. This by no means implies that 
hysterics or hypochondriacs do not occasionally and consciously make false 
reports, and may therefore be malingerers. 

Secondly, we cannot group among malingerers those injured persons who, 
while not hypochondriacs or hysterics, actually do misrepresent facts, but 
who are not sufficiently intelligent to appreciate that what they state (usually 
by the advice of others) in order to obtain damages is not the truth. Such 
people are often imbeciles from birth, and not possessed of even the most 
rudimentary education. 

Between the latter and the hysterical is a third group of patients who 
are difficult to handle. These are patients who actually have sustained certain 
injuries. For years past they have made innumerable reports, verbal as well 
as written, of their condition. To appear consistent, ailments which have 
gradually passed away or become secondary are continually included. They 
have observed that judges and physicians believed their reports. Without 
any special predisposition to nervous affections — except a congenital want of 
discernment — and from constantly brooding over the injuries in question a 
mental condition has gradually developed which impairs the injured one's 
faculty of criticism, nor does he desire to use this faculty. Such torpid 
mental appreciation, which for any psychological reason may gradually and 
completely be removed from criticism, which has become a " matter of 
faith " — this latter, however, being more or less true of all persons — by no 
means implies an aberration from the normal mental state. This approxi- 
mates what we moderns have called " autosuggestion," and has become the 
more firmly rooted the more sensibility and emotion have aided in its de- 
velopment. 

The chief difficulty in the clinical discrimination of these cases is on the 
one hand their similarity to hypochondriasis and a paranoic condition, and 
on the other hand to malingering. This perplexity is enhanced by the fact 
that the same case is at various times judged differently. What may to-day 
be regarded as simulation (according to the definition just given) may a 
year from now appear otherwise, and, just as in a previous examination, may 
be unconsciously simulated, therefore no longer feigned. No general rules 
can be formulated, but every case must be individually investigated and 
judged. 



DIAGNOSIS; SIMULATION 1119 

The perfect simulation of an entire symptom-complex, none of the symp- 
toms actually existing, is so exceedingly rare as to be seen but a few times 
among several hundred cases. For obvious reasons a few symptoms are often 
simulated, and these are interwoven with difficulties actually existing, there- 
fore etched into a real pathologic condition. Prominent among these is an 
apparent diminution or absence of sensory functions and those of the special 
senses. Hemianesthesia which actually exists in no small proportion of these 
cases is known to many patients (perhaps owing to the indiscretion of the 
physicians during their frequent examinations, their accompanying questions 
not being always cautiously framed) as a sign of disease, hence is often 
simulated. Among motor symptoms those adynamias and pareses are simu- 
lated which are generally limited to an extremity or a portion of an extremity. 
Arthritic and muscular rigidity are often feigned. 

In discussing Romberg's sign I called attention to the fact that swaying 
on closure of the eyes is a favorite method by which the accident patient 
attempts to prove the severity of his disease, but the movements of stooping 
and subsequent rising also furnish an opportunity for exaggeration, the in- 
jured either declaring themselves absolutely unable to stoop or that on doing 
so they sway or even fall. 

In eliciting the patella tendon reflexes we occasionally note that the pa- 
tients try to increase the jerk by their own efforts, or they produce all sorts 
of muscular contractions such as drawing up or drawing away the legs and 
the like, in order to show the active reflex effect upon the nervous system. 
In the course of objective investigation, attempted simulation in regard to 
sleep, appetite, and other subjective difficulties is much more common. 

After having enumerated the principal factors which incite the injured 
to feign, I must state that the recognition of simulation is one of the most 
difficult parts of the investigation of these patients. For, since actual sim- 
ulation as well as hysterical and hypochondriacal exaggeration originates 
psychically from the pathologic nature of the disease, and is therefore a 
psychically congenital phenomenon, it is not remarkable that simulation and 
pathologic exaggeration are often closely akin. Only a most experienced 
physician who understands the nature and the phenomena of the groat neu- 
roses will attempt to diagnosticate simulation from any of the aforesaid 
symptoms, and eases occur which even baffle an expert, as has been repeat- 
edly emphasized by Strihnpell and other authors. 

The demonstration of simulation is usually of greater practical than sci- 
entific import; and in practice one law must chiefly be our guide. We must 
always prove that the actual difficulty, the definite symptom-complex, or the 
individual symptom which has always been paramount, is simulated. There- 
fore evidence that the patient has a general tendency, even in other matters, 
to simulate does not warrant us in declaring that a suspicious individual 
symptom is feigned. For example, if a patient has paralysis of the arm which 
is difficult to explain, and we find upon investigating sensation or testing his 
visual field that he makes incorrect reports (therefore shows a tendency to 
feign) this is not sufficient to prove that the paralysis of the arm is also 
feigned. The fact that the patient at every investigation tries to deceive 
us will naturally strengthen our suspicions that the paralysis of the arm is 
simulated, but this presumption becomes certainty only when by some method 



1120 TRAUMATIC NEUROSES 

of examination — or even without this — it is proven that the patient can 
voluntarily move the arm which he exhibits as paralyzed. 

The foregoing postulate of a special proof of simulation determined in 
each case is based upon the experience that accident patients, who are usually 
of little education, attempt to make an actual infirmity, pain or the like, 
appear more plausible to the physician; perhaps because they have previously 
been met with suspicion. For this reason they are anxious to show that 
other portions of the body are affected as well as that which was injured, 
and for this they invent symptoms. Although these patients are really at 
fault, yet if the investigator condemns this tendency to simulation as ma- 
lingering, his judgment of the general pathologic picture is neither scientific 
nor just. Aside from this reason there is another ; we should be very cautious 
in attributing a symptom to simulation, for among the methods which are 
calculated to discredit the symptoms of the patient there are many which, 
as I have already intimated, in the hands of one not an expert may lead 
to most erroneous conclusions. I have called attention to the similarity of 
conditions in malingerers and in hysterics. This similarity is markedly 
apparent when we test the accuracy of the patient's statements by a method 
based upon spontaneous sensory impressions or those of the special senses. 
For this reason I advise caution in the use of a number of tests advised in 
text-books and employed to unmask malingerers. If the patient answers 
affirmatively certain absurd and suggestive questions as to whether "he sees 
double early in the morning," whether "both thumbs become numb," or 
similar ones advised in literature, the significance of these answers is purely 
problematical. The same care must be exercised in putting our questions 
in regard to pain upon pressure and the like, and even in the opposite 
direction skepticism should not carry us too far. Sometimes the physician 
makes pressure over a cicatrix upon the head, and the patient reports pain 
in the area touched, but does not state that the surroundings are also sen- 
sitive; shortly afterward the physician tells him that the cicatrix is in another 
area (very often the patient is unable minutely to localize the cicatrix upon 
the top of his head if he is prevented from touching it with his finger) and 
the patient now reports tenderness at a second place, the simulated cicatrix, 
while the actual cicatrix is no longer painful. In the great majority of cases 
this is regarded as conscious simulation. But it may be that, even here, 
we are dealing with a markedly hysterical person, and that the suggestion 
was conveyed by the physician. 

I must here call attention to another localization of pressure pain in 
discriminating which I believe errors are often made. Pain in the spine, 
which is frequently produced in these patients by pressure on or tapping the 
spinous processes of the vertebrae, sometimes varies so much in the course 
of a single examination that nearly all of the spinous processes may be de- 
clared to be the ones most sensitive to pressure. Those not familiar with 
this condition may readily suppose the patient to be feigning. But in pa- 
tients with functional nervous diseases, those who have not suffered from 
an accident, and who therefore have usually no incentive for simulating this 
condition, we often note the same phenomenon. 

What has been stated sufficiently indicates the difficulty of proving ma- 
lingering. Hence the question arises : How can we prove that a definite 



DIAGNOSIS; SIMULATION 1121 

symptom or complaint is simulated? And here it is much easier to state 
how we should not proceed than how we should. 

Only general rules can be given; above all, it is desirable that the method 
employed to determine simulation should be most simple. The more com- 
plicated it becomes and the greater the time it consumes the more it taxes 
the intelligence and good-will of the patient, and the more practice and 
familiarity with the condition it necessitates on the part of the investigator. 
I strongly object to the employment of complicated apparatus. If the pa- 
tients have the slightest degree of intelligence, in most of them such apparatus 
is calculated to produce immediately a certain ps} T chical irritation which 
proves a source of error. If the examination makes simulation appear likely, 
it is wise to make this diagnosis positive by giving the person an opportunity 
to make an " honorable " and apparently non-compromising withdrawal, the 
suspicious symptom for one reason or another seeming to disappear. Under 
long observation the patient sometimes realizes that he cannot keep up his 
role for any length of time, and is anxious to avail himself of the chance 
offered. It is inadvisable to use powerful agents, such as strong faradic cur- 
rents, to prove malingering. Such methods are cruel, and the results attained 
are not always uniform. 

After this somewhat lengthy but necessary digression and the description 
of simulation, we return to our proper theme — the diagnosis. Before dis- 
cussing simulation, we had assumed that the diagnosis had reached that point 
at which an organic affection could be excluded. The description of simula- 
tion became necessary for, after making a diagnosis, and after excluding 
organic disease, the affect ion is proven to be functional. Now we must decide 
two questions — whether the neurosis is traumatic, and what form of trau- 
matic neurosis it presents. In answering the first, we must distinguish be- 
tween the scientific and the practical proof that the disease is of traumatic 
genesis. Scientifically its accidental origin may often be proven from the 
fact that we knew the patient prior to the accident. Those forms are excep- 
tions which bear the stamp of traumatic genesis, because of either a typical 
ensemble of somatic and psychical disturbances, or the presence of hysterical 
paralysis or something similar at the seat of trauma. In practice the proof 
of a traumatic origin of the disease is easily demonstrated. As a rule it 
may be considered certain if no other etiology for the neurosis than that of 
trauma is apparent, and if the trauma from its nature and severity seems 
calculated to produce a nervous affection. Practically it suffices if we can 
prove the trauma to have been an essential or partial cause of the disease, 
or that the affection was aggravated by the accident. 

The last point to be decided in making the diagnosis is the accurate de- 
termination of the nature of the traumatic neurosis present. It has already 
been stated that by the designation "traumatic neuroses" we mean cases 
only of hysteria, hypochondriasis, neurasthenia, or combinations of these 
affections; but not those other neuroses which occasionally develop after 
trauma, such as chorea, epilepsy, etc. Therefore the decision as to the nature 
of the traumatic neurosis will chiefly depend upon a differential diagnosis 
between these three great ps3^cho-neuroses. 

The differential diagnosis is facilitated when there are conspicuous symp- 
toms which point to the fundamental hysterical character of the neurosis; 



1122 TRAUMATIC NEUROSES 

for instance, monoparesis of an extremity, astasia abasia, sensory hemianes- 
thesia, contractures in an extremity, or in the muscles of the tongue or face. 
These signs, or similar ones, generally justify us in regarding the functional 
symptom-complex as of hysteric nature. If there are no conspicuous, somatic, 
hysterical symptoms, but psychical ones are most prominent, if there is anxi- 
ety, metaphysical mania, or mental depression, the neurosis is usually hypo- 
chondriasis. When the psychical depression is less marked, or when it is 
only slight while adynamia and abnormal exhaustion are extreme, the path- 
ologic condition must be regarded as neurasthenia. Sensory disturbances 
contraindicate the hypochondriacal or neurasthenic character of the affection. 
Certain somatic symptoms, such as increase of the tendon reflexes, dermo- 
graphia, irritability of the heart and acceleration of the pulse, tremor of the 
tongue, hands, and eyelids, swaying upon closing the eyes, are found in all 
of these three neuroses and, as a rule, do not aid us in the differential 
diagnosis. 

The majority of the somatic symptoms enumerated as common to the 
three neuroses lead us to the conclusion that the differential diagnosis may 
often be exceedingly difficult, and in many cases it really is impossible to 
reconcile the pathologic picture as a whole with any of the forms of pure 
neurosis. This difficulty is enhanced by the fact that the psychical conditions 
appertaining to these three neuroses have many features in common: the 
tendency to excitement and irritability, fear, unrest, and distressing dreams. 
To these psychical conditions which belong to the picture of hysteria, neuras- 
thenia, and hypochondriasis of non-traumatic genesis are finally added those 
psychical peculiarities which may be attributed to the claim for damages. 
Since a suit for damages represents a mighty psychologic influence which 
often preponderates over the genuine psychical effects of the neurosis, we can 
easily understand why most cases of traumatic neurosis wear a similar psy- 
chical mask, and why the peculiar and original physiognomy of the neurosis 
can be recognized only by close study of the case. As, however, the marked 
psychical influence of the desire to " obtain damages " finally reacts upon 
the somatic signs of the disease, it does not appear strange that in the differ- 
ential diagnosis of neuroses of traumatic origin we do not advance very far 
before we perceive that we are dealing with a combination of three great 
neuroses. Nevertheless, even here we must act according to the fundamental 
law, " nominatio -fit a potiori" and designate the disease according to the 
symptoms which dominate the clinical picture. 

V. PROGNOSIS AND COURSE 

The prognosis of traumatic neurosis in regard to life, provided there are 
no special complications or sequels, is absolutely favorable. Among com- 
plications I include general invalidism and suicide. In rare cases, especially 
where there is profound psychical depression, but also in cases of Friedmann's 
vasomotor type, we note a gradual loss of strength which so lessens the power 
of resistance of the organism that even mild intercurrent diseases may cause 
death. Nevertheless, such cases are exceptional. 

Somewhat more frequently severe traumatic hypochondriasis, hysteria, 
and the mixed forms terminate by self-destruction. This is most often the 



PROGNOSIS AND COURSE 1123 

case when chronic alcoholism plays a role. Despair caused by inability to 
support a family, anger over presumable or actual injustice, and similar 
emotions, are usually the cause of suicide. 

The prognosis as regards cure is much more unfavorable than in regard 
to life. Here it may be laid down as a law that if there are claims for dam- 
ages, no matter of what nature, the prognosis is decidedly more grave. This 
law needs no elaboration after what has been stated of the psychical genesis 
of traumatic neurosis. For it is to be hoped that the connection between 
corporeal and psychical phenomena has been so clearly outlined that what 
I have said may not be construed as meaning that the patient who makes a 
claim for damages purposely, and as a rule, prolongs the period of con- 
valescence. Cases of deliberate prolongation of convalescence are certainly 
rare. On the other hand, the dominating thought of obtaining material 
damages for the injury, at the time, but only for the time, is such a dominat- 
ing psychical factor that, without straining conscience, it stimulates the 
development and continuance of a hypochondriacal mental state quite distinct 
from the injurious psychical effect due to the excitement of court investiga- 
tion of the claims. 

But even if no such claims are made, the prognosis is by no means good. 
Psychical and somatic factors may prolong the purely functional affection 
for many years, even when the patient is most anxious to recover. The indi- 
vidual traumatic neuroses show distinct differences in prognosis ; it is best 
in the mild or moderately severe cases of neurasthenic type following simple 
peripheral injuries or slight head trauma and in those with not too severe, 
purely hysterical symptoms. When pain is persistent — as, for example, with 
rigidity of the back and hysterical symptoms — the prognosis is more serious. 
In those hysterical cases combined with cardiac and respiratory symptoms or 
with conspicuous tremor, there is little tendency to improvement. In op- 
position to the foregoing statement, the prognosis is often somewhat better 
in hysteria accompanied by spasmodic attacks. Those cases in which the 
neurosis is based upon imbecility are exceedingly unfavorable, as are also 
all true traumatic cases of hypochondriasis. The gradual transition of the 
latter into paranoia is not rare, and this renders the prognosis absolutely 
hopeless. 

The duration of the disease is never to be reckoned by days or weeks, 
but usually by months, and sometimes by years. As a rule, the pathologic 
picture of cases with a bad prognosis remains not only qualitatively but quan- 
titatively unchanged. Remissions are rare; on the contrary, transitory ex- 
acerbations are common, usually occurring after excitement or the like. 
Intercurrent disease may temporarily make the symptoms of traumatic neu- 
rosis secondary. After this intercurrent affection has run its course, the 
nervous phenomena, appear unchanged, prominent, and sometimes even in- 
creased by the addition of symptoms which are nervous " transitional " forms 
of the sequelae of the intercurrent disease. 

The prognosis of traumatic neurosis is generally the same in men and in 
women; children occupy a special position. 1 The prognosis in children is 

i Compare Paul Schuster and Kurt Mendel : " Traumatische Nervenkrankheiten bei 
Kindern." Monatsschrift fur Unfallheilkunde, 1899, Nr. 6. 



1124 TRAUMATIC NEUROSES 

usually not worse, as might be supposed, but better than in adults; while 
the effect upon the course of the disease or filing claims for damages is more 
conspicuous in children than in adults. 

VI. PROPHYLAXIS AND TREATMENT 

There are few affections the treatment of which is so unsatisfactory to 
the physician as traumatic neuroses. An ideal plan of treatment is not diffi- 
cult to devise, but its carrying out will always be Utopian; for traumatic 
neuroses are more dependent upon external conditions than any other disease. 
In prophylaxis — by far the most important part of treatment — our best en- 
deavors are thwarted by difficulties due to external circumstances. As was 
shown at the beginning of this article, traumatic neuroses are most prone 
to develop in that period immediately following the accident. This nascent 
stage is the one in which conceptions and influences conducive to the produc- 
tion of the disease are most dangerous, and at this time the influence of- the 
physician is most powerful. Therefore, in the first few days after an accident, 
an injured person should be under the care of a physician who will take 
sufficient time to gain the confidence of his patient, and thereby secure a 
psychical influence over him. Moreover the physician who first treats the 
case should be possessed of sufficient psychiatric and neurologic acumen to 
recognize the first sign of a neurosis, and thus to control it. By a careful 
and full examination — which alone often quiets a nervous patient — and by 
telling him not to fear any further consequences, the physician may often 
relieve the mind of the injured person, and at the same time counteract the 
psychical shock which the patient has suffered by showing him that he regards 
the accident as of slight importance and a secondary matter. Furthermore, 
if possible, the physician should attribute the nervous disturbances resulting 
from the accident to some other cause. Inability to work should be spoken 
of as merely temporary, so that if possible the patient's anxiety for the 
future should be at once allayed. Briefly, the physician who first treats the 
patient should invariably and continuously manifest interest in the patient 
and give him attention and care, and should also try in every way to divert 
his mind from the accident, and eradicate its memory. 

In reality how are these indications usually fulfilled? After an accident 
the injured person is generally treated by a dispensary physician or is taken 
to a drug-store, and given cursory treatment by a passing physician. In 
either case the physician usually sees the injured person but once, and has 
no opportunity to acquire an influence over him. At this time the injured 
man hears from his fellow workmen or from some of the hospital nurses 
how serious and how prolonged such injuries usually are, etc. After receiv- 
ing first aid the patient is usually left to the care of a dispensary assistant 
(in Germany of a Kassenarzt). This physician is under such pressure of 
work that, as a rule, he treats only the local (surgical) symptoms of the 
accident without concerning himself with the mental state of his patient. 
If, during this time, general disturbances are noted for which no apparent 
reason can at once be assigned, or if they appear to be due to chronic alco- 
holism, arteriosclerosis, or other condition, the physician, with the hope of 
consoling the injured, will as a rule ascribe these difficulties to the accident, 



PROPHYLAXIS AND TREATMENT 1125 

saying that they will disappear with the gradual amelioration of the other 
sequelae. This method of ascribing all of the patient's difficulties to the 
accident has a very serious effect on the further progress of the case, and 
it is strengthened under the following circumstances : if the patient's case is 
pending in court, or (in Germany, when there are sick benefits, Kranken- 
kasse) if the question arises whether the pathologic condition is the result 
of an accident or an independent disease in no way connected with the acci- 
dent. The lodge or society, which in doubtful cases is anxious that the ex- 
isting condition be attributed to the accident, sometimes brings pressure to 
bear upon the physician. The firm sued for damages does not share this 
opinion, and thus the patient is pulled to and fro with conflicting opinions, 
his mind constantly busy with the origin of his condition. Of course, the 
knowledge that it will be better for him to be awarded substantial damages 
than to receive a certain sum of money from his society or lodge, will lead 
the attending physician to concur in the opinion of the physician of such 
beneficial society; therefore he will frequently ascribe the difficulties to the 
accident. Thus the external conditions are directly opposed to any rational 
prophylaxis. 

From the foregoing it might be deemed advisable to relegate the first 
treatment of the injured to specialists who are most familiar with accident 
cases, in order to secure the best mental effect upon the patients. But such 
a proposition is impracticable; for, in the event that the physicians had been 
appointed by the firm for whom the patient had worked, this would arouse 
prejudice and opposition on his part. The prophylaxis of traumatic neurosis 
is therefore not very encouraging, and even if some slight result is attained 
this is immediately more than counterbalanced by the adverse opinions and 
the conversation of the patient's friends and fellow workmen. 

"What measures are at our command when a traumatic neurosis develops 
in the injured? First, those measures which aid us in psychical treatment 
must here be employed. How these act has already been explained in dis- 
cussing the prophylaxis, but even without such explanation, and merely from 
his knowledge of the genesis of traumatic neurosis, the physician should 
understand how to treat a patient with this affection. The details of this 
knowledge cannot be imparted, but spring from the physician's professional 
tact and general knowledge of human nature. 

Secondly, we have at command all those physical remedies which are 
employed in the treatment of nervous patients in general : hydrotherapy in 
the form of baths, lukewarm plunge and partial baths, warm or cool ablu- 
tions, general or local manual massage, vibratory massage, and faradic and 
galvanic treatment. Drugs cannot be entirely dispensed with : bromid and 
valerian, and for many cases opium, are necessary. In patients not wholly 
degenerate these and similar measures sometimes bring about the improve- 
ment of one or several symptoms, or even the entire condition, but this 
improvement usually lasts only as long as the treatment is continued. 

On account of the difficulty of carrying out this therapy, it has been 
suggested that these patients be treated in a hospital, and in different places 
institutions have been equipped chiefly for the treatment of traumatic neu- 
roses. These offer the advantages of clinical observation and treatment of 
the patient by specially trained physicians. Nevertheless, the practical sue- 



1126 TRAUMATIC NEUROSES 

cess of these hospitals for accident cases appears to be neither great nor 
encouraging. This is because the injured entertain a prejudice against acci- 
dent clinics and similar institutions to which I have called attention, and 
have a strong disinclination to being treated in such. Moreover, the bringing 
together of many injured persons in one hospital has the disadvantage that 
the patients, by constant intercourse with one another and from the fact that 
they continually see the professional examinations of their associates, often 
acquire an astonishing and most undesirable education in the symptomatology 
of traumatic neuroses, and subsequently (consciously or unconsciously) make 
use of this knowledge. Finally, as I know from years of experience, there is 
engendered in the patients (not only in. those suffering from injury but from 
general neurologic conditions) a certain passive resistance and mute opposi- 
tion which makes treatment exceedingly difficult, and calls for the greatest 
patience on the part of the nurses and physicians. Yet if it is desirable for 
these traumatic neurotics to have the advantage of clinical treatment, nothing 
remains but institutions for the treatment of internal or nervous diseases or 
sanatoria. Now, as a rule, such hospitals refuse admission to patients of this 
kind, for reasons easily understood, and treatment in a sanatorium is usually 
beyond the patient's means. 

As the result of these considerations I can only repeat what I implied 
at the beginning — that the treatment of traumatic neurosis is in the majority 
of cases " love's labor lost." Its intractability to therapeutic measures is only 
slightly due to the fact that hysteria, hypochondriasis, neurasthenia, and re- 
lated neuroses are all likewise insusceptible to therapy. It is largely because, 
for most of these patients, the prolongation of the disease secures material 
advantages without working for them. And although there are many cases 
in which the affection persists in spite of obtaining damages, nevertheless 
the chief obstacles in the way of carrying out a rational therapy are a claim 
for damages and refusal to work. 

Yet a method must be devised by which to relieve the injured from ma- 
terial care for his future, and simultaneously to prevent him from directly 
transferring his injury into cash without an intervening attempt at cure. 
It should be made impossible for the person partially incapacitated to obtain 
indemnity unless he makes some effort toward recovery. It might be enacted 
that a person who is only partially disabled should receive indemnity in 
proportion to his disability only provided he is willing to exert himself and 
to work so far as his condition permits. Only the one absolutely incapaci- 
tated should at once receive damages. In order actually to utilize his remain- 
ing capacity for work, the injured first of all needs an opportunity. The 
meeting of "this want should be the object of large manufacturing concerns 
or, still better, of trades unions and the like. The majority of those partially 
disabled cannot utilize their powers because employers will not hire such 
persons when better material is to be obtained. Therefore cooperative asso- 
ciations should provide employment with wages corresponding to the capacity 
for work, and thus make actual opportunities for work — only in this way can 
a claim for damages be justified. It is clear that the finding of such employ- 
ment would be an arduous task for the cooperative associations, but such 
remedial measures may be forced upon us if we are to reduce the present 
number of suits for damages. It is to-day everywhere recognized that the 



PROPHYLAXIS AND TREATMENT 1127 

salient point in the treatment of traumatic neuroses consists in getting the 
patients to work, and that compared with this all mechanotherapy and sim- 
ilar measures are far behind. 

Recognizing these facts a society has been formed in Berlin which attempts 
to secure this action on the part of the cooperative associations. The " So- 
ciety for Accident Cases " in Berlin will aid persons suffering from accident 
by giving them an opportunity to work, or does this through their own so- 
cieties. The Society is still young and inexperienced, but there is no doubt 
of its beneficent work. Some large industrial establishments — for instance, 
a large brewery in Berlin — have adopted similar methods of aiding their in- 
jured workmen. 

The furnishing of suitable work to those only partially disabled I must 
designate as the chief thing to be provided for in the readjustment of accident 
insurance. Another but less important change would be to modify the regu- 
lations concerning work so that only complete disability and three or four 
grades of partial disability should be recognized. Small weekly payments 
should be altogether tabooed, and indemnity should consist of a single 
payment. 

Experience seems to indicate that by these or similar changes much of 
the baneful excitement which now attends the payment of weekly indemnity 
for accidents might be avoided. More might be done in this direction if 
cooperative institutions were to discard some of their irritating officialdom, 
and conduct affairs in a more business-like way. Even trivial things must 
be considered in establishing a new law ; its enforcement and beneficent effect 
depend in a high degree upon psychical coaction. 



VASOMOTOR TROPHIC NEUROSES 

By R. CASSIRER, Berlin 

The realm of vasomotor trophic neuroses which we shall review is ex- 
ceedingly vast. We find here a rich chain of pathologic pictures which some- 
times are substantive, and sometimes merge into other nervous symptom- 
complexes. Among these we include acroparesthesia, Raynaud's disease, 
erythromelalgia, sclerodactylia, and scleroderma. Somewhat apart from these 
is angioneurotic edema, but its relations to the other forms mentioned are 
so intimate that we shall describe it with this group. In this realm, we often 
meet with symptom-complexes which can be strictly classified only with the 
utmost difficulty, which in one respect resemble this, in another respect that, 
clinical picture, and which unite into every possible mixed or transitional 
form. If hereby the strict lines of our system are somewhat obliterated, yet 
certain lines of demarcation are absolutely necessary. The realm of these 
affections is so vast, and there are such differences between the terminal links 
of the chain, that a separation into groups appears to be absolutely necessary. 

As the name indicates, the symptoms which play the main role are chiefly 
those of disturbed vasomotility and trophic disturbance. To these are added 
sensory and secretory disturbances, while true motor and irritative symptoms 
are of subordinate importance. Individual symptoms of different kinds may 
develop. In those of vasomotor nature, we differentiate between a decrease 
{local anemia) and an increase {local or regional hyperemia) of the local 
amount of blood. The first may be subdivided into local syncope and local 
asphyxia. The clinical appearances will later become obvious. Change in 
sensation mostly appears as paresthesia and pain. Very frequently thermo- 
paresthesia, or objective disturbance of sensation, is of less importance, and 
this more often appears as hyperesthesia than as anesthesia. The secretory 
symptoms present themselves as anhidrosis and hyperhidrosis. But the 
trophic disturbances are of greater significance. When we speak in this way 
of trophic disorders, we refer chiefly to those which depend upon definite 
disturbances of some portion of the peripheral or central nervous system. 
The question as to how this trophic influence arises, what tracts it utilizes, 
what is its action, has of course been by no means decided, and is still open 
to discussion. Even to define its outlines would be beyond the scope of this 
article. 1 I can only state that experimental as well as clinical experience 
points to the great influence which the nervous system exerts upon the nutri- 
tion of tissue; as a rule it appears to be a pathologic change in innervation 
rather than its complete cessation which produces trophic disturbances. Each 

i See my monograph: "Die vasomotorisch-trophischen Neurosen," Berlin, 1901, 
S. Karger. 

1128 



VASOMOTOR TROPHIC NEUROSES 1129 

tissue and cell is nourished by attracting to itself from the nutritive fluids 
those products which it requires for its nutritive integrity. This function 
of the cell is subject to nervous influence, but the arrest of the trophic nervous 
influence does not cause a suspension of nutrition and consequently of growth 
and new formation, but only decreases and modifies these. " The necessity 
for nutrition/' says Samuel, 1 " lies in the cells, the measure of trophic in- 
fluence comes from the nervous system." Without these there would be, as 
we might say, a vita minima of the tissue; when there is no special exercise 
of its activity, its structure may remain undamaged, but any great exertion 
may change it. The tissue becomes " more sensitive. " Thus, when a patient 
suffers from myelitis, slight pressure on the skin ma}?- cause necrosis, a 
pressure which will be borne without damage by the healthy skin. The slight- 
est external damage to a cornea from which the nerve has been removed 
produces a severe inflammation. It has often been supposed that such im- 
mediate and external proof of damage by a nutritive disturbance sufficiently 
demonstrated that we were dealing with a trophoneurotic lesion. But this 
is not correct. The normally nourished tissue of the body has a power of 
resistance to external and internal damage, and is able to maintain its trophic 
integrity provided the damage does not go beyond a certain extent. The 
slightest irritation which causes destruction will disturb the trophic condition, 
and the cause of this disturbance is a pathologic change in the nervous influ- 
ence which is probably conducted by sensory and vasomotor tracts. 

We therefore find such trophic alterations as important features of the 
pathologic pictures which we are here considering. We meet them in the 
form of local tissue necrosis or gangrene, or as abnormally marked or abnor- 
mally decreased growth, also as a special nutritive disturbance which we desig- 
nate scleroderma, and which we shall discuss later. 

In addition to the nature of the symptoms, we find another common 
peculiarity; namely, that the projecting portions of the body are almost 
invariably the seat of the pathologic change. The fingers and toes, the nose, 
the ears, and the chin are preferably attacked, and there is a conspicuous 
tendency to symmetry. 

We are constantly dealing with neuroses, that is, we consider the seat 
of the affection to be in the nervous system, yet there is no pathologico- 
anatomical foundation for this hypothesis. Therefore we must assume the 
existence of " functional " diseases of the nervous system, and the vasomotor 
apparatus is probably the chief seat of these conditions. The fact that it 
extends throughout the entire body is one of the chief objections to this 
minute localization of the pathologic seat. The vasomotor system consists 
of numerous branches one above the other, and dependent upon each other. 
A vasomotor center appears to be located in the cortex of the cerebrum. 
Thence the vasomotor tracts, probably close to the sensory, pass to the great 
subcortical ganglia where their continuity is most likely interrupted by cell 
groups. They then pass on through the pons to the chief vasomotor center 
in the medulla, thence through the lateral divisions of the medulla spinalis 
to the various heights of the spinal cord. Here they split around the cells 
of the gray middle substance. From these spinal vasomotor centers new 

i Samuel, " Die tropkischen Nerven," Leipzig, 1860, 



1130 VASOMOTOR TROPHIC NEUROSES 

fibers arise which pass as white communicating branches to the boundary 
column of the sympathetic and to the periphery. Here again ganglion cell 
groups are intercalated, some in ganglions of the boundary column, some 
nearer the periphery, even in the walls of the vessels. 

Physiologically we differentiate two kinds of centers and tracts: Vaso- 
constrictors and vaso-dilators. The immensity of the tracts and centers is 
of the greatest physiologic and pathologic importance. It permits a rapid 
compensation of disturbance, which is further favored by the fact that the 
subordinate centers, in contrast to the more important ones, are relatively 
substantive. This naturally makes a local diagnosis difficult, particularly 
since we have no means of concluding from the nature of the lesion its seat, 
while in the motor system, for example, the nature of paralysis, whether 
spastic or of an atrophic degenerative kind, at once betrays the seat of the 
disease. Thus all the factors combine to render the pathogenesis and path- 
ology of these pathologic pictures very obscure, and much is left for in- 
vestigation. 

Acroparesthesia. — The symptom-complex which we most frequently meet 
is acroparesthesia. The following is the history of a case: 

A woman, aged 34, came to the Clinic because three weeks previously she sud- 
denly felt pain in her arms, particularly in the right one, which still persisted. She 
described the pain as deep-seated, of a stabbing and burning character, passing along 
the bone, and radiating from the elbow-joint to the hand and fingers. The pain was 
greater at night, the hands and fingers then becoming numb so that she could neither 
touch nor hold anything, and there was formication. These unpleasant sensations 
were so marked that they prevented the patient from sleeping. All these symptoms 
were said to have appeared for the first time three weeks previously after a day of 
washing. The patient stated that she had lately done a great deal of laundry work. 
Of former diseases she could report but little. According to her statements, she had 
quite a severe attack of syncope two years ago, and gastric ulcer fourteen years ago. 
From time to time she suffers from headache, but has not recently had it. 

Examination of this powerfully built, and not anemic, woman revealed no patho- 
logic condition in the internal organs, and objectively, in the nervous system, only 
that sensation was decreased in the fingers of both hands, the tips of all the fingers 
being insensitive to pin pricks. In the vola manus this intense analgesia extended to 
the middle phalanges. In the same region, strong brush contact was not experienced, 
and sensibility to heat and cold was decidedly disturbed. The nerve trunks were not 
sensitive to pressure, but in the region of the cervical sympathetic there was a very 
unpleasant sensation, especially upon the right side. The pulse was not increased, 
and was the same upon both sides. Vasomotor stimulation of the skin showed no 
change. There was no disturbance of motion in the hands, the muscles were not 
atrophic. The skin of the fingers was somewhat remarkable, being extraordinarily 
hard and dry, and traversed by numerous fissures. These changes were more marked 
than usual, even in persons of the laboring class. 

This case is a typical example of a conspicuous form of acroparesthesia. 
It occurred in a woman in the fourth decade of life. Women are more 
frequently attacked than men; among 90 cases which I observed, only 10 
occurred in men. I have an impression that a more intense damage is neces- 
sary to produce this affection in men than in women. People of a certain 
age are particularly liable to it. Among 184 cases (my own statistics and 
those of Frankl-Hochwart, 1 150 were between 30 and 60 years of age, but 

i Frankl-Hochwart, " Die Akroparasthesien," 'NothnageVs Spec. Pathol, u. Thera- 
pie. 1898, XI, 2. 



ACROPARESTHESIA 1131 

I once saw a child aged 7, and at another time a lady of over 70- who 
presented the same s} T mptoms. Our patient stated that the first symptoms 
appeared after washing clothes. This is in conformity with other experi- 
ences. Occupations which necessitate the use of much water, particularly 
cold water, are important factors in the development of the disease. Other 
forms of hard manual labor, also over-use of the hand, may predispose to it. 
In women there are additional etiologic conditions in the sexual life, the 
influence of the climacterium, the puerperium, pregnancy, etc. It is especially 
interesting to note, as I did in four or five cases, that acroparesthesia occurred 
after operative removal of the uterus and the ovaries with and without other 
symptoms of climax prcecox. Several of my male patients were alcoholics. 
Sometimes trauma may be the cause, compression of the fingers, or a fall 
upon the elbow as in one of my patients. Sommer also describes a case with 
traumatic etiology. 

The symptoms in my patient were unpleasant or even painful sensations 
in the fingers, hands and forearms. These are the main symptoms of acro- 
paresthesia. There is a sensation of formication, of itching, burning, or 
numbness, a feeling as if the finger or the entire hand were swollen. But 
there may be every transitional stage between these unpleasant sensations 
and actual pain, which, as in the history just quoted, may disturb the rest at 
night. 

Usually the paresthesia and pain are not continuous, but occur in attacks 
which are most marked toward morning. Then the fingers become stiff and 
clumsy, and they can be used only after rubbing, squeezing, or patting them. 
As a rule, the patients cannot sharply define their sensations. Sometimes the 
fingers of both hands, sometimes of one hand only, and occasionally the indi- 
vidual fingers, are said to be painful or numb. Exceptionally the upper arm 
is implicated as high as the shoulder. In my patient there was a conspicuous 
disturbance of sensation affecting the terminal and middle phalanges of all 
the fingers of both hands uniformly including their sensory qualities. Here, 
as elsewhere, the hyperesthesia was by no means limited to a peripheral nerve 
or its root area. This is important, for both Dejerine and Egger have re- 
cently reported cases in which the objective sensory disturbances were limited 
to the tract of certain posterior roots, and Pick had formerly observed a 
similar distribution of subjective disturbances. According to my experience, 
this is by no means the rule. Objective sensory disturbances may be entirely 
absent, and they are generally neither so frequent nor so well developed and 
extensive as in the patient whose history was just related. 

In many cases the only symptoms are these objective and subjective phe- 
nomena. Other cases, however, may show the following symptoms: Pallor 
of the fingrrs and of the hands simultaneously with the previously mentioned 
paresthesia, in which sensations of cold frequently predominate. The history 
of a patient presenting this type, which unquestionably is more rare than the 
first, is the following: 

A servant girl, aged 16, was compelled to work in a cold kitchen. For eight 
■weeks she complained of distress in her hands, numbness, a loss of sensation, and 
burning of the fingers, especially in the morning and after carrying heavy loads. 
Frequently the fingers became deathly pale and so stiff that she could not move 
them. This condition lasted several hours, formication extending to the shoulder. 



1132 VASOMOTOR TROPHIC NEUROSES 

Examination revealed absolutely normal conditions except for the paroxysmal numb- 
ness of the fingers. 

Here, in addition to paresthesia, we note local syncope. This form of 
the clinical picture, as already remarked, is rare, and should be differentiated 
from the first mentioned variety; it forms a transitional stage into one of 
the incomplete forms of Raynaud's disease. 

The affection usually begins with slight attacks and develops gradually, 
but an acute onset has also been observed, and this was the case with our first 
patient. When the disease has appeared it usually proves very intractable. 

This acroparesthesia is not uncommon in persons who present other symp- 
toms of a general neuropathic condition. The patients are easily irritated 
or frightened, they are anxious, they cry readily, and are often bad-tempered. 
Especially do they show the signs of the so-called neurasthenia vasomotoria, 
namely, a tendency to flushes and heat in the face, to vertigo, palpitation, 
and syncope; I, as well as others, have seen this neuropathic state combined 
with migraine; on the contrary it must be stated that such a neuropathic 
diathesis is not invariably present. 

I shall not discuss the pathogenesis of the disease. In my opinion we are 
dealing with a sensory or vasomotor sensory neurosis, and, as a rule, the 
peripheral, sensory, cutaneous, and vascular nerve terminations, also the 
peripheral vaso-constrictor nerves and ganglia are the seat of the disease. 

The diagnostic difficulties are not great; but we must be careful not to 
overlook other severe pathologic centers than those referred to by the patient, 
for acroparesthesia may be a partial symptom of severe organic affections of 
the nervous system. Not infrequently have I listened to the complaints of 
patients in whom investigation revealed the signs of tabes, usually of be- 
ginning tabes. Sometimes these proved to be (a fact which I can confirm) 
the early symptoms of acromegalia, a disease first described by Sternberg. 
Beginning polyneuritis may at the onset also develop similar symptoms. 
Latent cases of tetany may cause confusion. Most difficult is the differentia- 
tion from occupation neurosis. I recently saw a cigarmaker who for weeks 
had suffered from paresthesia of the tips of his fingers. His occupation 
obliged him to roll wet tobacco. Suspension of his work in a short time 
brought about a cure, and this beneficial effect of rest is certainly in the 
majority of cases a valuable differential guide. Positive differentiation is not 
always possible, for some occupations are very influential in the development 
of true acroparesthesia. The avoidance of these deleterious pursuits must 
often be advised in therapeutics, particularly the prolonged use of the hands 
in cold water should be interdicted; on the contrary, warm water in the form 
of local baths with or without additions (salt, various extracts) often has a 
beneficial effect. The symptoms of the patient are sometimes relieved by the 
use of the faradic or galvanic hand bath. 

Raynaud's Disease. — The pathologic picture next to be discussed, Eay- 
naud's disease, is much more rare. This was first reported in 1862 1 under 
the name of " local asphyxia and symmetrical gangrene of the extremities." 
The chief symptoms and the course of this remarkable disease are briefly as 

i Raynaud, " De l'asphyxie locale et de la gangrene symetrique des extremites." 
Paris, 1862. 



RAYNAUD'S DISEASE 1133 

follows : Usually in neuropathically predisposed individuals, but occasionally 
also in others sensory and vasomotor disturbances gradually develop which 
are localized principally to the peripheral portions of the body. These are 
manifested by local syncope, by local asphyxia, occasionally by a regional 
redness accompanied by severe pain. This redness appears paroxysmally in 
blotches showing symmetrical arrangement, and may dominate the pathologic 
picture for days and weeks. In many cases, however, especially in the severe 
ones, trophic disturbance of the implicated areas appears in the course of 
the first weeks in the form of an almost symmetrical, superficial, dry gangrene. 
The disease may exhaust itself with a single attack of gangrene, or re- 
newed attacks may occur for years. . I shall relate the clinical history of a 
case which presents all the important features of this pathologic picture. 

A man, aged 29, formerly a typesetter, and as such coming into contact with 
lead, had again, as a tavern-keeper, been exposed to alcoholic toxic influences. His 
preceding history shows that in 1892-3 he suffered from severe attacks of pain in 
the abdomen which were diagnosticated as neuralgia of the nerves of the liver, and 
were treated with morphin. 

Although he relinquished his occupation as a typesetter, the attacks did not at 
first cease, but after persisting for five years they disappeared. In the autumn of 
1900 new symptoms appeared: After bathing, the index finger of the left hand always 
lost sensation, it became numb, and as white as if it belonged to a corpse. A few 
months later a circumscribed black discoloration appeared upon the middle finger, 
and with great pain a part of the tip of the finger sloughed off. This bluish black 
discoloration paroxysmally appeared in other portions of the middle finger of the left 
hand and in the second finger of the right hand. The pains were intense, particularly 
at night; in the index finger of the left hand a vesicle occasionally appeared, and 
attacks of local syncope were noted. Ears, nose and feet remained uninvolved. The 
patient could not be described as of a nervous temperament. 

Examination revealed that the terminal phalanx of the left second finger was 
deformed, the skin was lilac red, and there was a depressed cicatrix. The patient felt 
as if a wound were being touched. The left third finger was a somewhat darker red 
and cooler than the others, the terminal phalanges of the second and third fingers were 
blackish as if they had been dipped in diluted ink, the terminal phalanx of the right mid- 
dle finger was deformed and pointed, the skin being tough, thick, and wrinkled. The 
skin at the tip of the second finger was distinctly hardened. Sensation in the hand 
was everywhere normal, even in the deformed second and third right fingers. Other- 
wise, examination of the nervous system revealed normal conditions. 

Two months later, upon the 19th of February, 1901, the following was noted: The 
right thumb also was for some time affected. During the examination, local asphyxia 
periodically appeared, especially in both middle fingers and in the right thumb. The 
difference in temperature between the individual fingers was very marked; for exam- 
ple, taken with a surface thermometer, in the right little finger it was 93° F., in the 
right middle finger 02.6° F., in the right thumb 68° F. A second test of the left little 
finger showed it to be 91.5° F., the tip of the right thumb 73.4° F. 

On the 25th of October, 1903, gangrene was no longer present. As the patient 
stated, the right middle finger remained the focus of the disease. He returned to his 
occupation of typesetting, and pursued it for a year without any ill effects. In the 
open air or in a room of low temperature, his fingers became stiff, sensitive to the 
touch, pale, or blue. When examined they were icy cold. In parts they showed great 
change, being light red, violet, or extremely pallid, and particularly in the second and 
third fingers of the right hand, fine brush touches were not felt. The patient reported 
a remarkable effect that alcohol had upon his fingers : In two minutes after taking a 
little spirits the activity of the fingers was completely restored. 

This patient showed the characteristic symptoms of this remarkable dis- 
ease. In its well marked forms, it is a very rare affection, more frequent in 



1134 VASOMOTOR TROPHIC NEUROSES 

women than in men, and is somewhat more common in infancy. As to the 
etiology, it must be mentioned that occupations carried on in the cold air, 
or by using cold water, etc., as well as those which expose persons to toxic 
influences as, for instance, working in lead, are said to be predisposing factors. 
Trauma may be a cause, especially when it repeatedly affects the fingers 
(injuries from a circular saw, Brasch; injuries to the fingers of bricklayers, 
Hess). Psychical trauma must also be considered. Dehio's patient was 
greatly frightened by an attempted rape. The disease has been observed after 
various infectious diseases: Enteric fever, typhus fever, influenza, erysipelas, 
etc. It has been noticed with particular frequency after an attack of malaria. 
Symptoms of Raynaud's disease have been observed in the course of chronic 
infectious diseases, and here both hereditary and acquired syphilis must be 
considered; from the course it was determined that the affection was actually 
a manifestation of syphilis. Of course, in such cases we must always seek 
to ascertain whether or not organic vascular processes, especially gangrene, 
have produced the symptoms. 

Without doubt, the most important of all etiologic factors is the nervous 
predisposition of the individual, whether acquired or congenital. 

In my patient (whose history has just been given), as is usually the 
case, the disease was ushered in by vasomotor symptoms. Three different 
forms may be differentiated: Local syncope (regional ischemia, Weiss x ), local 
asphyxia (regional cyanosis), and regional hyperemia which was not correctly 
appreciated by Raynaud. The parts attacked by syncope, usually one or sev- 
eral fingers, more rarely the toes, suddenly become white and cold (dead 
fingers). At the same time there is usually paresthesia, also pain which may 
increase enormously in severity. Pallor and coldness disappear, the affected 
part resumes its normal color or becomes intensely blue, even bluish black 
(local asphyxia), while in other parts under reaction redness and heat appear. 
These vasomotor symptoms may occur in irregular order, affecting at one 
time this, at another time that, portion of the implicated area, and may 
produce an actual play of colors. 

With asphyxia, as with syncope, there is usually a decided decrease in 
temperature; this may be extreme, falling nearly 20° C. below the normal 
temperature of the affected surface. As stated, this decrease in temperature 
was observed thermometrically in my patient. We were actually startled 
when the patient's ice-cold fingers were touched. The affected areas are also 
often swollen. 

The duration of the vasomotor symptoms varies greatly. The individual 
attack may terminate in a few minutes; it may last for hours or even for 
days, and may recur several times during the day, etc. 

The vasomotor symptoms are followed by gangrene, at least in the typical 
cases. Small vesicles form which rupture and leave ulcers that very slowly 
heal. At other times the vesicles do not rupture but dry up, or the skin 
thickens from the onset, to be later sloughed in hard, brown crusts. The 
slight extent of the gangrene is characteristic. It is usually limited to small 
areas of a phalanx; very seldom is an entire phalanx sloughed off. As a 
rule, it affects symmetrical areas, but asymmetry has also been observed, 

iM. Weiss, "Ueber symmetrische Gangran." Wiener Klinik, 1882, p. 347. 



RAYNAUD'S DISEASE 1135 

and has been attributed to certain individual conditions. For example, I 
saw a case of this kind in which there appeared to be a congenitally weaker 
development of the arterial vascular system of the affected arm. The seat 
of the gangrene is usually a finger, a toe, the margin of the ear, more rarely 
the nose, the tip of the tongue, the nipple, or the lips. The nutrition of the 
affected areas may also be modified in another manner. These changes belong 
largely to the realm of sclerodactylia, a condition to which I shall revert 
later. But the parts may become larger, hemorrhages may take place into 
the skin or into the subcutaneous tissue, changes in the nails are common, 
whitlow which runs its course with intense pain has often been observed, and 
this is important in the differential diagnosis. 

I saw a patient, aged 32, who was suddenly attacked by ulcers in the fingers, the 
tips of the lingers became black, the superficial areas and even portions of bone 
sloughed away, and there was very severe pain; recovery was extremely slow. Since 
that time pain in the fingers has persisted although there has been no whitlow for 
seven years. The hands are now deformed ; bilaterally a considerable part of the 
terminal phalanx of the second finger is absent. An X-ray picture shows that the 
bone also is implicated. In the pulp* of most of the fingers there are superficial cica- 
trices, the fingers in toto are bluish red, cyanotic, and cold, and in them sensation is 
somewhat below normal. 

Always prominent among the symptoms is pain, which in fact occasion- 
ally appears to be excruciating ; Raynaud says : " I have known persons, other- 
wise quiet and patient, to howl with pain, their bodies bent and contorted 
as they sit in bed. These unfortunate subjects appear to pass their entire 
time in seeking a position which will afford some relief." Sometimes the 
pains are not severe, and like the very slight objective sensory disturbances, 
they are usually not circumscribed. 

Occasionally secretory disturbances, particularly hyperhidrosis, accompany 
the vasomotor symptoms. Motor phenomena are here very inconspicuous, 
but no doubt localized motor symptoms, particularly atrophy of a non-degen- 
erative character in the muscles of the hand, do arise in the course of Ray- 
naud's disease; I have reported such an instance. 

In one case Eaynaud observed a very peculiar ophthalmoscopic picture. 
The patient called his attention to the fact that while he was able to see 
during the attack after it passed away there was disturbance of sight. Oph- 
thalmoscopic examination showed that during the time in which the sight 
was affected there was an abnormal contraction of the central artery of the 
retina at its origin in the vicinity of the papilla ; Weiss, as well as a few later 
observers, saw a similar condition, but, upon the whole, such findings are 
rare. Periodic contraction of the arteries has also been noted in other iso- 
lated areas. Usually, however, the pulse shows no change ; this may be proven 
graphically. 

Interstitial abnormalities are sometimes observed in the secretion of urine. 
The coincidence of paroxysmal hemoglobinuria and Raynaud's disease is 
peculiar. The relation of these symptom-complexes to each other varies; 
sometimes the symptoms of Raynaud's disease are prominent and hemo- 
globinuria is scarcely noticeable, at other times the reverse is true. Here it 
must be noted that the pathologic picture of paroxysmal hemoglobinuria is 
in many respects similar to paroxysmal symmetric gangrene. It would lead 
73 



1136 VASOMOTOR TROPHIC NEUROSES 

us too far from our theme were we to discuss this interesting subject. 
Paroxysmal albuminuria and paroxysmal melituria have also been observed. 
In such cases the diagnosis of Raynaud's gangrene must, of course, be cau- 
tiously made in order to prevent confusion with gangrene due to vascular 
disturbance, particularly when an intermission of the symptoms is observed. 
A moderate excretion of uric acid has also been noted. English authors have 
ascribed great etiologic importance to the uric acid diathesis. My experience 
leads me to assume that gouty persons and members of gouty families show a 
great tendency to variations of the vasomotor innervations. 

The heart and the vascular system usually show no organic changes. In 
a few cases, besides typical Raynaud symptoms, an organic cardiac affection 
existed, apparently, independent of these. I saw an instance of this. There 
are also a few cases reported in which, besides arteriosclerosis, the typical 
symptoms of Raynaud's disease appeared intermittently. But all of these 
are exceptions, the diagnosis of which 'necessitates special care. 

As a rule, the general condition of the patient is not below par. Dis- 
turbances occur gradually, and are usually due to the pain, the processes 
which cause gangrene, etc. 

As has been several times reiterated, the course of the disease is typically 
intermittent. An attempt has been made to divide it into different stages, 
but this adds little to our knowledge of the affection. Vasomotor symptoms 
usually precede the gangrene which appears with an increase of pain. The 
acme of the disease appears to be reached in the area first affected, but 
paroxysms may develop in other areas. The disease is often exhausted in a 
single attack which comprises several paroxysms. This was the case in the 
two patients whose histories I quoted; the various later disturbances were 
merely sequelae; but renewed attacks may appear at shorter or longer inter- 
vals. In the subsequent course the paroxysmal type gradually becomes oblit- 
erated, and the intensity of the symptoms decreases. There are, however, 
cases in which the vasomotor symptoms (from the onset) show a chronic and 
progressive development; certainly the local asphyxia points to such a form. 
In addition to the other deviations from type we frequently note that the 
gangrene and the pains become less marked, but, in their place, other trophic 
disturbances are observed, enlargement of the fingers or other peripheral 
parts, in which, however, the bones are not involved; in other cases objective 
sensory disturbances are very conspicuous. I have described such cases as 
acroaspliyxia chronica, and have attempted to separate them from the ordinary 
cases of Raynaud's disease. Other authors (Hirschfeld, Pehu) have observed 
similar cases, but their number is small. 

Unquestionably there are many cases in which only vasomotor disturb- 
ances persist, in which gangrene never occurs, or, at least, only in the later 
course, in which there are slight trophic disturbances, such as thickening 
or enlargement of the terminal phalanges, and hardening of the skin. Some 
of these types are the forms which undergo a transition into Nothnagel's 
vasomotor neurosis, some are closely allied to sclerodactylia, and perhaps are 
cases of the last named affection. 

The fact that we not infrequently meet with a combination of Raynaud's 
disease and diseases of the central nervous system is especially interesting. 
Among the latter is tabes dorsalis, particularly gliosis spinalis, syringomyelia 



RAYNAUD'S DISEASE 1137 

(Schlesinger, Pospelow), also tumor of the spinal cord or of the spinal cord 
roots. We frequently find Raynaud's symptoms in hemiplegia; on the other 
hand, I am aware of no positive case of neuritis combined with Raynaud's 
disease. 

Functional disturbances of the nervous system are common; the neurotic 
diathesis often paves the way for the disease. Among other symptoms we find 
those of neurasthenia, traumatic neurosis, chorea, epilepsy, and also of 
hysteria. In many of these cases Raynaud's symptoms are merely the signs 
of a general neurosis, and lose their substantive character. 

These disturbances are most frequent on the part of the nervous system, 
and may be regarded as an expression of especial sensitiveness of the 
vasomotor system : Impaired tone of the cutaneous vessels, dermographia, 
tinnitus aurium, vertigo, a tendency to vomiting, to urticaria and to transitory 
edema. The combination with migraine and Graves' disease has frequently 
been noted. Such pathologic pictures have .been described by Solis- Cohen 1 
and Herz 2 as vasomotor ataxia. 

Our knowledge of the pathological anatomy of the affection is very lim- 
ited, in spite of the no small number of cases in which necropsy was per- 
formed. Changes have been found in the peripheral nerves; to this, how- 
ever, I attach no causal significance; also changes of the peripheral vascular 
system, especially of the smaller arteries, which are, however, only of sec- 
ondary importance. 

In regard to the pathology, the vasomotor symptoms of syncope, asphyxia, 
and hyperemia unquestionably depend upon the abnormal irritative, and per- 
haps also paralytic, symptoms of the vascular nerves, yet we are unable to 
define all of the individual points. Gangrene is certainly not merely the 
consequence of vasomotor symptoms; nor can it be referred to changes in 
the peripheral nerves which are most likely of secondary nature, and the 
slight organic changes in the central nervous system so far demonstrated 
cannot be regarded as the cause. Nevertheless I do not doubt that the 
gangrene in Raynaud's disease is a neuropathic form of gangrene. Upon 
the whole I regard this malady as a " functional " disease of the vasomotor 
and trophic tracts and centers. The pathogenesis of the pathologic picture 
does not appear to be uniform. Congenital loss of resistance and an acquired 
lability in which cold, intoxication, and infection play a role, perhaps also 
reflex changes in diseases of the peripheral organs (mostly of the vessels, 
rarely of the nerves) are prerequisites for the development of the affection. 
Whether the locus moroi is always the same has not been demonstrated. I 
have already stated that, at the present time, it is well not to speak of Ray- 
naud's disease but of Raynaud's symptom-complex. 

The diagnosis and the differentiation from other diseases must be briefly 
discussed. If we remember the criteria of the disease previously given, its 
recognition will usually not be difficult, but its relation to such affections as 
acroparesthesia, erythromelalgia, scleroderma and angioneurotic edema are 
so close, the transitional stages between these affections so ill-defined, that 
discrimination is not always possible, and not always desirable. The differ- 

i Solis-Cohen, " Vasomotor Ataxia : A Contribution to the subject of Idiosyncrasies." 
Am. Journ. Med. Sciences, CVII, p. 130. 

2 Herz, " Ueber vasomotorische Ataxie," Berlin, 1902. 



1138 VASOMOTOR TROPHIC NEUROSES 

ential diagnosis from syringomyelia, above all from one of its secondary 
forms, Morvan's disease, is important. I must emphasize the fact that in 
the latter the course is a chronic and progressive one, that the whitlow is 
usually painless, and that motor disturbances and sensory phenomena are 
more prominent. Some cases of leprosy resemble Raynaud's disease, perhaps 
also certain forms of beri-beri. When gangrene appears in the course of a 
cardiac or vascular affection, the differential diagnosis from senile gangrene 
and gangrene due to endarteritis obliterans also raises the question of the 
differential diagnosis from Raynaud's disease. The paroxysmal, preceding, 
vasomotor symptoms, the slight extent and the localization of the gangrene, 
will usually point to Raynaud's disease. The differential diagnosis from 
intermittent claudication (limping) sometimes necessitates careful consid- 
eration. The long persistence of sensory symptoms and the dependence of 
the disturbances upon the use of the legs are typical of this affection. 
Gangrene due to ergot produces a picture similar to Raynaud's disease. A 
careful history is of the utmost importance, and this also is true of the 
differentiation from chilblain. 

The disease in itself does not threaten life, but it may become very dis- 
tressing, and last for years or decades. 

The treatment must be of two-fold nature: First, prophylactic measures, 
which consist mainly in the avoidance or decrease of thermic deleterious 
agents, particularly of the effect of cold. The period of suffering for these 
patients is winter. 

Second, constitutional treatment which is directed against the under- 
lying neurotic or, rather, the vasomotor neurotic diathesis. Quinin has been 
especially advised, in solution (2.0:180 = a tablespoonful three times daily, 
Herz), as a tincture, or in combination with ergotin, strychnin, or iron. 
From the vasodilators, amyl nitrite, nitroglycerin or trinitrin, not much is 
to be expected; occasionally they mitigate an attack, but for this purpose 
alcohol has the same effect and is a much better remedy; its beneficial effect 
was praised by one of the patients whose history I have quoted. Hot drinks, 
as I know from experience in other patients, are likewise beneficial. Adrenal 
extract is highly recommended by Solis-Cohen; I have not had much experi- 
ence with it; at all events, it is not harmless, and we should be cautious in 
its use. Cocain has been advised by Herz (0.1 : 60 = a teaspoonful three times 
daily). Derivatives may also be tried. But the greatest care is necessary in 
making local applications to the diseased part. Raynaud once saw asphyxia 
of the hands immediately change to gangrene while in a local mustard bath. 
Treatment by laxatives is better: Laxative waters, Carlsbad salt, Marienbad 
tablets, etc. Hydriatic procedures are often of advantage. I have repeat- 
edly seen good results from lukewarm hand baths, sometimes with the addition 
of table salt; Tallermann's hot air apparatus may be tried. Mild massage 
is also beneficial. Electricity was praised by Raynaud; but a remedy to 
attack the affection at its root we do not possess. Sometimes amelioration 
follows the use of the faradic or galvanic hand bath. Herz advises galvaniza- 
tion of the head, the intensity of the current to be gradually increased. In 
the last few years I have several times seen good results in a form of local 
hyperemia from bandaging the upper arm with a firm flannel bandage so 
that distinct venous stasis appeared. This was done daily for a few weeks, 



ERYTHROMELALGIA 1 139 

the bandage being kept on for about ten minutes. Courtney reports favor- 
able results after the application of a tourniquet. The symptomatic treat- 
ment of the pain and gangrene requires no special description. 

Erythromelalgia. — In 1872 Weir Mitchell 1 described a pathologic condi- 
tion characterized by the paroxysmal appearance of pain, reddening and 
swelling of the feet, to which he gave the name erythromelalgia, "red, 
painful limbs/' Lannois 2 added to our knowledge of this affection by his 
description in 1880 of this unquestionably very rare affection; up to the 
present time perhaps 90 to 100 cases in all have been reported. In regard 
to the age and sex of those attacked by the disease, nothing noteworthy is 
reported. As deleterious agents, thermic influences are especially to be con- 
sidered. The predominant symptom of the disease is peculiar pain, a burning 
and stabbing sensation of great intensity, " as if living fire were under the 
skin." The pain either sets in with great severity or develops gradually to 
its height ; it is increased by letting the painful member hang down, by heat, 
and by exertion. Simultaneously with the pain or a little later redness and 
swelling appear. The redness is that of active hyperemia, the affected areas 
are light red to purplish red, the arteries pulsate, the veins are dilated. 

The temperature in the diseased parts is increased, being 5° to 8° F. 
higher than in the normal parts. In contrast with this, during the subse- 
quent course or sometimes with the appearance of cyanosis, the temperature 
declines. 

The sensitiveness of the affected areas is increased, even the pressure of 
the bed-clothes annoying the patient; stockings and boots can no longer be 
worn. Otherwise, sensation is usually normal. The disturbances occur 
periodically, and are produced by a number of causes : By heat, by a pendant 
position of the affected portion, and by movements. Most frequently the 
feet are implicated, sometimes the toes, sometimes the ball of the foot, some- 
times the heel. Usually both feet are symmetrically affected, more rarely 
both hands, or merely one foot or one hand may be involved. Sometimes 
the disturbances are limited to the region of a single nerve. As accompany- 
ing symptoms I must mention disturbances in the secretion of sweat, usually 
hyperhidrosis. ISTot rarely trophic disturbances are present, although this 
was formerly denied. There are usually dystrophic changes which are only 
slightly conspicuous; hyperplasia of the connective tissue, changes in the 
nails, and either thickening or atrophy of the skin. Gangrene has also been 
mentioned. In my opinion the • cases in which gangrene appears do not 
belong to this affection. I believe they should be attributed to endarteritis 
obliterans or to intermittent claudication. In a few instances, however, true 
neurotic gangrene was present. These then are the transitional cases of Ray- 
naud's disease. I observed a case in which the symptoms of both neuroses 
were combined. 

The pure cases in which only the local symptoms are present form about 
one-fourth of all the cases reported. Those which show signs of a general 
neurotic diathesis may be determined in about the same proportion. The 

i Weir Mitchell, " On a Rare Vasomotor Neurosis of the Extremities." Philadel- 
phia Med. Times, 1872, p. 81. 

2 Lannois, " Paralysis vasomotrice des extremites ou erythromelalgie." These de 
Paris, 1880. 



1140 VASOMOTOR TROPHIC NEUROSES 

cardiac and vascular nervous systems are sometimes most intensely damaged, 
and we also see the symptom-picture develop upon the base of marked hysteria. 
It is also noted in organic diseases of the brain: In multiple sclerosis (Col- 
lier), in tumors (Schlesinger), in gliosis spinalis (Pospelow), and in tabes 
dorsalis. 

Finally, there are cases in which the symptoms of erythromelalgia are 
limited to the region of one or a few definite peripheral nerves without other 
symptoms of neuritis being discernible. The regions of the median, the 
internal plantar, the posterior tibial, and the radial nerves have thus been 
found diseased. 

Pathologic anatomy has furnished no conclusions regarding the nature 
of the disease, although a few investigations have been reported. Changes 
have been found in the peripheral nerves of the posterior roots and in the 
small arteries, but there is no unanimity between the individual reports. The 
symptoms appear, as a rule, to be the expression of irritation in definite 
vasomotor (dilator), sensory, and secretory tracts and centers. In one group 
we may assume that the pathologic focus is situated in the peripheral nerves, 
and these are the cases in which the symptoms are limited to a circumscribed 
region of one nerve. In the future we must observe whether this is not, on 
the contrary, a root distribution; disease of the posterior root appears to be 
a very plausible theory for these cases. In the second group the assumption 
of a central nervous genesis appears to be best founded. The areas attacked 
are apparently of the same physiologic importance as those of the first group : 
Vasomotor, secretory, and sensory elements. Here then we are dealing with 
a central (spinal or bulbar) neurosis with vasomotor, trophic, sensory, and 
secretory symptoms. Whether the posterior gray substance is, as Eulenburg 
and recently also Lannois and Porot have believed, the point of lesion is still 
undecided. That a disease of peripheral vessels may produce similar phe- 
nomena I cannot deny. In this instance it may be as it is in various forms 
of muscular atrophy in which the primary muscular and primary neuropathic 
symptom pictures closely resemble one another. At all events, it appears to 
me necessary to regard a number of cases of erythromelalgia as nosologic 
entities. 

The diagnosis is not difficult if we bear in mind that we are dealing with 
a combination of vasomotor and sensory symptoms which form the picture 
of the affection. Vasomotor disturbances alone are not sufficient basis for 
a diagnosis. From them may result such pathologic pictures as erythroder- 
mia, erythromelia and the like. Nor are merely sensory symptoms sufficient. 
Here we must differentiate between the more or less substantive topoalgias 
of the foot (tarsalgia, talalgia, etc.). As a rule, the differentiation between 
certain vascular processes causes the greatest perplexity. Reflex disturbances 
of innervation apparently occur in a vessel changed by arteriosclerosis, and 
this leads to active hyperemia and thence to pain. This furnishes an ex- 
planation of the many points of similarity which exist between this affection 
and intermittent claudication. 

The course is usually very protracted, but improvement may take place 
even after a long duration of the affection. In a purely symptomatic sense, 
we attain most in the treatment by keeping the member in a horizontal 
position, and by the application of cold in some form. Electrical treatment 



SCLERODERMA 1141 

should be tried (galvanic local baths). Operative treatment such as neurec- 
tomy lias been several times proposed, and nerve stretching has been recently 
performed. The results of these operations are not particularly brilliant; 
nevertheless, in very stubborn cases it seems that nerve stretching should be 
at least tried. 

Scleroderma. — The symptomatology of Raynaud's disease has made us ac- 
quainted with certain nutritive disturbances in the skin of the peripheral 
parts, which we designate as sclero dermatic. There is also a disease in which 
this trophic disturbance of the skin is the most conspicuous symptom. This 
disease, scleroderma, is no doubt related to Kaynaud's disease, at least to 
some of its forms, while others deviate so far that a combination of the path- 
ologic pictures does not appear feasible. 

Scleroderma appears at all ages, but preferably in the third and fourth 
decades of life. Women are most frequently affected. In the etiology thermic 
causes are especially to be mentioned: Damp dwellings, working in the wet, 
a fall into water, etc. Psychical shock occasionally appears to have some 
influence, especially when it occurs in persons with an acquired or congenital 
neurotic diathesis. Barely has an organic nervous disease been found. Occa- 
sionally it is combined with Graves' disease, Addison's disease, or hemi- 
atrophia facialis. 

The first symptom noted in a patient suffering from scleroderma is a 
change in the skin, .which becomes hard, firm, and tense; often there is a 
marked luster, a glass-like transparency, and occasionally it looks as if 
lacquered. It gives us the impression of being too tight for the parts in- 
cluded within it, like a too-narrow glove; the small folds of the skin disap- 
pear, and if these changes affect the skin of the face it assumes a peculiar 
mask-like expression. The appearance of such a patient with his immovable 
features, his narrow pointed nose, his drawn and contracted mouth, eyes 
which can be closed onby with difficulty, and the glistening luster of the skin, 
is exceedingly characteristic; the picture is impressed upon every one who 
has once seen it. Occasionally an edematous stage precedes this indurative 
stage. During this time the affected area of the skin is somewhat swollen 
and edematous, but there is no pitting upon pressure with the finger. The 
changes in the skin vary in different cases. They may appear acutely, and 
increase greatly in intensity and extent in the course of a few days or weeks, 
or may gradually develop in the course of years, and show a steady progress. 

Often a patient will show abnormal pigmentation, partly pigment atrophy, 
partly pigment hypertrophy, in various areas of the body. Pigmentation may 
become so marked as to suggest a combination with Addison's disease, but 
usually true Addison's disease does not exist. 

The secretion of sweat and of the sebaceous follicles is disturbed as well 
as the growth of the hair; more significant is the vaso motility. We meet 
with local cyanosis and local syncope as well as with local hyperemia, and a 
form of scleroderma, namely, sclerodactylia, is marked by the fact that 
vasomotor symptoms appear early and either persist for a long time, or per- 
manently. Local changes in the temperature of the skin are frequently 
noted in these forms. 

Sensory disturbances play no great role. Paresthesia sometimes appears 
at the onset. It is usually not limited to a certain nerve region but is diffuse. 



1142 VASOMOTOR TROPHIC NEUROSES 

There are rarely any objective sensory disturbances, even in the parts which 
are most markedly altered. 

Trophic changes are not limited to the skin but affect also other tissues, 
and in the light of most recent investigations we may state that all the tis- 
sues of the body are affected. The changes, as a rule, pass directly from the 
skin to the muscles, but occasionally the muscles are affected in areas in which 
cutaneous changes are either slight or wholly absent. I recently observed a 
case in which the myoscleroses were much more extensive and intense than 
the dermatoscleroses. Of course this increases the difficulty of differentiating 
myositis interstitialis, muscular induration. Furthermore, there is atrophy 
of the bones with shortening and thinning (acromicria). I was several times 
able to determine this in the X-ray picture. If the joints are implicated, the 
differentiation from chronic articular rheumatism becomes very difficult. 
Only exceptionally are the mucous membranes affected. A significant fact 
in the prognosis is that the internal organs may be affected by sclerotic and 
atrophic processes, and cachexia often appears in the later stages of the 
disease; thus scleroderma finally becomes a systemic affection. 

The manner in which sclerodermatic changes appear has led to the sub- 
division of scleroderma into distinct forms. Scleroderma of the entire body 
is quite rare; in such extreme cases the individual resembles a mummy, a 
condition Grasset has described by word and picture. In contrast to this 
scleroderma diffusa, which is not always complete, is scleroderma circum- 
scripta, in which we recognize the striaform or flake-like arrangement of the 
sclerodermatous plaques. Under diffuse scleroderma, owing to its special 
localization, we differentiate sclerodactylia which chiefly affects the hands, 
more rarely the feet. This latter form bears, as I have several times indi- 
cated, the closest relation to other vasomotor trophic neuroses. It is cer- 
tainly not a disease sui generis, but besides its localization it has a few indi- 
vidual characteristics which I must mention: The frequent implication of 
the deep-lying tissues, the tendons, fascia, muscles, joints and bones, the 
well marked vasomotor and sensory disturbances which often accompany 
these conditions, the frequent deviation of the trophic disturbances from the 
true sclerodermatic type so that, besides scleroderma, more or less distributed 
gangrenous processes also occur. These cases are so closely related to Ray- 
naud's disease that, not rarely, their positive differentiation from it is im- 
possible. 

The relations between scleroderma and hemiatrophic faciei progressiva 
are very close. Hutchinson simply classifies the latter as scleroderma local- 
ized to the region of the trigeminal nerve. It usually affects young persons, 
most commonly those between 10 and 20 years of age; we are uncertain as 
to the cause, but a neurotic predisposition appears to play a role. It begins 
gradually; in a limited area, perhaps in the brow or cheek, there is atrophy 
of the skin, as well as of the subcutaneous tissue, the bones, and also the 
muscles. From this starting point atrophy involves gradually the entire 
half of the face; there is a diminution of the size of all parts. Changes in 
the pigment, and anomalies in the secretion of sweat, in the secretion of the 
sebaceous glands, in the temperature of the skin, and in the growth of the 
hair appear, similar to those of scleroderma. Just as in the latter affection, 
there are no objective sensory disturbances, but paresthesia and even severe 



SCLERODERMA 1143 

pain resembling true neuralgia of the fifth, nerve are frequently observed in 
the diseased area. I recently saw a case of scleroderma of the trunk and of 
the extremities without hemiatrophy combined with neuralgia of the fifth 
nerve. The combination of scleroderma and hemiatrophy has been reported 
by various authors, and in the main the opinion appears to be correct that 
hemiatrophy is only a specially localized form of scleroderma. There are, 
however, cases of scleroderma in which the sclerodermatous changes are lim- 
ited to the region of one or a few nerves. Recently the distribution of sclero- 
derma throughout certain root territories has been recognized. 

In the majority of cases the course of scleroderma is exceedingly chronic. 
Cases lasting thirty and even forty-eight years have been described. In these 
chronic cases the progress is not always steady; there are stages of arrest 
and even of apparent cure; but these are most common when the changes 
have appeared acutely, especially in children. Sclerodactylia shows the least 
tendency to recover. Death may be due to cachexia or to the implication of 
vital organs. More frequently the patients succumb to some intercurrent 
affection. 

I shall not minutely discuss the local and anatomical changes of the skin. 
They are chiefly evident in the corium, but are also found in the subcutaneous 
cellular tissue; they lead to flattening of the papilla, to an increase of the 
substance which produces cement, and an increase of the connective tissue 
trabecular Of other tissues which are implicated, the vessels must be espe- 
cially mentioned. "We find fibrous changes in all of the three coats of the 
small cutaneous arteries, while in contrast to this the peripheral nerve 
branches are usually normal. The process in the muscle is designated as 
myositis interstitialis. Other changes (as in the bones, joints, and internal 
organs) are usually due to inflammatory hyperplasia of the supporting tissue 
with subsequent cicatricial atrophy. So far as we know, the central nervous 
system shows no important anatomical change. The anatomical findings a 
few times noted are apparently without significance in regard to the nature 
of the disease, although they are quite necessary in the individual case. 
Occasionally, as in a case reported by Westphal, these appear merely to indi- 
cate a localization of the sclerodermatous process in the brain. At all events 
we have nothing but hypotheses as to the nature of the disease, and I shall 
not detail those which have been utilized to explain the affection; my opinion 
is that the disease has its seat in the sympathetic nerve of the central nervous 
system and that it is to be regarded as an angiotrophoneurosis, as also is Ray- 
naud's disease which is closely allied to it. 

The diagnosis rarely causes perplexity if the characteristic cutaneous 
changes can be determined. When these are not so prominent, and there 
are alterations in the deeper lying structures, especially the joints, bones, 
and muscles, its recognition is difficult; in this case, as has already been 
stated, there are transitions into and relations to chronic articular rheu- 
matism, to myositis interstitialis, etc. Among organic nervous affections, 
the differentiation from Morvan's disease is chiefly to be considered. In 
scleroderma, or sclerodactylia which is here most in point, the absence of 
sensory disturbances, of muscular atrophy and paralysis, is characteristic. 
The differentiation from leprosy is of primary importance. Treatment has, 
in the main, not been successful, but good results have sometimes followed 
74 



1144 VASOMOTOR TROPHIC NEUROSES 

certain methods. For a time treatment by thyreoidin was employed. This 
was based upon very uncertain theoretic conclusions, and the result of the 
treatment has been quite problematical. Adrenal extract has also been un- 
successful. Of drugs used internally salol or the preparations of salicylic 
acid have occasionally been beneficial. Thiosinamin in a 15 per cent, alco- 
holic solution has several times been highly praised; as it is generally used 
to loosen cicatricial tissue, its employment in scleroderma appears rational. 
Massage is unquestionably of use, as are also hydriatic procedures, such as 
warm douches, and peat, sulphur and ichthyol baths. Neumann strongly 
advocates hot air treatment in Tallermann's apparatus. Brocq states that 
he has seen brilliant results from electrolysis. 

Acute Circumscribed Edema. — In 1882 Quincke 1 described a new path- 
ologic picture, the main symptom of which was transitory edematous swell- 
ings in circumscribed areas. Although some authors had published cases be- 
longing to this category before Quincke's report appeared, yet the attention 
of physicians was generally directed to this affection solely by Quincke's re- 
searches, and these were followed by a number of publications. The disease 
has been known by various names : Quincke called it " acute circumscribed 
cutaneous edema." Among other designations I shall mention only that of 
Schlesinger, 2 the one most often employed of late, "hydrops hypostrophus." 
I call the disease " acute circumscribed edema/' By this I intend to indicate 
edema, not cutaneous edema, and that the disease occurs in many other por- 
tions of the body besides the skin. If we wish to attach an author's name, 
the affection should be called " Quincke's disease." 

It is not a rare affection. With a great number of nervous patients, we 
may meet it every few months. It generally occurs in youthful persons. 
From 130 cases I calculated an average age of 25.8 years; after the fortieth 
year the number of cases decreases rapidly, the aged being almost entirely 
exempt. 

The affection belongs to those which we designate as family affections; 
not rarely it occurs in several members of the same family. Osier has pub- 
lished the ancestral tree of a family in which this disease appeared in five 
successive generations. Other cases of neurotic predisposition are also men- 
tioned ; migraine was often observed in the ascendants. As a factor which 
immediately produces the disease we occasionally find exogenous intoxication. 
Alcohol is often mentioned, also carbonic oxid poisoning. In contrast to the 
closely related urticaria, there is no decided influence from the ingestion of 
certain foods. Occasionally the eating of fish or mushrooms has been re- 
ported to be the cause. 

The relation of the affection to nervous diseases is especially interesting. 
Transitory swellings have been observed in tumors of the spinal cord, in 
tabes (especially as an accompaniment of the lightning-like pains and con- 
fined to their area), and in gliosis, more commonly in some neuroses, in 
neurasthenia, and in hysteria, but most often in migraine and in Graves' 
disease. In all of these instances the edema apparently had only the value 
of an incidental symptom. Thermic agents were reckoned as the immediate 

1 Quincke, " Ueber acutes umschriebenes Hautodem." Monatsh. f. prakt. Dermat., 
Juli, 1882. 

2 Schlesinger, " Hydrops hypostrophus." Munch, medic. Wochcnschr., 1899, Nr. 35. 



ACUTE CIRCUMSCRIBED EDEMA 1145 

cause, especially the effect of cold, and no matter how skeptical we may be, 
was undoubtedly the etiology in a number of cases. Under these circum- 
stances, it is frequently only the parts of the body not covered by clothing, 
therefore, the face and hands, which are attacked by edema. I recently saw 
a woman in whom edema developed after exposure to a severe hail-storm. 
Only the parts which the patient could not protect were affected. Here, in 
addition to the influence of cold, local trauma may have played a role, as it 
has occasionally in other diseases. In a closely related affection, designated 
epidermolysis bullosa hereditaria, the causative effect of slight trauma is well 
known. Anger and excitement also favor the appearance of edema. 

The chief symptom of the disease is a rapidly developed swelling, which 
is more or less sharply defined, is pale or of normal color, in rare cases some- 
what red, and soon produces a feeling of tension, sometimes with marked 
itching; the impression of the finger does not cause pitting, or for but a 
very brief time, and the swelling declines after a few hours or days. When 
it disappears in one part, it reappears in others, and the disease is usually 
not exhausted in one attack, but there are intermissions followed by a varying 
number of renewed attacks. 

The extent of the swellings may vary. They may be as large as a pea 
or hazelnut, at other times the size of a plate or even larger. They are 
situated in the skin and in the subcutaneous connective tissue, but apparently 
may also be in the deeper parts and in the periosteum. In consistence they 
are coarse, elastic, somewhat like a contracted muscle, and, as previously 
mentioned, the pressure of the finger produces no prolonged pitting. 

The color of the skin is usually paler than normal, occasionally there is 
a slight or marked red discoloration. The temperature of the skin usually 
corresponds to the degree of redness. In rare cases there are extravasations 
of blood which cause the edema to assume a blue or bluish red color. There 
may also be severe cutaneous hemorrhages, yet the edema be slight. Such 
cases, however, should be regarded as purpura. 

The acute development of the swelling is an important symptom. In a 
few moments decided edema may appear; as a rule, however, hours elapse 
before the maximum is attained. The swelling remains at its height for 
some time, and then disappears without leaving a trace except that, should 
this area be re-attacked, permanent changes may gradually take place, but 
are usually not very marked. 

Subjective symptoms may be entirely absent. With decided swelling 
there are, of course, some symptoms such as stiffness and difficulty in moving 
the affected parts. Sometimes, however, the edema is accompanied by intense 
burning and itching, hence the pathologic picture closely resembles ordinary 
urticaria, especially if, instead of pallor of the skin, we have dark red, burn- 
ing edema, therefore true urticaria. By transitional stages the two diseases 
merge into each other. 

Edema may appear in different areas; some portions of the body are 
more commonly implicated than others, but no part is exempt. According 
to Collins, 1 among 71 cases in 29 the first swelling was in the face, in 22 

i Collins, " Angioneurotic (Edema." Am. Journ. of Med. Sciences, 1892, CIV, 
p. 654. 



1146 VASOMOTOR TROPHIC NEUROSES 

in the extremities, in 3 in the genitalia, in 3 in the back of the neck, and 
in one behind the ear. There seems to be no preference for the projecting 
parts of the body, and the edema is rarely symmetrical. The area in which 
the edema first appears often becomes less resistant to later attacks. These 
cases with definite localization do not warrant the construction of special 
pathological forms. Transitory edema of the eyelid is undoubtedly a variety 
of this affection. 

Edema may also attack the mucous membranes, and most frequently that 
of the mouth, pharynx and larynx. Edema of the larynx produces very 
marked, and sometimes alarming symptoms, such as difficulty in deglutition 
and dyspnea. In such cases scarification may become necessary; according 
to recent reports there is no doubt that edema of the larynx so produced may 
be the immediate cause of death (cases of Collins, Mendel and Straussler). 
Fortunately, these are great exceptions. Even edema of the lung was once 
regarded as a partial phenomenon of acute circumscribed edema (Quincke 
and Gross). 

Of other mucous membranes which are attacked I must mention the con- 
junctiva and the mucous membrane of the nose (nervous coryza), etc. Cer- 
tain intermittent gastrointestinal symptoms the genesis of which is not quite 
clear are important; for instance, pain, persistent vomiting, meteorism, con- 
stipation, exhaustive diarrhea, extreme and burning thirst. The gastric 
symptoms may be so severe as to closely simulate the picture of a gastric 
crisis. 

Schlesinger observed paroxysmally a swelling of the tendon sheaths. I 
saw several times, particularly in one case, peculiar painful contractions of 
certain muscles, among others the biceps; the muscles were very sensitive 
to pressure, but this condition lasted only a few hours. From the entire 
course of the case I concluded that the condition was analogous to edema 
(edematous infiltration of the muscles). Another localization is important, 
particularly because it forms a new disease: Intermittent articular dropsy 
(hydrops articulorum intermittens). This is a very rare symptom-complex, 
but is usually seen in nervous individuals. The chief symptom is swelling 
of one or several joints, almost invariably of one or both knee-joints. The 
swelling is often painful; there is no fever; the symptoms yield after a 
short time, but reappear at regular or irregular intervals. Schlesinger first 
called attention to the fact that this intermittent articular dropsy is merely 
a variety of hydrops hypostrophus, as is proven by the transitory swellings 
of the skin in this affection, and by the fact that there is articular swelling 
in ordinary circumscribed edema. 

Certain cerebral symptoms must be regarded as indicating the special 
localization of transitory edema. Lassitude, somnolence, headache, even 
serious symptoms of cranial pressure, loss of consciousness, slow pulse, and 
general convulsions have all been observed under these circumstances, and 
the symptoms were consequently referred to effusions in the meninges which 
were analogous to cutaneous edema. In some cases polyuria and transitory 
albuminuria have been observed and, exceptionally, paroxysmal hemoglobinu- 
ria. I have reason to believe that the latter occurred in one of my cases. 

Sometimes the attacks begin with general symptoms: Lassitude, malaise, 
loss of appetite, slight fever (in one case I found 100.8° F.). 



ACUTE CIRCUMSCRIBED EDEMA 1147 

Quincke's disease shows certain relations to, and transitions into, other 
vasomotor trophic neuroses. I have several times seen the combination of 
transitory edema and acroparesthesia. In one of my cases there were typical 
symptoms of Raynaud's disease : Asphyxia and local syncope of the finger, 
and more rarely of the toes, alternated with transitory edema in all parts 
of the body. In these patients we occasionally find other evidences of the 
want of stability of the vasomotor system: Transitory erythema, dermo- 
graphia, cardiac palpitation, vertigo, rush of blood to the head, and tinnitus 
aurium; therefore the picture of vasomotor ataxia (Solis-Cohen, Herz) which 
was previously described. 

The course of the disease is often exceedingly chronic. Relapses con- 
stantly occur, between which there may be free intervals of considerable 
duration. 

The prognosis in regard to life is good, but, as already stated, life may 
be endangered by the laryngeal involvement and sometimes by pulmonary and 
cerebral symptoms. 

As to the pathogenesis of the affection, I must state that it is impossible 
to explain the pale edema merely by the assumption of a change in the cir- 
culation of the blood, but that unquestionably another factor is operative. 
This must be sought either in the fact that a nervous influence upon the 
capillary cells increases the secretion of lymph or that other nervous influ- 
ences produce a change in the composition of the walls of- the vessels, so that 
these permit more of the transudate to pass through than under normal 
circumstances. It must also be borne in mind that the so-called internal 
symptoms of the affection cannot be attributed to edema of the affected 
mucous membranes. Therefore acute circumscribed transitory edema is not 
simply an angioneurosis ; on the contrary, besides the undoubted disturbance 
of the circulation of the blood other factors are active which must be re- 
garded as trophic or secretory disturbances. In transitory edema we are not 
always dealing with a disease sui generis, but it is sometimes merely of 
symptomatic value, as has already been stated. At all events, the view that 
Quincke's disease is in its nature a vasomotor trophic neurosis appears to me 
quite justifiable. 

The diagnosis causes little difficulty inasmuch as we are not dealing with 
a modification of a similar pathologic picture; I need not discuss this point. 
The differentiation from hysterical edema occasionally causes some perplexity. 
The main point is this, that in hysterical edema a multiplicity of swellings 
is quite unusual. Moreover, in hysterical edema we are most likely to find 
local sensory symptoms. 

Meige described under the name " trophatdema chronicum hereditarium " 
a chronic neuropathic edema which develops gradually, almost invariably 
affects the lower extremities bilaterally, and shows a marked tendency to 
appear in families. The dissimilarity to transitor}^ edema is obvious on com- 
parison. With a rare localization of the edema we can of course only make 
a certain diagnosis if, while under observation, typical cutaneous edema 
finally appears. 

In treatment most value is to be attached to the regulation of the diet 
and to the general management of the neurotic condition. All articles of 
food which formerly proved injurious must of course be avoided. The regu- 



1148 VASOMOTOR TROPHIC NEUROSES 

lation of the bowels is most necessary, either by drugs (menthol and camphor 
are advised as intestinal antiseptics) or by spa treatment in Kissingen, etc. 
Strychnin, quinin, atropin and arsenic have all been praised for internal 
medication; I have several times used the latter with success. Electricity, 
massage, and hydriatic procedures deserve consideration. That tracheotomy 
is sometimes indicated I need scarcely mention. The gastrointestinal symp- 
toms occasionally necessitate the use of morphin. 



OCCUPATION NEUROSES 

By R. CASSIRER, Berlin 

In another article in this volume I have described a pathologic condition 
which I designated as professional neuritis (occupation paresis or professional 
paresis). We defined professional paresis as an affection due to continuous 
and uniform employment giving rise to damage of the peripheral nervous 
system in which there are also other clinical manifestations of neuritis. Over- 
exertion, which may be primarily regarded as the cause of occupation paresis, 
may also produce other damage, with which we must concern ourselves some- 
what more in detail. 

WRITER'S CRAMP 

The patient is a man, aged 21, a clerk by occupation. He first appeared for treat- 
ment in April, 1905; he came to us because, in the last few months, he was no longer 
able to write, after having been engaged in writing for the last 5 years. During the 
last year he had been writing about 10 or 11 hours daily. The disturbance appeared 
gradually, the writing became uneven, tremulous; the patient was no longer able to 
write fluently and became tired much sooner than formerly. In addition there were 
pains upon the inner surface of the hands and upon the back of the hand, and, finally, 
a spasmodic condition of the thumb appeared. This member was drawn away from the 
penholder, so that it fell out of the patient's hand. He attempted to continue writing, 
by holding the penholder between the 3d and 4th fingers. 

Finally this had to be given up and since a few days he is no longer able to 
Write at all. The disturbances in the use of the hand are entirely limited to the 
faculty of writing. In other operations, such as in eating, in carrying objects in the 
right hand, and even in piano playing there is no difficulty. Questioned in regard to 
other symptoms he reports that he is easily excited, somewhat nervous, and often 
suffers from headache. His father suffers from migraine but all the other members 
of the family (3) are well. He has had a curvature of the vertebral column since his 
9th year, which is said not to be hereditary in the family. If we ask the patient 
to make an attempt at writing, he presses the penholder between the first 3 fingers, at 
first, almost in a normal manner. Shortly the penholder is held spasmodically, but 
it is still possible for the patient to write a few words although slowly and with 
uneven letters. Very soon the thumb or the third finger slips away, interrupting the 
writing. Each renewed attempt is rendered futile in the same manner. A minute 
examination of motility and sensation of the hand revealed no change from the normal. 
The muscles are neither atrophic, paretic, nor painful upon pressure. Nor are the 
nerve trunks especially tender upon pressure. There is no disturbance in the contact 
•or pain sense. Briefly, the entire objective finding of the right hand as well as the 
remainder of the body, with the exception of a very pronounced kyphoscoliosis, is 
entirely negative. In conclusion I shall report the further progress of the case. For 
9 months the patient was not permitted to write; his treatment consisted of galvanism, 
massage and especially resistance gymnastics. There has been some improvement; he 
is able to write a few lines, the handwriting being fairly good, but he is compelled 
to write slowly, Tbere can be no thought of resuming his occupation for a long time. 

1149 



1150 OCCUPATION NEUROSES 

The preceding history illustrates a typical case of writer's cramp (grapho- 
spasm, mogigraphia) . The condition consists of spastic contractions in cer- 
tain muscle groups of the hand, which appear only when certain coordinated 
movements are attempted, such as writing. Corresponding to this relation- 
ship Benedikt chose the term " coordinative occupation spasm " for this affec- 
tion. Writer's cramp is the type of these coordinative occupation spasms. 
As the spasm is often of secondary importance in the malady and pain be- 
sides paralytic conditions appear from the onset the term " coordination 
occupation neuroses " was chosen for the entire group. The designation 
neurosis states at once, that according to our present knowledge, there is no 
underlying organic lesion of the nervous system. Charles Bell was the first 
to describe cases of this kind, Duchenne 1 adding greatly to our knowledge 
of the malady. The latter author called the condition " function spasm " 
and " function paralysis." He states that it is an affection that only appea s 
when definite movements are practiced, being characterized partly by spas- 
modic, tonic or clonic, painful or painless contractions, partly by tremor and 
partly by paralysis. Here we encounter the various subvarieties of the 
malady, the knowledge of which we owe to Benedikt, Berger, Remak and 
Bernhardt. (Complete report of the literature will be found in Remak, 2 
and Bernhardt. 3 ) 

Regarding the various forms in which the affection appears, our case is 
an example of writer's cramp in the strictest sense, in which actual spastic 
conditions appeared in separate muscles and muscle groups. As is the case 
in the majority of instances our patient showed spasmodic flexion in the 
thumb and index finger rendering writing impossible. Other fingers may 
also be attacked by the same spasm. Thus the little finger may be drawn 
into the palm of the hand. An immoderate flexion of the hand may prevent 
the act of writing. In other cases spasmodic extension of the long extensors 
of the fingers, and spreading of the fingers, so that the pen slips out of the 
hand, may prevent writing. The hand may actually be raised from the 
paper by spasm of the supinator. Duchenne reports 2 cases, where there was 
a supinator movement in the hand as soon as an attempt was made to write 
so that the point of the pen was directed upward; the patients were unable 
to alter the spasmodic position of the hand. I have never seen spasm in 
the muscles of the shoulder in my cases; these are said to occur, but only 
in the later stages of the affection. 

The spasmodic condition is not always so restricted and distinct as in the 
case just reported. Very often sensory irritative symptoms are present, either 
accompanying the spasm or appearing alone. 

A police assistant, aged 21, complained of severe pain, which originated in the 
ball of the small finger, radiating distally and proximally. In addition there were 
spasms, the little finger being retracted in the hand, which held the pen firmly upon 
the writing paper. An attempt at writing which the patient made in my presence, 
proved the correctness of his description. It was impossible for him to write more 

i Duchenne, " L'Electrisation localisee," 2. Edition, p. 928. 

2 Remak, " Beschaftigungsneurose." " Eulenburg's Real-Encyklopadie," 1894, III. 
Aufl., Bd. Ill, p. 270. 

3 Bernhardt, " Die Erkrankung der peripherischen Nerven." I. Aufl., II. Theil, 
p. 180. 



WRITER'S CRAMP 1151 

than a few words. The local condition in this case was also entirely negative. Motion 
and sensation are quite intact, there is nowhere sensitiveness of the nerves or muscles 
upon pressure, no thickening or other pathologic condition. The patient was an abnor- 
mally large man — nearly 6 feet — with poor constitution, his mother being of a nervous 
temperament and suffering from headache ; in addition the patient complained of gen- 
eral neurasthenic symptoms. For some years he has been compelled to write for eight 
hours daily in his occupation besides having to do similar work at home. 

These pains usually show no exact localization, they do not follow the 
course of a nerve, but radiate from the point of greatest pain in a diffuse 
manner both distally and proximally; the pain is present in the fingers, 
sometimes reaching the shoulder and the back. Pain upon pressure is not 
present in the majority of cases, but it is not uncommon to find one or more 
nerves abnormally sensitive upon pressure at one or the other point. I have 
already insisted upon the fact that, according to Oppenheim, this painful- 
ness upon pressure is quite common in neuropathically predisposed persons, 
without any local affection being present. Objective, sensory disturbances, 
according to general experience, are not present; they do not belong to the 
symptoms of this or any other form of occupation neurosis : this is one of 
the points by which these clinical pictures differ from occupation neuritis. 

Pain may be the only symptom of the disease — then we no longer refer 
to writer's cramp but to writers pain: but symptomatologic transitional 
stages between both groups, which apparently have identical pathogenesis, 
give us the right to speak of " writer's pain/"' In many persons the pain 
which .appears during writing and which soon prevents a continuance of the 
act is the only symptom; occasionally there is pain upon pressure over some 
of the nerve trunks. Remak and Bernhardt have shown that this pure, 
sensory, neuralgic form of occupation neurosis is even more common in piano 
players. The pain more often than the spasm is the cause of the disturb- 
ance in function. I must add that typewriters, as might be expected, show 
disturbances which are closely allied to those of piano players ; here, also, 
the pain is the significant factor ; but we shall return to this subject. 

Vasomotor symptoms are usually not prominent; only in rare instances 
is there a report of local syncope or local asphyxia. In a case reported by 
Brissaud, Hallion et Meige, 1 which presented many unusual features, the 
acrocyanosis was a symptom of coordinative importance. More often the 
patients complain of acroparesthesia, of tingling and numbness in the finger 
tips. That in some of the other forms of occupation neurosis nervous symp- 
toms of the kind just mentioned are very prominent and that in these in- 
stances the decision whether we are dealing with acroparesthesia, in the 
restricted sense, or occupation neurosis, is exceedingly difficult will be de- 
scribed later on. 

In addition to the spastic and the neuralgic forms of writer's cramp 
there are also tremor and paralytic varieties. The tremor form is quite rare. 

A court clerk, aged 48, presents a disturbance in writing characterized by tremor, 
which appears after the patient uses the pen for a short time. The patient states he has 
a sensation as if he grasped the penholder too firmly, almost spasmodically. The 
tremor is especially severe when the patient knows that he is being observed; this 

1 Brissaud, Hallion et Meige, " Acrocyanose et crampe des ecrivains." Arch, gene- 
rale d. medec, 1903, 37. 



1152 OCCUPATION NEUROSES 

report requires careful attention. He further states that recently, at night, his fingers 
become numb. He also suffers from general nervous symptoms. The patient is well 
nourished and other objective conditions appear to be normal. The character of the 
handwriting coincides with the tremor: it is zig-zag, but the individual lines are regu- 
lar, not elongated or interrupted. 

As a further and final form of writer's cramp the paralytic variety may 
be mentioned. It is very difficult to separate this form precisely. True 
paralysis is not present; on the contrary, there is here as in the other form 
a sensation of fatigue which soon renders writing impossible; in addition 
to this there is pain ; there is no tremor or spasm, " hand and forearm appear 
as if chilled, the pen rests upon the paper without being moved" (Bern- 
hardt). Occasionally there is a direct impression of an inability to write 
which produces the difficulty as in the case reported by Brissaud, Hallion et 
Meige. Meige, 1 in another article, refers to " aboulie motrice." At all events 
we are here very close to the symptomatologic limits of our theme. 

The positive phenomena of the affection are exhausted by what has been 
stated: spasm of the muscles, in which, as has been very aptly remarked, 
not merely the muscles that are concerned in writing contract, but also those 
that should not contract are affected; tremor, paretic sensations of fatigue, 
and pain, eventually also a certain tenderness upon pressure of the nerves 
and muscles; in addition it must be stated that all these symptoms appear 
only while writing, and only during this function. Naturally there are a 
few exceptions to this last rule. There are other activities in which the finer 
movements are necessary, such as piano playing, sewing, etc., in which the 
same symptoms may be present, while coarser movements requiring greater 
power do not give rise to symptoms. These exceptions are even more fre- 
quent in other occupation neuroses than in true writer's cramp. 

There are no exceptions to the rule that objective sensory disturbances, 
such as appear in neuritis, are constantly absent. A qualitative change of 
electric contractility such as partial DeR is foreign to the picture of grapho- 
spasm and in fact to all of the occupation neuroses. Slight quantitative 
changes of contractility are said to be present in rare instances, both as an 
increase and decrease of contractility, as well as an inversion of the normal 
formula, so that the anodal contraction is stronger than the cathodal. But 
this may be noted in the small muscles of the hand even under other condi- 
tions, for instance, when they are cold or cyanotic. The size of the muscles 
remains unchanged. 

In addition to writer's cramp all three of the patients whose history has 
been given showed other general nervous symptoms which, to a greater or 
lesser extent, correspond to neurasthenia. This coincides with the general 
experience that the majority of cases of writer's cramp develop from neuras- 
thenia. This neuropathic disposition may be congenital or acquired. A 
hereditary predisposition shows itself in the family appearance of the occu- 
pation neuroses. Gallard reported the cases of a notary, his mother and 
sister having also suffered from writer's cramp. One of my patients, a lad 
aged 15, attending school, had the sensory form of writer's cramp; his father, 
a piano teacher, had piano player's cramp. 

i Meige, " Cramps fontionelles et professionelles." Traite de medec, X, p. 344. 



WRITER'S CRAMP 1153 

Among the deleterious factors that may affect the patient, all that give 
rise to neurasthenia may be present: primarily over-exertion must be men- 
tioned. The work of a copyist is poorly paid, and as SeeligmuLler aptly re- 
marks, not authors out copyists are attacked by writer's cramp. The poor 
remuneration requires prolonged work, "over-time" and even in addition to 
this, very frequently, it does not even provide sufficient food; thus over-exer- 
tion and under-nutrition act together in the same individual. Night work, 
which is so common in these cases, is of especial import. In addition to 
writing there are also other occupations such as mental over-exertion, in pre- 
paring for examination, which are often of consequence in these conditions. 
After the malady has once developed, the fear of the social consequences 
(which are often quite serious) are a constant aggravating factor both to the 
writer's cramp and to the neurasthenia. The presence of other neuroses has 
also been noted : epilepsy, hysteria, tic 'convulsif, hemicrania, neuralgia. I 
saw a combination of graphospasm and meralgia paraesthetica. Oppenheim 
mentions an association with tabes and with poliomyelitis. It is, therefore, 
possible that organic diseases of the nervous system may prepare the ground 
for the development of the neurosis. 

On the other hand chronic intoxication, first alcoholism and then nicotin- 
ism, etc., may be important factors. 

Certain local changes appear to be of etiologic importance in some cases. 
Bernhardt reports the case of a policeman, aged 45, who for a long time had 
been occupied with writing; after having his right hand run over, he began 
to present tremor in the thumb and index finger while writing. In other 
cases injuries to the shoulder, the hand, the fingers, were the cause of the 
appearance of the affection. I have a patient with general well-developed 
neurasthenic symptoms, in whom a markedly developed Dupuytren's con- 
tracture was present in addition to the tremor form of writer's cramp. In- 
durations and thickening of the tendon sheaths have been regarded as the 
cause, as well as periosteal inflammation (periostitis of the external condyle 
of the humerus). Any of the changes may predispose to the development of 
the affection but they are hardly likely to develop it. 

The neurosis develops gradually under the influence of writing as an 
occupation upon the prepared groundwork. In some persons the regular 
work of writing 6-8 hours is sufficient, in others the frequent over-time has 
the injurious influence; upon the whole only a minority of those occupied 
with penmanship are affected. The malady is not very common. There can 
be no doubt that the style of pen and penholder may be of some influence in 
the causation of the affection; of most unfavorable import is the marked 
right angle position of the finger which fixes the pen. Writing teachers may 
do much to prevent the development of the malady in teaching their pupils 
a free position in holding the pen, avoiding too thin penholders, preventing 
the use of too pointed pens. The posture while seated during writing is also 
of importance. 

Although the development of graphospasm, upon the whole, is gradual, 
nevertheless in connection with exertion, psychical shock, etc., acute exacer- 
bations may occur. Oppenheim reports a case in which the affection ap- 
peared in immediate connection with attendance upon a spiritualistic 
meeting. 



1154 OCCUPATION NEUROSES 

The diagnosis presents no difficulties. Naturally difficulties in writing 
may occur under all possible varieties and circumstances; every localized 
paralysis, every spasm, every tremor, every change in sensation hinders writ- 
ing; but the cause may be determined from the individual symptoms and 
careful examination, at the onset of an organic affection, will reveal the 
reason for the changes. In these instances, not alone the writing but every 
other function, in which delicate movements are necessary, will be disturbed. 
The differentiation from occupation neuritis will hardly present difficulties; 
in the other professional neuroses (milker s spasm and similar conditions) 
a careful examination for sensory and motor symptoms of absence of func- 
tion and for qualitative electric changes will be sufficient for a correct diag- 
nosis. 

There is occasionally some doubtin deciding the question as to whether 
the writer's cramp originated from the neurasthenia or whether the condi- 
tion is one of neurasthenia with the prominence of local symptoms. Thus 
a simple neurasthenic or hysterical tremor may cause difficulty in writing; 
the tremor may attack the right hand, which has borne the brunt of the 
exertion alone or most markedly, and finally the difficulty may be more 
marked in writing than in any of the other finer movements. A decision 
is all the more difficult in these cases for the reason that there are very inti- 
mate relations between these conditions. It must be stated that these tran- 
sitional affections are even more common in the other varieties of occupa- 
tion neurosis, such as the neurosis of typewriters, piano players, telegraph 
operators, etc. 

Which of the varieties of writer's cramp is present is very easy to de- 
termine from the symptomatic criteria that have been described. In regard 
to the correct treatment it is necessary to determine not merely that the 
affection is one of writer's cramp, but to discover which muscles reveal im- 
moderate tension, where pressure points are situated, and so forth. 

The prognosis was formerly always regarded as unfavorable. Recent au- 
thors are, upon the whole, less pessimistic; from my experience I coincide 
with this latter view; I have frequently seen, especially in cases that were not 
too severe, great improvement even after work had been resumed. In very 
severe cases prolonged writing and especially that required by occupation 
is impossible, or if after a prolonged interval writing is resumed this must 
speedily be given up. Among those who, in order to resume writing, learn 
to write with the left hand, a large proportion suffer later on from similar 
conditions in the left hand. A better remedy for those who, on account of 
their social position, find it necessary to continue writing is to learn to use 
the typewriter. It appears that the disturbances that arise under these cir- 
cumstances are, upon the whole, milder or at least not so tenacious as in 
writing with the pen; but the fact that our experience in this field is still 
limited indicates that we must be cautious in giving an opinion of this kind. 

TYPIST'S CRAMP 

In accordance with an entirely different muscular activity in writing upon 
a typewriter, the disturbances are necessarily of a different nature and re- 
semble those that we meet with in piano player's and telegrapher's cramp. 



TYPIST'S CRAMP 1155 

In these forms, as it appears, the spastic phenomena are of a more secondary 
nature, the sensory-paralytic features dominating the scene. 

Lady, aged 38, for many years has been using the typewriter. For a year she 
has been suffering from pain in the right hand, which radiates from the middle finger 
to the elbow ; in addition there is an intense feeling of weakness in the right arm and 
often a sensation as if the fingers were stiff without there being actual spasmodic con- 
ditions. Her hands are very cold and, occasionally in the autumn, actual numbness is 
present in the fingers. While formerly — as is done by most persons — she used the 
right hand in writing, she now employs the left. In other uses to which the hand is 
put — carrying an umbrella, sewing — there is frequently a sensation of fatigue. She is 
easily excited, nervous and readily frightened. Examination revealed that at the tips 
of the first and second fingers pin pricks and contact with the brush were not distinctly 
perceived. But the skin at these points is also somewhat thicker. It is worthy of note 
that in writing short-hand and employing the pen there is but slight difficulty, but she 
never continues this for any length of time. 



I have seen quite a number of cases of this kind. They always occurred 
in the female sex; I emphasize this because formerly (see Bernhardt, I. c. 
p. 187) importance was attached to the fact that writer's cramp appeared 
almost exclusively in men; this is naturally due only to social conditions. 
The persons affected are usually pale, anemic, underfed individuals with the 
usual symptoms of general nervousness : headache, poor sleep, hyper-irrita- 
bility, and mental depression. These persons have, as a rule, been occupied 
in writing many hours daily for a long number of years (8 hours and longer 
upon the typewriter) ; in addition some of them, as the patient just referred 
to, have written with the pen, employing short-hand. 

The affection commonly begins with pain in the finger tips; at other 
times there is paresthesia, a feeling of numbness, tingling, formication and 
cold. Later the pain radiates to the shoulder and the back of the neck. The 
limitation of function is especially marked if in addition to the paresthesia 
and the pain there is fatigue which compels the patient to cease his activity 
after a short time. The patient often complains of spasmodic sensations, but 
I have never met with actual spastic conditions; nevertheless it cannot be 
doubted that these may occur. 

Objective sensory disturbances are sometimes present, such as a decrease 
of sensation in the finger tips. It must, however, be remembered that the 
skin is somewhat thicker at these points. 

Occasionally there are abnormal vasomotor symptoms, such as local syn- 
cope in the fingers. In these cases the differentiation from acroparesthesia is 
exceedingly difficult. Where the vasomotor symptoms appear in immediate 
connection with the occupation and disappear when the occupation ceases, 
they may be regarded as a symptom of the occupation neurosis; there are, 
however, cases in which there can be no positive decision. 

As the right hand is the member most in use, the symptoms are first noted 
there; as a rule, this member is the more skillful and better able to stand 
the strain. But the left hand is by no means exempt. 

It has already been stated that very frequently in this variety there is 
great fatigue in the performance of other activities: pain and numbness of 
the hands in dressing, arranging the hair, carrying an umbrella, in sewing, 
in piano playing, etc. 



1156 OCCUPATION NEUROSES 

There is great resemblance between writer's cramp and another occupation 
neurosis, telegrapher's cramp. This was first described by Onimus in 1874. 
Cronbach 1 a few years ago published a comprehensive study of the malady. 
I have seen a few cases of this kind. The affection usually occurs in men. 
Among the sensory symptoms there are pains of various kinds, dull, stabbing, 
pulling, boring. Further, there are sensations of cold, tingling, formication, 
hypersensitiveness and numbness, feelings of swelling and stiffness. Among 
the motor symptoms there are pareses of some of the fingers, fatigue of the 
entire hand, of the forearm as well as of the entire member. Spastic symp- 
toms are not prominent. Cronbach has reported a case in which the extensor 
tendons of the fingers revealed contractions. He emphasized the occurrence, 
in his cases, of vasomotor (cold, heat) and of secretory disturbances (abnor- 
mal sweating, abnormal dryness). He also noted pain and numbness of the 
hands in his cases even when the patient was not employed in his occupation. 
Among the objective symptoms there are pressure points along the nerves, 
the importance of which I have already considered. In some instances there 
were also pressure points in some of the muscles; in a case of Bernhardt's, 
in the sheaths of the tendons. In some instances in addition to telegrapher's 
cramp there was also writer's cramp, or this appeared later. Cronbach was 
able to demonstrate that not merely Morse's but also Hughes's apparatus is 
capable of developing the neurosis; hence, it follows that the advice to pa- 
tients to give up the Morse apparatus and employ the Hughes (as was for- 
merly done) is absolutely valueless. 

Another form of occupation neurosis is piano player's cramp. Here the 
activity of the muscles is similar to the previously mentioned forms, but the 
technic is very much more delicate and much more difficult, requiring a very 
much longer practice of a purely mechanical nature. Professionals are most 
commonly attacked, apparently oftenest during their student days. In addi- 
tion to intense and extensive activity they present the evidences of somatic 
and psychic symptoms. The clinical symptoms need not be described in 
detail. Among them are pains which are most prominent in the forearm, 
thence radiating upward and downward, these being the most important. 
The localization may vary, on the one hand, according to the technic em- 
ployed, and, on the other hand, according to individual circumstances (small 
hand, incomplete stretching of the fingers, insufficient power of individual 
muscle groups, etc.). Fatigue, which appears with abnormal rapidity, causes 
prolonged pauses; gradually fatigue, present even at the onset, renders play- 
ing impossible. In addition there are spasmodic conditions (extension of 
some of the fingers). Pain upon pressure is especially marked in those mus- 
cles which are implicated in the function. 



CRAMPS CAUSED BY PLAYING OTHER MUSICAL INSTRUMENTS 

The picture of the occupation neurosis in violinists, cellists, etc., is the 
same. In these the spasm may affect the hand that uses the bow or the left 
hand which comes in contact with the strings. 

i Cronbach, " Die Beschaftigungsneurose der Telegraphisten." Archiv fiir Psychi- 

i> YYYVTT rx VA1 



atrie, XXXVII, p. 243. 



OTHER FORMS OF OCCUPATION NEUROSIS 1157 

A cellist, aged 23, complained of pain in the last 3 fingers of the left hand, the 
pain having started in the middle finger. The pain resembled pin pricks and was most 
marked in the tips of the fingers ; gradually a sensation of numbness and tingling 
appeared. The patient believed that there was some loss of sensation in the tips of 
the fingers but this could not be demonstrated by objective examination. He com- 
plained of abnormal dryness in these fingers, in spite of the fact that there appeared 
to be an unusual amount of sweating. The difficulties were entirely limited to hi3 
cello playing. 

Remak has reported tenacious hyperesthesia of the pulpa of the left index 
finger in violin players; in a patient of Oppenheim's there was paresthesia 
upon every attempt to play the violin, which prevented the continuance of 
the playing, the patient having no other difficulty. Remak reported a case 
of flute player s cramp in an aged musician, the patient showing spastic phe- 
nomena in the left thumb and little finger. 

Thus the professional use of any instrument may give rise to similar 
symptoms, which need not necessarily affect the upper extremity. 

Oppenheim has reported an occupation spasm in the muscles of the lips 
in a cornet player; as soon as the patient attempted to play, spasm appeared 
in the orbicularis oris and the patient was unable to produce a tone. Whether 
spasm or paralysis was present could not be determined. Striimpell reported 
an occupation spasm of the tongue in a clarinet player; similar conditions 
have been reported in trumpet players by Diims, Kalmus and Stadler. 

CRAMPS OF THE LOWER EXTREMITIES 

Meige reports a case of liarp player's cramp, due to using the pedal of 
the instrument. Occupation spasms of the lower extremity are otherwise 
quite rare; a patient of Aldxich's was compelled to use the right foot daily 
for a number of years upon a pedal which worked a drum and kettledrum. 

Rigidity and stiffness, as well as fatigue and pain, appeared in the muscles 
thus employed. In workmen who are compelled to use pedals, lathe workers, 
knife grinders, sewing machine workers, similar conditions may develop. 
These cases must be carefully examined for the s}'mptoms of occupation 
neuritis. In professional dancers, such occupation neuroses, affecting the 
lower extremity, are also found. I have often noted a pain in the heel which 
presented all of the signs of an occupation neurosis. In a man who was 
compelled to stand for several hours daily in the same position, there was 
pain in the heel, which was not present under any other circumstances, such 
as prolonged walking or mountain climbing; in shop girls I have observed 
similar conditions. No local changes could be observed. Discontinuing the 
occupation was always of favorable influence. Bernhardt 1 has reported sim- 
ilar cases. 

OTHER FORMS OF OCCUPATION NEUROSIS 

As many forms of occupation neurosis are met with as there are occupa- 
tions. I must mention some of the most common. 

A patient, a jeweler, who was compelled to hold a tool firmly in his hand, 

i Bernhardt, I. c, p. 466. 



1158 OCCUPATION NEUROSES 

suffered from numbness in the little finger and in the ball of the member; 
there were no spastic symptoms, but, on the other hand, there was slight 
tremor. Meige observed the same symptoms in an engraver; Stephan reports 
occupation neurosis in diamond cutters. The same symptoms are common 
in persons who fold paper as an occupation. One of my patients who held 
the paper firmly with the left hand while he used the folder with the right 
hand complained of pain in the left elbow joint, thence radiating to the 
neck and region of the ear; in the right hand there was paresthesia. As he 
formerly suffered from neuralgia par aesthetic a this is interesting in a patho- 
genetic sense. In paper sorter's I have seen paresthesia; others have reported 
this in addition to spastic symptoms in money counters. 

Occupation neuroses have been reported in watchmakers. T. Cohn re- 
ported spasm of the orbicularis in a watchmaker; Oppenheim reports spasm 
of accommodation; similar conditions have been noted after the prolonged 
use of the microscope. A drill-ocular muscle spasm (spasmodic position of 
the eyes to the right upon rotation of the head to the right) is reported by 
Franjen. Oppenheim regards the nystagmus of miners as belonging to this 
category. 

The occupation spasms of seamstresses, tailors and cobblers affect the 
small muscles of the hand. Here sometimes the question of tetany may arise. 
The objective signs of the latter affection, especially Trousseau's sign, and 
the characteristic increase of electric contractility, will permit a differen- 
tiation. 

Oppenheim has reported 2 cases of barber's spasm. This consisted of 
spasmodic tension of the muscles of the hand and fingers, occurring in barbers 
upon any attempt at using the razor in shaving. 

Painful and spasmodic conditions appear in the muscles of the upper arm 
in connection with the labor of loch-smiths, blacksmiths, tin-smiths and car- 
penters. Duchenne reports the case of a tailor whose arm was rotated inward 
by contraction of the subscapularis ; a fencing master had the rapier rotated 
inward, at the same time presenting extension of the forearm. 

In the course of this article I have several times pointed out that it is 
necessary to discriminate between professional pareses and professional neu- 
roses. There are, however, cases in which, in addition to spasmodic and 
neuralgic symptoms, atrophic paresis is added. It has been known for a 
long time that in milkers spasmodic symptoms appear {milker's cramp) : 
there is painful spasm in the flexors of the hand and fingers upon any at- 
tempt at milking, sometimes associated with vasomotor, irritative symptoms. 
In the cases reported by Eemak, Stephan, Bernhardt, and myself, there were, 
however, in addition, degenerative-neuritic symptoms. Eemak insists upon 
the great importance of this fact in the consideration of some of the varieties 
of occupation spasm, as it lends support to the view that they originate from 
peripheral, irritative conditions and are maintained by these. Similar symp- 
toms have been observed in cigar-makers. Wood-sawer's cramp also belongs 
to the occupation spasms. 

B. Frankel reports a professional neurosis of the voice (mogiphonia). 
In analogy with writer's cramp he differentiates three varieties, spastic, 
tremor, and painful fatigue. The voice refuses to act in the professional 
employment; the laryngoscopic examination reveals normal conditions. 



TREATMENT 1159 

PATHOGENESIS 

The question of pathogenesis has just been touched upon. A few words 
more are necessary: it is very questionable whether this is the same for all 
of the varieties. It has already been stated that peripheral causes may be 
at fault; but this is merely the case in the minority of instances. On the 
other hand, it is sometimes the impression of " not being able " in addition 
to disagreeable peripheral sensations which are the causes of the affection. 
Between these extremes there are transitional forms; but I shall not discuss 
this very difficult subject; the causes which have been given by Benedikt, of 
which there are numerous adherents, at the very least are not applicable to 
every case. 

TREATMENT 

A successful treatment of all forms of occupation neurosis depends upon 
complete rest for a long time. This requirement, unfortunately, very often 
meets with insurmountable difficulties. The chances of lasting results are 
greatly decreased by this fact ; but, nevertheless, treatment must be attempted. 
Different forms of the affection require a varied management. The cases in 
which the neurosis depends upon a general, neuropathic predisposition — and 
we have seen how often this is the case — require observation of this fact in 
the therapy: general exhaustion as well as irritability is to be combated by 
hydriatic measures, tonics, and a change of climate; we cannot enter upon a 
detailed description of these measures here. 

In writer's cramp — and also in other neuroses with proper modification — 
it is necessary to note whether the manner in which the pen is held, the nature 
of the pen and penholder, are responsible for the origin of the malady. I 
have mentioned that stiffness in holding the pen is injurious; the same is 
true of very thin penholders and very pointed pens. All this is to be avoided 
or altered if necessary. In addition, definite exercises are necessary; these 
are very advantageous. Zabludowski * has given explicit advice on this sub- 
ject as well as Konindjy, 2 who speaks of a "reeducation de Vecriture." 
Zabludowski begins by having the patient make circular figures in the air, 
such as are employed in large initial letters. In milder cases exercises in 
writing may be begun at once upon paper, in which, at first, an apparatus, 
Nussbaum's bracelet or Zabludowski's penholder carrier, may be employed. 
This apparatus prevents the fingers from assuming the faulty position. Writ- 
ing exercises, with the inverted hand, so that the volar surface of the hand 
is placed upward, is also advised; this gives the hand an entirely new base 
of support. These writing exercises should be practiced 3 times daily, the 
duration of each being 5 minutes. Konindjy emphasizes the necessity of 
writing slowly, many pauses being introduced, so that the pen may be dipped 
frequently into the ink. The movements of the arm necessary for this pur- 
pose causes the contraction of other muscles than those required in writing. 
In very severe cases, in which the pen can no longer be used, it is sometimes 

i Zabludowski, " Ueber Schreiber- und Pianistenkrampf." Tolkmann's Samml. 
klin. Vortr., 1901. 

2 Konindjy, " La crampe professionelle." Nouv. Icon. d. I. Salpetr., 1905, p. 521. 



1160 OCCUPATION NEUROSES 

possible to have these exercises practiced with a lead pencil, progress being 
attained in this manner. 

Massage of the entire arm and hand may be used in addition to the exer- 
cises. This treatment is especially advisable in cases in which there are in- 
flammatory changes. Zabludowski advises energetic vibration massage. I 
have attained excellent results with vibration massage, gradually increasing 
the intensity, special muscles in the arm and hand being treated separately. 
I have used this treatment in combination with resistance movements; this 
method is especially indicated in the occupation neurosis of typewriters. The 
same apparatus may be employed for resistance movements. 

Electricity may be used according to general principles. The galvanic 
current is most often applied, the anode being placed upon the brachial plexus 
or upon the elective points of the different nerves of the arm. The strength 
of the current should be 4-6 ma. Galvanization of the cervical cord has 
also been advised. Most authors (Gowers, Bernhardt, Oppenheim and others) 
report but slight favorable results from electricity. The faradic current is 
applied when there is great muscular debility : strong faradic currents should, 
however, never be used. Faradic hand-baths have been employed with ad- 
vantage (Laquer) as well as the static machine, arc light, and the incan- 
descent light (Laquer). 

Where local changes are present these should be relieved if possible with- 
out surgical intervention. Cutting the flexor and extensor tendons of the 
fingers, which was formerly practiced (Dieffenbach) is now quite properly 
rejected. 

In those instances in which it was impossible for the patient to write 
with the previously mentioned apparatus, an attempt was occasionally made 
to use the left hand for the purpose; in some cases this has been successful 
for a number of years. Often, however, difficulties soon appear in the left 
hand which render writing impossible. The use of the typewriting machine 
is now urged. That this remedy may also fail in some cases was to be ex- 
pected; in a case of Simpson's, writer's cramp also originated from the use 
of the type machine. 

It cannot be doubted that suggestion plays an important role in the 
therapy of writer's cramp and the other occupation neuroses. That its influ- 
ence will vary in the different forms is obvious. 

The treatment of the other varieties of occupation neurosis may be de- 
duced from what has been advised for writer's cramp. I have already called 
attention to some of the modifications. 

Patience and perseverance on the part of the physician and patient and 
certain favorable circumstances in the social position of the sufferer are of 
the greatest advantage in procuring favorable results in the treatment of 
these maladies. 



INDEX OF AUTHORS 



Abadie, Ch., 965. 

Abbe, 850. 

Abercrombie, 371, 908. 

Achard, 813. 

Adam, 973. 

Adamkiewicz, 379. 

Adriax, 793. 

Albu, 914. 

Aldrich, 1157. 

Alexander, 799. 

Althaus, 458. 

Alzheimer, 1037. 

Andral, 266, 587. 

Anfimow, 458. 

Anstie, 844. 

Anton, 905, 909. 

Apathy, 37, 46, 103. 

Aran, 908, 964. 

Arndt, Rudolf, 229, 250, 978. 

Arnold, 603. 

Arxoldi, 314. 

Arnozan, 780. 

Askanazy, 969. 

Astie, 603. 

Audrey, 906. 

Avellis, 948. 

axenfeld, 512. 

Babes, 424. 

Babinski, 202, 203, 460, 462, 505, 532, 887, 

908, 967. 
Babinsky, B., 686. 
Babonneix, 817. 
Bach, 512, 513. 
Baginsky, 806, 807. 
Baillarger, 5. 
Baldwin, 967. 
Bali xt, 561. 

Ballet, 532, 713, 748, 1014. 
Bamberger, 959. 
Banti, 288. 
Bardenheuer, 369. 
Barensprung, 764. 
Barnes, 823. 
Bartelmes, 821. 
Basedow, 960. 



Bastian, 269, 271, 285, 290, 292, 293, 294, 
303, 317, 318, 395. 

Bauer, 823. 

Baumann, 969. 

Beard, George M., 976. 

Bechat, 894. 

v. Bechterew, 38, 55, 75, 100, 106, 669, 
930, 1024, 1042. 

Beevor. 11, 18. 

Begbie, 963. 

Bell, 662, 679, 925, 1150. 

Belugou, 458. 

Benda, 43. 

Bendix, 234. 

Bexedikt, 466, 523, 546, 839, 899, 915, 
964, 965, 977, 1150, 1159. 

Berger, A., 904, 905. 

Berger, O., 279, 702, 946, 1150. 

v. Bergmaxn, 415. 

Berkhax, 330. 

Berlin, 297, 558. 

Bernard, Claude, 685, 964. 

Bernhardt, 330, 432, 458, 602, 603, 630, 
631, 632, 649, 651, 744, 717, 748, 750, 
757, 772, 773, 789, 801, 925, 928, 940, 
942, 945, 946, 948, 951, 953, 954, 957, 
958, 1098, 1150, 1151, 1152, 1153, 1156, 
1157, 1158, 1160. 

Bernheimer, 512. 

Beselin, 944. 

Bethe, 37, 46, 103, 777, 778. 

Bettmann, 698, 699, 929, 931. 

Bewy, 801. 

Bezold, 278, 622, 1069. 

Bickel, A., 517. 

BlELSCHOWSKY, 402, 414. 

Bienfait, 965, 969. 

Bier, 254, 439. 

Billroth, 908. 

Bing, 613, 620, 621, 622. 

Binswanger, 944, 949, 959, 1013, 1014, 
1017, 1018, 1020, 1021, 1022, 1023, 1024, 
1025, 1027, 1028, 1029, 1030, 1031, 1032, 
1034, 1035, 1036, 1037, 1039, 1043. 

Bischoff, 395, 629, 630, 631, 632. 

Blazizek, 918. 

1161 



1162 



INDEX OF AUTHORS 



Bleuler, 304. 

Bloch, 761. 

Blocq, 1070. 

Blokusewski, 1001, 

Blondel, 546. 

Blum, F., 36, 967, 968. 

Blumenthal, 255. 

Bockhart, 538. 

Boinet, 969. 

Bolk, 405. 

Bolton, 808. 

Bompaire, 951. 

Bonar, 458. 

bonhoeffer, 815. 

Bonhoffers, 793, 794, 796. 

Bonnet, 437. 

bonninghaus, 231. 

Bonuzzi, 546. 

Bordas, 797. 

borgherini, 889. 

Borst, 573. 

Bousquet, 548. 

Botton, 806. 

Bouillaud, 266. 

Bourneville, 944. 

Bramwell, Byrom, 405, 417, 458. 

Brandt, Thure, 1007. 

Brasch, F., 251, 425, 460, 1134. 

Bratz, 1035. 

Brauer, 803. 

Braun, 239, 252, 712, 719, 721. 

Braunstein, 234, 248, 250. 

Breitung, 1036. 

Brenner, 831. 

Brera, 371. 

Breuer, 1093. 

Brieger, 428. 

Briquet, 1048. 

Brissaud, 505, 532, 597, 823, 935, 940, 944, 

951, 954, 965, 1151, 1152. 
Broc, 809. 
Broca, 8, 11, 266, 267, 268, 269, 270, 272, 

273, 274, 330. 
Brocq, 1144. 

Brookshank, James, 460. 
Brouardel, 745. 
Brousse, 612. 

Brown-Sequard, 382, 386, 542, 1036. 
Bruck, 964. 
Bruns, 217, 297, 395, 405, 557, 558, 566, 

567, 578, 650, 713, 769, 772. 
Bubnow, 444. 
Buch, 745. 
Bugge, 252. 
Buhl, 816. 
Bumke, 513. 
Bunl, 819. 



BURDACH, 313. 

Burr, 815. 
Busch, 633. 
Buschau, 965, 974. 
Buzzard, 797, 825. 
Bychowski, 884. 

Cajal, Ramon y, 8, 38, 50, 63, 92, 99. 

Calot, 438. 

Campbell, 764. 

Carini, 255. 

Caro, 270. 

Carre, Mar., 452. 

Cassirer, 216, 512, 642, 648, 679, 765, 771, 
782. 

Cavazzani, A., 235, 255. 

Cavazzani, E., 255. 

Centanni, 819. 

Cestan, 559, 614, 658, 803. 

Chapmann, 438. 

Charcot, 72, 73, 266, 276, 284, 285, 313, 
351, 378, 390, 397, 398, 452, 466, 485, 
490, 491, 522, 523, 541, 546, 557, 558, 563, 
564, 565, 568, 569, 572, 573, 575, 578, 
580, 627, 633, 646, 647, 651, 658, 733, 
747, 773, 880, 893, 897, 905, 909, 935, 
944, 961, 1014, 1049, 1062, 1070, 1099. 

Charcot, J. B., 747. 

Charpentier, 1021. 

Chaslin, 1037. 

Chatelineau, 775. 

Chevalier, 969. 

Chiari, 233. 

Chipault, 217, 250, 251, 850. 

Chvostek, Jr., 908, 916. 

Chvostek, Sr., 907, 908, 916, 961. 

Clarke, L., 371, 378, 633. 

Clarus, 330. 

Cohen, Solis, 1137, 1138, 1147. 

Cohn, Hermann, 1000. 

Cohn, P., 468. 

Cohn, Toby, 945, 1158. 

Coignet, 538. 

Cole, 814, 815. 

Colella, 810, 811. 

Collier, 1140. 

Collins, 457, 458, 467, 495, 523, 1145, 
1146. 

cololian, 1034. 

Constant, 907. 

Cooper, 963. 

v. Corval, 546. 

Coster, 458, 713, 771. 

Courmont, 685. 

Courtellemont, 771, 773. 

Crede, 541. 

Crocq, 425, 1074. 






INDEX OF AUTHORS 



1163 



Cronbach, 1156. 

Cros, 966. 

Cruveilhier, 372, 557, 633. 

Curschmann, 424, 425, 768, 771, 772, 773, 

918, 1056. 
Cushing, H., 369. 
Cton, E., 968. 

CZERMAK, 684. 

Damron, 787. 

Dana, 458, 889, 890, 892, 897, 929, 965. 

Dance, 907, 910. 

Danjou, 332. 

Daus, 759. 

Dax, Marc, 309. 

Debove, 425, 801. 

Decroly, 811. 

Deibel, 684. 

Dejerine, 220, 270, 272, 273, 274, 276, 
279, 284, 286, 288, 289, 290, 292, 294, 
297, 312, 313, 315, 317, 318, 321, 323, 
398, 458, 478, 487, 493, 495, 498, 499, 
504, 510, 523, 618, 619, 633, 639, 647, 
648, 722, 745, 761, 765, 779, 780, 791, 
813, 816, 820, 906, 925, 929, 930, 1095, 
1131. 

Delamare, 485. 

Delasiauve, 1015, 1031. 

Deleage, 929. 

Delille, Armand, 779. 

Delpech, 908. 

Deniges, 235. 

Deshusses, 971. 

Destot, 713. 

Determann, 458, 528, 541. 

DlEFFENBACH, 1160. 

Dinkler, 458, 498, 692, 969. 

DlRCKSEN, 235. 

Dochmann, 946. 

Dogiel, 38. 

Donaggio, 629. 

Donat, 1019. 

Donath, 220. 

Donne, 1019. 

Donner, 984. 

Dreschfeld, 788. 

Drobnik, 776. 

Dubois, 684. 

Dubois-Reymond, 343, 878. 

Dubreuilh, 647. 

Duchenne, 157, 378, 452, 454, 513, 612, 
633, 639, 641, 675, 698, 712, 720, 725, 
945. 956, 957, 959, 1150, 1158. 

Durdufi. 965. 

Dujardin-Beaumetz. 452. 

Dums, 716, 1157. 

Dutil, 813, 894. 



Duvante, 747. 
Dydinski, 460. 

Ebstein, 425. 

Edinger, 54, 55, 67, 83, 92, 100, 128, 353, 
358, 405, 427, 467, 507, 541, 613, 621, 
622, 625, 643, 768, 774, 776, 827, 978. 

Eeman, 808. 

Egger, 196, 395, 479, 779, 1131. 

Ehrlich, 38, 847. 

Ehrmann, 846. 

Eichhorst, 558, 559, 561, 684, 763, 795, 
814, 817. 

Eide, 815. 

V. ElSELSBERG, 911, 949. 

Eisenlohr, 458, 809, 819, 905, 906, 1099. 

ElSENMANN, 452. 

Elias, 684. 

Ella, 559. 

Embden, 216, 562. 

Engelmann, 338, 365. 

Engesser, 520. 

Epstein, 334, 807, 808, 812. 

Erb, 118, 126, 381, 402, 414, 452, 460, 463, 
465, 469, 474, 477, 627, 628, 629, 630, 
633, 639, 640, 641, 644, 645, 654, 656, 
665, 670, 671, 692, 720, 746, 915, 925, 
927, 928, 930, 947, 959, 1098. 

Erbsloh, 802. 

Erichsen, 1098, 1099. 

Erlenmeyer, 946, 947, 1041. 

Erlicki, 798, 815. 

Escherich, 908, 937. 

Etienne, 587, 802. 

Eulenburg, A., 459, 625, 716, 760, 763, 
844, 887, 905, 931, 953, 955, 957, 959, 
1140. 

Ewald, R., 60, 655, 912. 

Exner, 290, 313, 444, 697. 

Facio, 651. 

Falkenheim, 780. 

Faure, 458, 967. 

Faworski, 803. 

Fehre, 457. 

Feindel, 940, 942, 947, 948, 949, 951, 952, 

956, 959. 
Fere, 332, 491, 946, 979, 1012, 1013, 1014, 

1015, 1018, 1019, 1021, 1023, 1027, 1028, 

1030, 1032, 1034, 1035, 1039, 1040, 1042, 

1043. 
Fereol, 487. 
Ferras, 458. 
Ferrier, 11. 
FlCKLER, 375. 
Filehne, 679, 965. 
Finkelstein, 937. 



1164 



INDEX OF AUTHORS 



FlNKLER, 429. 

Fischer, 458, 516, 929, 961, 963. 

Flatau, Th. S., 339, 425, 781. 

Flechsig, 13, 15, 280, 497, 1042. 

Fleiner, 907, 914, 921. 

Flemming, 814. 

Fleury, 948. 

Flourens, 6, 266, 417. 

Forster, Otfried, 483, 515, 516, 518, 520, 

551, 1065, 1070. 
Fournier, 452, 454, 458, 460, 466, 468, 

535, 1033. 
Franceschi, 813. 
Francotte, 811, 812. 
Franjen, 1158. 

Frank, 371, 566, 570, 579, 973. 
Franke, 743, 776. 
Frankel, 276, 793, 796, 1158. 
v. Frankel, 558, 664, 679. 
Frankel-Hochwart, 558, 565, 575, 576, 

1130. 
Fraser, 619. 
Frenkel, 219, 454, 476, 481, 483, 505, 

515, 516, 517, 519, 520, 541, 546, 549, 

550, 551, 552, 553, 580, 625, 887, 889. 
Frenkel, H. S., 516. 
Frensberg, 1025. 
Frerichs, 557, 633. 
Freud, 83, 1093. 
Freund, 566. 
Freund, C. S., 303. 
Freund, S., 277, 278, 303. 
Frey, 959. 
Freyhau, 249. 
Friederichsen, 459. 
Friedmann, 629, 1100, 1114, 1115, 1117, 

1122. 
Friedreich, 452, 519, 593, 605, 612, 615, 

617, 620, 623, 633, 638, 641, 642, 643, 644. 
Fritsch, 6, 11, 213, 267, 268, 442, 1024, 

1026. 
Frohlich, 928. 
Frohmann, 930. 
Frohse, 677, 718, 747. 
Furst, A., 969. 
Fuchs, 216, 217, 930. 
Furbringer, 985, 993. 

FtJRNROHR, 1018. 

Furstner, 424, 427, 890. 

Gajkiewicz, 458. 

Galen, 1015. 

Gall, 8, 11, 32, 214, 266. 

Gallard, 1152. 

Gallerain, 946. 

Ganghofner, 908, 937, 948. 

Gaspero, 788, 792. 



Gauthier, 966. 

van Gehuchten, 38, 112, 395. 

Geizel, 965. 

Generali, 912. 

Geraudel, 235. 

Gerhardt, 395, 458, 459, 505, 576, 577, 

578, 684, 766, 843, 850, 914. 
Gerlach, 36, 371. 
Gessler, 713. 
Giese, 659. 
Gley, 912. 
Glisson, 437. 
Glogner, 809, 819. 
Gluge, 371. 
Goffroy, 962. 
goldflam, 948. 
Goldscheider, 117, 193, 285, 324, 378, 405, 

425, 444, 453, 501, 517, 550, 551, 712, 

953. 
Goldzieher, 664. 
Golgi, 63. 
Goltz, 11, 384. 
Gombault, 559, 780, 789, 815. 
v. Gorski, 884. 
Gothard, 815. 
Gowers, 358, 361, 369, 405, 412, 453, 454, 

458, 659, 716, 784, 801, 802, 878, 883, 

902, 905, 941, 953, 1026, 1027, 1028, 1160. 

CrRABOWER, 683. 

v. Graefe, 938, 942. 

Grashey, 285, 286, 287, 298, 302, 303, 304, 

305, 306, 307, 308. 
Grasset, 454, 1142. 
Graupner, 551. 
Graves, 960. 
Green, 697. 
Greenfield, 962. 
Greidenberg, 894, 900. 
Griesinger, 816. 
Grober, 235, 247. 
Gros, L., 963. 
Gross, 249, 1146. 
Grube, 812. 
Grunbaum, 11. 
Grunnach, 429, 430. 
Gubler, 710. 
Gudden, 788, 814. 

Guillain, 500, 503, 504, 559, 713, 799. 
Guinard, 254. 
Guinon, 935. 
Gull, W., 389, 485. 
Gumpertz, 460. 
Gumprecht, 235. 
gussenbauer, 841. 

GtJTERBOCK, P., 949. 

Guttmann, 459, 550, 959. 
Gutzmann, Albert, 343. 



INDEX OF AUTHORS 



1165 



Haidenhein, 444. 

Haig, 1034. 

v. Halbau, 458, 465, 904, 905. 

Hall, Marshall, 1023. 

Halliburton, 235. 

Hallion, 425, 1151, 1152. 

Hammond, 899, 936. 

Hansemann, 807, 929. 

Hardy, 763. 

Harless, 371. 

Hartman, 220. 

Hasebrock, 952. 

Hauser, 503. 

Hayem, 378, 425, 1019. 

Head, 353, 358, 361, 389, 405, 764. 

Hebold, 1035. 

Hegar, 546. 

Heilbronner, 281, 307, 308, 320, 814. 

Heimann, 887. 

Heine, 378. 

Held, 36, 46. 

Helfft, 963. 

Helleda, 866. 

Hellendahl, 249. 

Heller, 233, 382, 427, 803, 815. 

Helmholtz, 270. 

Henkel, 249. 

Henle, 224, 313. 

Henneberg, 229, 402. 

Henoch, 934. 

Henocque, 1019. 

Henriquez, 425. 

Henschen, 368, 369, 561, 743, 802, 815, 

866. 
Hering, E., 441. 
Hering, H. E., 517. 
Herz, Max, 599, 1137, 1138, 1147. 
Herzen, A., 1001. 
Herzog, 425. 
Hess, 1134. 
Hessing, 437, 547. 
Heubner, 320, 654, 674, 722. 
Heussner, 712. 

HlFFELSHEIM, 963. 
HlLDEBRANDT, 371. 

v. Hippel, 512. 
Hippocrates, 344. 
Hirsch, W., 426. 
Hirschberg, 550, 551, 552. 

HlRSCHEL, 917. 
HlRSCHFELD, 1136. 

Hirschl, 967. 

Hirt, 458, 946. 

His, 73. 

Hitzig, 6, 11, 60, 213, 224, 267, 268, 442, 

458, 467, 469, 478, 663, 942, 1024, 1026, 

1031, 1043. 



Hlavacek, 932. 
Hoche, 395, 425, 1101. 
Hochhaus, 630, 917, 954. 
hochsinger, 908. 

H6DEMAKER, 720. 
HOEFLMAYR, 772. 

Hoffa, 441, 450, 645, 646. 

Hoffmann, 459, 498, 559, 560, 600, 633, 
640, 641, 646, 647, 648, 649, 651, 653, 
684, 699, 773, 892, 907, 913, 916, 917, 
928, 929, 931, 934, 957. 

Holm, 887. 

Homen, 425, 458, 766, 777. 

honigschmied, 681. 

Horn, 452. 

Horner, 965. 

Horsley, 11, 18, 369. 

Hosslin, 757, 758, 759, 760, 789. 

hudovernig, 461. 

Huet, 713. 

Humphrey, 558. 

Huneric, 344. 

HtJNERMANN, 758. 

Hunt, 889. 

Hurthle, 967, 968, 969. 

Immermann, 840, 846. 
Infeld, 904, 905. 

Jaboulay, 971. 

Jaccoud, 452. 

Jackson, Hughlings, 1027, 1028. 

Jacob, P., 444, 517, 550, 551. 

Jacob-eus, 788. 

Jacobsohn, 782. 

Jacoby, 252. 

Jacquin, 1019. 

Jadassohn, 464. 

Jakob, 248, 254, 255. 

v. Jaksch, 562, 908, 909, 913, 919, 958, 967. 

Jamin, 645, 650. 

Janet, 1070, 1077. 

Japha, 937. 

Jastrowitz, 728. 

Jendrassik, 620, 664. 

Jensen, 927. 

Joffroy, 532, 763, 810, 813. 

Jolly, 458, 459, 660, 721, 794, 799, 892, 

936, 949, 1025, 1099. 
Jonas, 914. 
Jones, 773. 
Jonnesco, 971. 
Jores, 929. 
Jutzler, 558, 562. 

Kadyi, 379. 
Kahlden, 378, 379. 



1166 



INDEX OF AUTHORS 



Kahler, 269, 413, 414, 415, 560, 617, 654, 

789, 905, 961. 
Kaiser, 929. 
Karger, S., 1128. 
Karplus, 218, 797, 887, 888, 889. 
Kashida, 915. 
Kasparek, 931. 
Kassowitz, 908. 
Kast, 458, 716, 766, 787, 817. 
Kattwinkel, 498, 531, 777. 
Katz, 816. 
Kausch, 766. 
Kerschensteiner, 777. 
Ketly, 802, 803. 
Ketscher, 889. 
Kiewltcz, 562. 
Kiliani, 252. 
Kinsman, 797. 
Klemperer, 467, 957. 
Klippel, 489. 
Klohss, 371. 
Klumpke, 415, 722, 780. 
Kny, 934, 957. 
Koch, 957. 
Kocher, 353, 387, 390, 394, 395, 405, 415, 

416, 440, 939, 952, 966, 972. 
Koeben, 966. 
Koester, 759, 801, 803. 
Kohn, A., 912. 
Kohts, 428. 
Kojewnikoff, 459. 

van der Kolk, Schroder, 1025, 1026, 1137. 
Koller, 889. 

KoLLIKER, 38. 

Konindjy, 1159. 

korniloff, 517. 

Kornilowicz, 887. 

Korsakow, 794, 817, 938. 

Korzou, 798. 

Koster, 664, 670, 671. 

Kowalewski, 967. 

Kraeplin, 917. 

v. Krafft-Ebing, 458, 461, 465, 470, 558, 

560, 883, 884, 887, 892, 894, 918. 
Krainski, 1034, 1035. 
Kramer, 788, 799. 

Kraus, Fr., 381, 405, 414, 450, 909, 966. 
Krause, F., 369, 677, 679. 
Kredel, 491. 
Krehl, 766. 

Kron, 457, 459, 460, 464, 467, 773, 798, 799. 
Kronig, 228, 237, 248, 254. 
Kronthal, 1100. 
Krukenberg, 442. 
Kuhn, 457, 458. 
Kuhn, 630, 631. 
Kulmus, 1157. 



KUNDRAT, 902. 

Kuner, 748. 

Kunn, 564, 917. 

Kussmaul, 269, 271, 282, 318, 334, 336, 

378, 633, 808, 813, 816, 907, 910, 915, 
1025. 

KtJSTERMANN, 817. 

Kutner, 748. 

Labbe, 458. 

Lacquer, L., 953. 

Laehr, 478, 597, 609. 

La.hr, 747. 

Laitinen, 766. 

Lallemand, 996. 

Lamy, 813. 

Lancereaux, 785, 813. 

Landois, 396. 

Landouzy, 639, 663. 

Lange, 913, 946. 

v. Langenbeck, 633. 

Langer, 249. 

Langerhans, 426, 920. 

Lannois, 883, 1139, 1140. 

Lantz, 972. 

Lapinsky, 405, 812, 819. 

Laquer, 365, 369, 1160. 

Lasalle, 843. 

Lasegue, 1015, 1027. 

Lazarus, P., 405, 440, 442, 443, 446. 

Le Gendre, 638. 

Leloir, 765. 

Lenhartz, 251, 253. 

v. Lenhossek, 38. 

Lennander, 369. 

Lent, 558. 

Lepine, 425, 1019. 

Leppmann, 847. 

Leredde, 504. 

Leri, 523. 

Lesage, 811. 

Le Sourd, 250. 

Lesser, Fritz, 457, 504, 506. 

Lethievant, 776. 

Letulle, 618, 803, 815. 

Leube, 965, 969. 

Leubuscher, 1040. 

Leudet, 713. 

Leva, 884. 

Levinsohn, 512. 

Levy, 756, 771. 

Lewandowsky, 255. 

Lewin, 802. 

v. Leyden, 31, 101, 228, 255, 372, 373, 378, 

379, 380, 381, 385, 390, 395, 400, 414, 
424, 425, 427, 429, 431, 434, 444, 452, 
453, 454, 459, 467, 481, 495, 499, 501, 



INDEX OF AUTHORS 



1167 



517, 549, 550, 551, 553, 557, 560, 569, 

573, 633, 639, 651, 793, 795, 802, 827, 

925, 1098. 
Lhermite, 799. 

Lichtheim, 219, 269, 275, 277, 282, 283. 
Liepmann, 270, 277, 278, 300, 308, 319, 

320, 321, 324, 1037. 
Linser, 460. 
Lissauer, 324. 
Liveing, 878. 
Londe, 613, 651. 
Loos, 908. 
Lorenz, 639, 651. 
Lorrain, 630. 

Lowenfeld, 714, 763, 802, 938. 
Lubarsch, 376, 962, 963. 
Lubbers, 566, 567, 572. 
Luce, 664. 
Luce, 809, 810, 817. 
Luchsinger, 1025. 
Luderitz, 406, 417, 418, 747. 
Lugaro, 781, 817. 
Luzzato, 817. 

Mackenzie, 963. 

Mader, 817. 

Magendie, 679, 680. 

JVJager, 427. 

Maier, 813. 

Majano, 512. 

Mally, 721. 

Manasse, P., 953. 

Mann, 908, 937. 

Marchi, 400. 

Marey, 337, 338, 340. 

Marie, Pierre, 101, 378, 463, 500, 503, 

504, 505, 522, 523, 531, 532, 558, 560, 

603, 612, 614, 615, 624, 639, 646, 647, 

658, 944. 
Marina, 513, 936. 
Marinesco, 373, 378, 424, 425, 478, 599, 

766. 
Martin, 249, 523. 
Martineau, 458. 
Martius, 929, 961. 
Mayer, 219, 382, 378, 505, 909. 
Mayer, Moritz, 118. 
Mayer, Sigmund, 777. 
Meerwein, 638. 
Meige, 505, 773, 940, 942, 947, 948, 949, 

951, 952, 956, 959, 1151, 1152, 1157. 
Meinert, 917. 
Mendel, 467, 562, 897, 965, 1018, 1099, 

1146. 
Mendel, Kurt, 1123. 
Meniere, 490. 
Menz, 713. 

75 



Menzel, 619. 

Merkel, 224. 

Merkxen, 809. 

Merzbacher, 517. 

Meyer, 743, 787, 792. 

Meyer, H., 512. 

Meyer, S., 38. 

Meyerson, 948. 

Meynert, 267, 268, 269, 270, 280, 281, 

289, 291, 292, 314. 
Mezkowski, 803. 
Mikulicz, 441. 
Milan, 528. 

Minkowski, 369, 779, 787. 
Minnich, 428. 

Minor, 426, 429, 457, 458, 461, 909. 
Mitchell, 946. 
Mitchell, Weir, 748, 753, 761, 840, 954, 

1139. 
Miura, 792, 812. 
Mobius, 32, 214, 451, 452, 457, 466, 470, 

471, 493, 526, 528, 716, 745, 759, 760, 

766, 878, 925, 966, 972. 
Moczutkowski, 453, 458, 466, 546, 709. 
Moeli, 813, 1098. 
Mollier, 699, 701, 704. 
moltschanow, 930. 
v. Monakow, 19, 20, 69, 284, 288, 291, 

293, 299, 313, 315, 317, 318, 319, 320, 

322, 324, 900, 905. 
monckeberg, 777. 
Monod, 235. 
Moos, 816, 819. 
Moreira, 685. 

MORGAGNI, 587. 

Morvan, 600. 

Mossdorf, 948. 

Mosso, 341. 

Mott, 458, 493, 517. 

Moussu, 912. 

Muller, 797, 942. 

Muller, Fr., 118, 885. 

Muller, Johannes, 343. 

Muller, L. R., 981, 982, 985, 987. 

Muller, M., 371. 

Murdoch, 907. 

Muthmann, 773. 

Munk, H., 12, 60, 263, 442. 

Munk, Herman, 11. 

Naegeli, 849. 

Nageotte, 463, 493, 498, 499, 502, 503, 

504, 505, 510, 511, 529. 
Nalabandoff, 929. 
Naunyn, 290, 305. 
Navaratski, 229, 250. 
Navratil, 683. 



1168 



INDEX OF AUTHORS 



Nawratzki, 817. 
Negro, 458, 459. 
Neisser, 309, 468, 535, 995. 
Nemann, 331. 
Netter, 248. 
Neugebauer, 255. 
Neumann, 419, 1144. 
Newmark, 630, 940. 
Nicati, 965. 

Nichols, Langworth, 425. 
Nicoladoni, 776. 
Niebergall, 957. 

NlKOLAJEVIC, 918. 

Nissl, 36, 116, 400, 505, 635, 890. 

Nogues, 928. 

Nolda, 558. 

Nolke, 231, 233, 250. 

Nonat, 587. 

Nonne, 119, 456, 457, 460, 499, 612, 616, 

619, 623, 788, 818, 844, 889, 944, 1107. 
Norstrom, 866. 
Nose, 465. 
Nothnagel, 213, 403, 669, 759, 903, 904, 

922, 931, 1016, 1025, 1043, 1136. 
Notkin, 968, 969. 
nove-josserand, 953. 

Obersteiner, 55, 103, 379, 501, 781, 903. 

Oberthur, 548, 812. 

Okada, 817. 

Oliviero, 1034. 

Ollivier, 371, 379, 587, 944. 

Onimus, 1156. 

Onodi, 683. 

Onuf, 276. 

Oppenheim, 119, 204, 216, 217, 309, 310, 
324, 331, 380, 405, 415, 425, 439, 466, 487, 
499, 557, 561, 562, 563, 565, 566, 567, 568, 
569, 570, 579, 580, 627, 638, 642, 648, 653, 
655, 657, 659, 660, 675, 685, 722, 732, 742, 
743, 749, 756, 761, 766, 770, 771, 772, 774, 
775, 777, 783, 786, 787, 788, 793, 794, 796, 
797, 800, 803, 805, 809, 810, 812, 813, 814, 
815, 816, 817, 819, 821, 824, 827, 890, 894, 
897, 906, 926, 928, 929, 942, 945, 947, 948, 
951, 952, 958, 959, 1015, 1016, 1020, 1023, 
1028, 1037, 1038, 1039, 1052, 1099, 1151, 
1153, 1157, 1158, 1160. 

Ordenstein, 880, 887. 

Orglmeister, 219. 

Ormerod, 647, 894. 

Osann, 768. 

Osler, 1144. 

Pacinotti, 946, 
Pagf- 1099. 



Pal, 811, 814. 

Parinaud, 944. 

Parisot, 467. 

Park, 369. 

Parkinson, 880 

Parrot, 723. 

Parry, 960. 

Pastrovich, 789. 

Pehu, 1136. 

Peiper, 131. 

Peliz^eus, 928. 

Peltesohn, 344. 

von Penzoldt, 535, 551. 

Personali, 946, 947. 

Peters, 917, 925. 

de Peters, 723. 

Petrone, 459, 928. 

Peyser, 948. 

Pfaundler, 227, 228, 249. 

Philippe, 815. 

Pick, 54, 269, 279, 290, 309, 318, 321, 322, 

560, 617, 789, 817, 905, 1131. 
Pierret, 496, 499, 656. 
Piltz, 209. 

Pineles, 903, 904, 905, 911, 912, 913. 
Pini, 567. 
Piorry, 966. 
Pitres, 290, 300, 305, 306, 308, 313, 765, 

780, 810, 1070. 

POCHHAMMER, 425. 

Poehl, 542. 

POIRIER, 32. 
Porot, 1140. 
Portal, 587. 
Pospelow, 1137, 1140. 
pourreyron, 461. 
Probst, 20, 317, 558, 567. 
Preiss, O., 1007. 
Pribytkow, 817. 
Pryce, 819. 
Putnam, 369, 843. 

de quervain, 952. 

Quincke, 1, 137, 139, 140, 218, 250, 251, 
684, 1075, 1144, 1146. 

Rabaud, 908. 

v. Rad, 460. 

Raecke, 461. 

Ramon, 112. 

Ranvier, 40, 843. 

Rauchfuss, 437. 

Ravaut, 505. 

Raymond, 140, 222, 362, 493, 505, 532, 550, 

630, 658, 722, 771, 773, 821. 827. 
Raynaud, 1132, 1134, 1135, 1136, 1137, 

1138. 



INDEX OF AUTHORS 



1169 



Redlich, 101, 103, 292, 295, 379, 428, 457, 
458, 501, 754, 767, 889. 

Rehfisch, 982. 

Rehlen, 466. 

Rehn, 369. 

Reichardt, 513. 

Reid, 31. 

Reilly, 839. 

Remak, 40, 458, 544, 711, 713, 723, 727, 
743, 748, 749, 751, 754, 755, 757, 758, 
761, 762, 763, 765, 766, 768, 770, 771, 
772, 773, 775, 776, 782, 785, 789, 791, 
792, 795, 800, 801, 803, 805, 806, 807, 
809, 810, 811, 812, 820, 823, 938, 942, 
946, 1150, 1151, 1157, 1158. 

Renaut, 962, 969. 

Rendu, 235, 894. 

Rennert, 787, 788. 

Rennie, 620. 

Retzius, 38. 

Reynolds, 797, 799. 

Richer, P., 949. 

RlCHET, 1041. 

Rieder, 768. 

Riegel, 907, 1025. 

Rieger, 286, 297, 298, 299, 301, 302, 303, 
305, 306, 307, 308. 

Rigler, 1098. 

RlNDFLElSCH, 557. 

Rinne, 1069. 

Rissler, 378. 

Roche, 972. 

Romberg, 452, 453, 493, 515, 532, 676, 880, 

946, 954. 
Rosenbach, 766, 906, 978. 
Rosenberg, 885. 
Rosenfeld, 973. 
Rosenthal, O., 539. 
Rosin, 36, 101, 116. 
Ross, 405, 784, 787, 797. 
Rossolimo, 986. 
Roth, 378, 729, 748, 787. 
Rothe, 806. 
Rothmann, M., 1, 69. 
Roussy, 761. 
Roux, 288, 621, 663. 
Rudinger, 914. 
Ruge, 512. 
Ruhle, 633. 

Rumpf, 458, 460, 545, 809, 810, 817. 
Russell, 405. 
Rybalkin, 798, 815. 
Rydel, 196. 

Sabrazes, 235. 

Sachs, H„ 270, 291, 303, 317, 318, 320. 

Sahli, 253. 



Salomon, 251. 

Salomonson, 664, 713, 771. 

Salzwedel, 425. 

Samuel, 398, 1129. 

Sander, 889. 

Sandstrom, 912. 

Sanger, 944, 946, 947. 

Sano, 770. 

Sarbo, 457, 458, 476. 

v. Sass, 889. 

Sayre, 546. 

Schafer, 11. 

Schauz, 792. 

Schapringer, 693. 

Schede, 369, 636. 

Scheich, 948. 

Schiefferdecker, 925, 929. 

Schiff, 219, 425, 512, 592, 911. 

Schittenhelm, 458, 459, 465, 467. 

Schlesinger, 348, 593, 596, 597, 599, 600, 
601, 603, 605, 606, 610, 611, 813, 908, 
916, 918, 930, 1137, 1140, 1144, 1146. 

Schleutzka, 773. 

Schmaus, 377, 378, 379, 380, 381, 426, 427, 
495, 496, 504, 1100. 

Schmidt, A., 235, 269, 693. 

Schnee, C, 445. 

Schnitzer, 394. 

schoenborn, 219. 

Scholz, W., 966. 

SCHONBORN, 505. 
SCHRANK, 334. 

Schroder, 697. 

Schroff, 1025. 

v. Schrotter, 382, 427, 433. 

Schule, 557, 558, 627. 

Schultz, 773, 777. 

Schultz, Fr., 118, 255, 424, 430, 458, 572, 
612, 613, 614, 616, 617, 618, 620, 662, 
664, 738, 803, 907, 909, 913, 915, 916, 
919, 925, 929, 931, 934, 936, 941, 957, 
959, 972, 1099. 

Schulze, E., 467. 

Schumacher, 1034. 

Schuppel, 520. 

Schuster, Paul, 1123. 

Schwalbe, 32, 33. 

schwartze, 248. 

schwarzenberg, 551. 

schwerdt, 963. 

Scwitalski, 523. 

Seeligmann, 458. 

Seeligmuller, 705, 840, 925, 926, 951, 
1153. 

Segelken, 251. 

Seguin, 458. 

Seiffer, 196, 353, 358, 390, 405, 613, 625. 



1170 



INDEX OF AUTHORS 



Semon, 689. 

Senator, 72, 112, 374, 613, 824. 

Seppilli, 947. 

Serbski, 817. 

Sherrington, 11, 353, 405, 441, 517. 

Siccard, 219, 234, 255, 505. 

Sick, 369. 

Siemerling, 499, 647, 813, 814, 817, 819, 

948. 
Silbert, 969. 
Silex, 457, 458, 459. 
Simpson, 1160. 
Singer, Douglas, 379. 
Sirol, 928. 
Skoda, 910. 
Slavyk, 215, 805. 
Soulier, 906, 967. 
soltmann, 301. 
Sommer, 286, 287, 297, 298, 303, 304, 

1134. 
Sorgo, 787. 
Sottas, 925, 929, 930. 
Souques, 460. 
Spath, 520. 
Sperling, 1100. 
Spiegel, 1009. 
Spiller, 560. 
Spillmann, 425, 467, 802. 
Spitzer, 802, 803. 
Spitzka, 67, 458. 
Sprimon, 546. 
Ssikorski, 334. 

Stadelmann, 234, 250, 251, 426. 
Stadler, 1157. 
Stanley, 452. 
Starr, 54, 353, 405, 623. 
Stcherbak, 617, 618, 619, 620, 622. 
Steidel, 716. 
Stein, 732, 812. 
Steinert, 948. 
Steinhausen, 701, 703. 
Steinheim, 907, 910. 
Steinthal, 452. 
Stem bo, 548. 
Stenon, 374. 
Stephan, 1158. 
Stepp, 1100. 

Sternberg, 395, 687, 745, 1132. 
Stewart, 816. 
Stieglitz, 815. 
Stier, 650. 
Stilling, 383, 413. 
Stintzing, 535, 551. 
Stolper, 387, 405, 416. 
Stolting, 558, 566, 567, 578. 
Storbeck, 459. 
Storch, 290, 291. 



Stork, 903. 

Storring, 331. 

Stransky, 780. 

Straussler, 1146. 

Strauss, H., 961. 

Strumpell, 72, 118, 205, 424, 453, 458, 507, 
521, 559, 560, 565, 575, 576, 624, 629, 630, 
631, 633, 651, 654, 659, 745, 787, 792, 810, 
821, 899, 925, 948, 1100, 1119, 1157. 

Stuertz, 959. 

Taylor, 963. 

Teitelbaum, 820. 

Tenner, 1025. 

Testaz, 672. 

Thane, 32. 

Theohari, 779. 

Thieme, Gg., 551. 

Thiemich, 937. 

Thiersch, 850. 

Thomas, 274, 288, 323, 458, 495, 503. 

Thomsen, 788, 925, 1021. l 

Thomson, 813. 

Thornburn, 405, 415, 416. 

Todd, 452, 880, 1109. 

Tooth, 630. 

Topinard, 452. 

Toulouse, 1041. 

de la Tourette, Gilles, 217, 935. 

Traube, 633. 

Treub, 766. 

Troitzki, 965. 

Troltsch, 190. 

Trommer, 467. 

Trousseau, 266, 267, 276, 282, 304, 452, 

816, 832, 837, 880, 907, 910, 915, 961, 

964. 
Tuczek, 468, 530, 541. 
Tuffier, 254. 
Tumpowski, 458. 
Turck, L., 372. 
Turner, .440, 948. 
Twistleton, 344. 

Uhthoff, 512, 513, 566, 567, 572, 788, 

792. 
Ullmann, 425. 
Ulrich, 967. 
Unger, 558. 

Unverricht, 1023, 1028. 
Urban, 551. 

Vaillard, 765, 780, 810. 
Valleix, 830, 831. 
Vallin, 948. 
Variot, 800. 
Vassale, 912. 



INDEX OF AUTHORS 



1171 



Vaughen, 909. 
Verdier, 908. 

VlBERT, 1100. 
VlDAL, 250. 
VlGOUROUX, 961. 
V. VlNTSCHGAU, 681. 

Virchow, 379. 

Vitek, 940. 

Vizioli, 899. 

Vogel, F. C. W., 516, 551. 

Voigt, 458, 466. 

Voisin, 1019, 1034, 1040, 1042. 

Volta, 439, 445. 

v. Voss, 931. 

Vulpian, 452, 454, 510, 557, 815. 

Wagner, 387, 405, 416, 909. 

v. Wagner- Jauregg, 911. 

Waldeyer, 10, 32, 50. 

Wallenberg, 92. 

Waller, 121. 

Walton, 952, 1098. 

Walz, 884. 

Warburton, 963. 

Warrington, 751, 815. 

de Watteville, 445. 

Weber, 716, 755, 800. 

Weidner, 684. 

Weigert, 43, 400, 496, 500, 516, 619, 621, 

978. 
Weiss, 908, 911, 916, 917, 920, 1134, 1135. 
Wendt, 946. 
Werdnig, 649, 650. 
Wermel, 909. 
Wernicke, 162, 328. 
Wersiloff, 930. 
Wertheim, 771. 



Westphal, 54, 215, 331, 378, 402, 425, 493, 
499, 573, 578, 779, 781, 790, 817, 820, 
917, 925, 1036, 1098, 1143. 

Wettendorfer, 917. 

Wichmann, 353, 405. 

Widal, 219, 235. 

w^ilbrand, 944. 

Wilks, 1098. 

WlLLE, 839. 

Williams, 948. 
Williamson, 823. 
Wilms, 237. 

WlNDSCHEID, 467. 

WlNTERNITZ, 970. 

WlTZEL, 840. 

Wlassak, 42. 

Wochenius, 819. 

Wolff, 286, 298, 301, 302, 303, 304, 306, 

307, 308, 663. 
Wollexberg, 217, 889, 892. 
Woolacott, 805. 

WORMSER, 771. 

Wright, 809. 

Zabludowski, 625, 1007, 1159, 1160. 

Zander, 442, 677, 716, 747, 769, 1007. 

Zanietowski, 965. 

Zexi, 1034. 

Zenker, 558, 773. 

Zenner, 945. 

Ziegler, 379, 558. 

Ziehen, 279, 287, 313, 341, 977, 1025, 1026, 

1037, 1043. 
Ziemssen, 471, 539, 697. 

ZlTTMANN, 539. 
ZUCKERKANDL, 565. 
ZWAARDEMAKER, 340. 



INDEX OF SUBJECTS 



Abdominal muscles, spasm of, 956. 
Abducens, origin of, 57. 
Aboulie matrice, 1152. 
Acinesia algera, 1072. 
Acromicria, 1142. 
Acroneuritis, 648. 
Acroparesthesia, 771, 1130. 

etiology of, 1130, 1131. 

symptoms of, 1131. 

treatment of, 1132. 
Acute circumscribed edema, 1144. 

diagnosis of, 1147. 

etiology of, 1144. 

pathogenesis of, 1147. 

prognosis of, 1147. 

symptoms of, 1145. 

treatment of, 1147. 
Adiposis dolorosa, 143. 
Adipsia in hysteria, 1069. 
Adynamia, 165. 
After-brain, 2. 
Ageusia in hysteria, 1069. 
Agrammatism, 322. 
Agraphia, 284. 
Ala cinerea, 81. 
Albuminuria, 785. 

in Graves' disease, 961. 

in tetany, 917. 
Alcoholism, effects of, in nervous disease, 

133. 
Alexia, 284. 
Algesimeter, 599. 
Alimia, 1069. 
Alopecia in Graves' disease, 961. 

in nervous disease, 144. 

in tabes, 492. 

in traumatic neurosis, 1113. 
Amaurosis in tabes, 480. 

swaying in, 171. 
Amblyopia in tabes, 480. 
Amenorrhea in Graves' disease, 961. 
Amnesia in hysteria, 1081. 

retroactive, 794. 
Amygdala, 16. 
Anal reflex, 986. 
Anamnesis. See History. 
Anaphrodisia, 992. 



Anarthria, 265. 

Anatomy, macroscopic, of central nervous 

system, 1. 
Anesthesia dolorosa, 748. 
in neuralgia, 833. 
in polyneuritis, 790. 
Aneurysm in tabes, 506. 
Angina pectoris in hysteria, 1071. 

in tabes, 487. 
Angioneurotic edema. See Acute circum- 
scribed edema. 
Anhedonia, 1069. 
Anisocoria, 207. 
Anorexia in hysteria. 1069. 
Anosmia in hysteria, 1069. 
Ansa nuclei lentiformis, 106. 
AnteFior thoracic nerve, paralysis of, 700. 
Antithyroidin, 972. 
Anuria in hysteria, 1076. 
Ape's hand in syringomyelia, 594. 
Aphagia algera, 1072. 
Aphasia, 146, 265, 328. 
conduction, 269. 

symptoms of, 274. 
diagnosis of, 320, 321. 
hysterical, 332. 
jargon, 271. 
motor, 13, 269, 331. 
prognosis of, 273. 
symptoms of, 272. 
optic, 12. 

pathology- of, 277, 283. 

sensory, 12, 269, 331. 

prognosis of, 271. 

symptoms of, 271. 

subcortical motor, 275. 

sensory, 275. 
transcortical motor, 279. 

sensory, 279. 
treatment of, 323. 
Aphemia, 267. 

symptoms of, 272. 
Aphonia, 163. 

hysterical, 333. 
Aphthongia, 948. 
Apoplexia canalis spinalis, 428. 
Apoplexy. See Cerebral hemorrhage. 
1173 



1174 



INDEX OF SUBJECTS 



Apoplectiform attacks in multiple sclerosis, 
568. 
in syringomyelia, 605. 
in tabes, 490. 
Arachnoid, 223. 
Arcencerde, 181, 1114. 

in hysteria, 1079. 
Arc, reflex, function of, 201. 
Aran-Duchenne's disease, 651. 
Arms, pental, 22, 23. 
Arterise sulci, 30. 

Arteries, cerebral, aneurysm of, 217. 
Artery, anterior cerebral, 26. 
basilar, 26. 
calcarine, 26. 
choroid, 26. 
intercostal, 30. 
lumbar, 30. 
middle cerebral, 26. 
posterior cerebral, 26. 
communicating, 26. 
spinal, 30. 
sacral, 30. 
Sylvian, 26. 
ventral, 30. 
vertebral, 26, 30. 
vertebrospinal, 30. 
Arthralgia in hysteria, 1070. 
Arthritis in syringomyelia, 600. 
Arthropathia tabidorum, 490. 
Articulation, spasm of, 339 
Ascending neuritis, 749. 
Aspermia, 993. 
Aspermatism, 994. 
Association system, 45. 

tracts, 268. 
Astasia-abasia, 1113. 
Astereognosis in polyneuritis, 807. 
Asthenia, 976. 
Asthenospermia, 993. 
Ataxia, 168. 
acute, 623. 

cerebellar, 171, 174, 485. 
hereditary, 612. 

clinical course of, 613. 
differential diagnosis of, 623. 
etiology of, 623. 
history of, 612. 
pathogenesis of, 620. 
pathology of, 615. 
prognosis of, 625. 
symptoms of, 613. 
ataxia in, 614. 

choreiform movements in, 614. 
coordination, disturbance of, in, 613 
lassitude in, 613. 
reflexes in, 614. 



Ataxia, hereditary, symptoms of, walking 
in, 613. 
treatment, 625. 

in epilepsy, 1019. 

in polyneuritis, 806. 

polyneuritis, acute, 791. 

reflex theory of, 521. 

sensory theory of, 517. 

static, 483. 

test of, 169. 

vasomotor, 1137. 
Ataxic polyneuritis, 805. 
Athetosis, 899. 

clinical history of. 899. 

pathology of, 902. 

prognosis of, 906. 

symptoms of, 901. 

theories of, 905. 

treatment of, 906. 
Atrophy, degenerative, 148. 

due to inactivity, 147. 

due to pressure, 147. 

inactivity, 147. 

muscular, in syringomyelia, 594. 

pressure, 147. 

progressive muscular, 70. See Progressive 
muscular atrophy. 

reflex, 147. 

simple, 148. 
Attacks, epileptic, 179. 

hysteric, 179. 

spasmodic, 179. 
Auditory nerve in polyneuritis, 792. 
Aura, epileptic, 180. 

intellectual, 1016. 
Aura cursativa, 1015. 
Aurae, 1014. 

Axillary nerve, paralysis of, 705. 
Axis cylinder, 40. 

albumin structure of, 40. 

process, 38. 
Axon. See Axis cylinder. 
Azoospermia, 993. 

Babinski's reflex, 202, 205, 614. 

in epilepsy, 1018. 

in hysteria, 1074. 
Barber's spasm, 1158. 
Bartholin's glands, 982. 
Basedow's disease, 960. 
Basedowsan, 972. 
Basophilia, 36. 
Bechterew's reflex, 725. 

tract, 92. 
Bed-sore, acute, 398. 
Bell's palsy, 662. 
Belly-dance, hysterical, 1060, 1080. 



INDEX OF SUBJECTS 



1175 



Beri-beri, 809. 

clinical picture of, 809. 

pathology of, 817. 
Bishat's furrow, 17. 
Blacksmith's spasm, 1158. 
Bladder, disturbance in, in polyneuritis, 792. 

evacuation of, center for, 985. 
Blepharospasm, 941. 
Blicklahmung, 788. 
Blood, examination of, 222. 

in epilepsy, 1019. 
Blood pressure, in epilepsy, 1023. 

transmissible, 227. 
Blood relationship, significance of, 131. 
Blue edema, 1075. 
Bone conduction, test of, 189. 
Bones, atrophy of, 148. 

friability of, 149. 

hypertrophy of, 149. 

hypoplasia of, 148. 

trophic changes in, 148. 
Borborygmi, 1077. 
Bottle cells, 110. 
Brachial plexus, 772. 
Brachialgia, 1072. 
Brachium eonjunctivum, 21, 22. 
Bradycardia, 595. 

in hysteria, 1075. 

in paralysis of the pneumogastric nerve, 
685. 

in polyneuritis, 787, 805. 
Bradylalia, 614. 
Bradyphasia, 568. 
Brain, abscess of, 216, 250, 310. 

acute softening of, 114. 

arterial supply of, 26. 

base of, structures at, 24. 

blood supply of, 226. 

convolutions of, 6. 

diseases of, diagnosis of, 213. 

fissures of, 6, 7. 

fossae of, 6. 

in tabes, 499. 

lobes of, 7. 

nervous diseases of, 71. 

pressure in, due to pathologic processes, 
230. 

relations of, to bony skull, 31, 32. 

richness of, in convolutions, 5. 

fc.and, 20. 

secondary degeneration in, 125. 

structures at base of, 24. 

tumor of, 216, 249, 310. 

weight of , 5. 
Brain tumor, headache in, 869. 
Broca's convolution, 8, 12. 
Bromid acne, in epilepsy, 1042. 
75* 



Brown-Sequard's paralysis, 352, 367, 598. 
Bruit de pot fele, 138. 

Bulbar nerves, paralysis of, in syringomy- 
elia, 595. 
Bulbus olfactorius, 86. 
Bulimia, 1069. 

Buphthalmus hystericus, 964. 
Burdach's column, 28. 

Cachexia in Graves' disease, 961. 
Caisson paralysis, 382, 427. 
Calcar avis, 16. 
Canal, central, 29. 
Capsule, external, 16. 

internal, 3, 15, 17. 
Caput obstipum, 952. 
Carbon bisulphid poisoning, 803. 
Carbonic oxid poisoning, 803. 
Cardialgia, hysterical, 1072. 
Carpenter's spasm, 1158. 
Carotid, internal, 26. 
Catalepsy, 1082. 
Cauda equina, 28. 

diseases of, 423. 
Cell process, 37. 

staining of, 38. 
Cell proliferation, 115. 
Cella media, 16. 
Cellist's cramp, 1156. 
Cells, granular, in myelitis, 115. 

motor ganglion, 28. 

proliferated, in myelitis, 115. 
Center for evacuation of bladder, 985. 

for erection, 986. 

of defecation, 9S5. 
Centers, cerebral, 11. 

higher psychical, 14. 

of association, 14. 

psychical, 14. 
Central fossa, 4. 
Central nervous system, diseases of, 113. 

embryologic structure of, 1. 

fluid of, 224. 

normal and pathological histology of, 34. 

structure of membranes of, 223. 
Central organs, motor, mechanism of, 150, 

151. 
Centrum ovale, 17. 
Cephalaea adolescentium, 858. 
Cerebellum, 21,22,23, 107. 

in tabes, 500. 

lobes of, 22. 

nervous diseases of, 112. 
Cerebral cortex, structure of, 63. 
Cerebral embolism, 258. 

etiology of, 260. 

symptoms of, 259. 



1176 



INDEX OF SUBJECTS 



Cerebral hemorrhage, 258, 310. 

etiology of, 261. 

symptoms of, 258, 259. 

treatment of, 261. 
Cerebral localization, 6, 11. 

softening, 310. 
Cerebropathia psychica toxaemia, 794. 
Cerebrospinal fluid, absorption of, 231. 

admixture of blood in, 234. 

albumin in, 235. 

coagula in, 235. 

composition of, 233. 

elastic pressure of, 227. 

estimating pressure in, 242. 

height of pressure of, 228. 

hydrostatic pressure of, 227. 

normal pressure of, 229. 

pathogenic microbes in, 234. 

rises in pressure of, 232. 

specific gravity of, 235. 

subnormal pressure of, 232. 

sugar in, 235. 

tubercle bacilli in, 249. 

variation of, 229. 
Cerebrum, 2. 

ganglia of, 15, 
Cervical cord, 28. 
Cervical enlargement, 28. 
Chapmann's tubes, 438. 
Charcot's disease, 656. 

edema, 1075. 
Cheilophagia,949. 
Chiasm, optic, 19, 24. 
Children, headache of, 856. 
Chlorosis in neuralgia, 839. 

lumbar puncture in, 251. 
Choked disc, 184. 
Cholin, 235. 

in cerebrospinal fluid, in epilepsy, 1019. 
Chorea, 178, 936. 

in hysteria, 1062. 
Chorea electrica, 936, 950. 

rhythmica, 936. 
Choreic contractions in syringomyelia, 593. 
Chromatolysis, 778. 
Chvostek's sign, 916. 
Cigar-maker's cramp, 1158. 
Cingulum, 104. 

Circumflex nerve, paralysis of, 705. 
Clarinet player's cramp, 1157. 
Clarke's column, 107. 
Claudication, intermittent, 786. 
Claustrum, 3, 16. 
Clavus, 1071. 

Claw-foot, in lead paralysis, 801. 
Claw-hand, 714. 

in median nerve paralysis, 717. 



Claw-hand in syringomyelia, 594. 

Clitoral crises, 985. 

Clonus, pattela, 198. 

Closure, automatic, 231. 

Cobbler's spasm, 1158. 

Coccygalgia, 1071. 

Coccygeal plexus paralysis, 736. 

Coccygodynia, 1071. 

Cochlea, 82. 

Colic, lead, lumbar puncture in, 251. 

Collaterals, 38. 

Colporrhea in hysteria, 1076. 

Column, anterior, 28. 

lateral, 28. 

posterior, 28. 
Column cells, 87. 
Columnar fornicis, 16. 
Commissure, anterior white, 29. 

posterior gray, 29. 
Commissures, cerebral, 16. 
Commotio cerebri, 1100. 

labyrinthi, 190,1109. 

medullas spinalis, 1100. 
Conception center in brain, 279. 
Concrete conception, 279, 287. 
Confabulations in polyneuritis, 794. 
Congressus interruptus, 134, 998. 
Conjugate deviation in epilepsy, 1017. 
Consensual dilatation, 209. 
Contraction, ideo-muscular, 178. 
Contracture, differentiation between active 
and passive, 154. 

hysterical, 154, 935. 

muscular, active, 153. 

passive, 154. 
Conus medullaris, 28. 

terminalis, 4. 
Convolutions, formation of, 4. 
Convulsions, 935. 

in traumatic neurosis, 1114. 
Coordination, centers of, 514. 

cerebellar, 514. 

cerebral, 514. 

examination of, 168. 
Coordination fibers, 104. 
Coordination system, 45. 
Coprolalia in hysteria, 1061. 
Cord. See also Spinal cord. 

cervical, 28. 

lumbar, 28. 

sacral, 28. 

spinal, 28. 

ascending degeneration in, 124. 
central canal of, 29. 
vascular supply of, 30. 

thoracic, 28. 
Cornea, reflex of, 208. 



INDEX OF SUBJECTS 



1177 



Cornet player's cramp, 1157. 
Cornu ammonis, 9, 17. 
Cornu ammonis, 17. 
Corona radiata, 98. 
Corpora amylacea, 374. 

mammillaria, 16, 19. 

quadrigemina, 20, 21. 

restiformia, 22, 107. 
Corpus callosum, 3, 104. 

dentatum, 22. 

geniculatum, 19, 86. 

striatum, 2, 3, 4, 15. 

trapezoideum, 90. 
Cortex, cerebellar, structure of, 110. 

cerebral, 14. 
structure of, 63. 

motor cerebral, 61. 

of brain, structure of, 44. 
Cough, spasmodic, in tabes, 487. 
Cowper's glands, 982. 
Coxalgia, hysterical, 1072. 
Coxitis, 1072. 
Coryza, nervous, 1146. 
Cramp in the leg, 958. 
Cramp, writer's, 1149. 
Cramps, 935. 
Crisis, gastric, 485. 
Crura fornicis, 16. 
Crural nerve, paralysis of, 726. 
diagnosis of, 727. 
etiology of, 728. 
symptoms of, 726. 
treatment of, 728. 
Cuneus, 10. 

Cutaneous reflexes, 155. 
Cyanosis in polyneuritis, 793. 
Cystalgia, 981. 
Cystospasm, 981. 
Cytodiagnosis, 139, 140, 219. 

in tabes, 505. 

Daltonism in tabes, 480. 

Dancer's cramp, 1157. 

Deaf-mutism, 325. 

Deafness in epilepsy, 1021. 

Debility, nervous, 976. 

Decubitus, 398. 

Decussation, pyramidal, 28, 68, 69. 

Defecation, center of, 985. 

Defecation spermatorrhea, 985. 

Degeneration, ascending columnar, 372. 

descending columnar, 372. 

retrogressive, 778. 

secondary, 120. 

stigmata of, 136. 
Deiter's cells, 43, 375, 

nucleus. 107. 



Delirium tremens in polyneuritis, 794. 
Deltoid, atrophy of, 146. 
paralysis of, 705. 

etiology of, 705. 

prognosis of, 706. 

symptoms of, 705. 

treatment of, 706. 
Dementia, paretic, 219, 1034. 
Dendrites, 38. 

Dental arches in lisping, 345. 
Dercum's disease, 143. 
Dermatomyositis, 143. 
Dermographia, 142, 143, 1113. 
Determann's method in the treatment of 

tabes, 541. 
Diabetes insipidus in syringomyelia, 605. 
Diagnosis, general neurological, 129. 
Diamond cutter's cramp, 1158. 
Diaphoresis in treatment of neuritis, 775. 
Diaphragm, paralysis of, 695. 

prognosis of, 696. 

symptoms of, 696. 

treatment of, 697. 
spasm of, 956. 
Diaphragmatic respiration, 787. 
Diencephalon, 3. 
Dinkler's reflex, 725. 
Diplegia facialis, 675. 
Discharge theory in epilepsy, 1026. 
Disease, combined system, 113. 
Dissociated sensory paralysis, 194. 
Disturbances of speech, 325. 
Dropsy, ventricular, 226. 
Drummer's paralysis, 769. 
Duchenne-Erb type of paralysis, 721. 
Dumbness, idiopathic, 326. 
Dura, slitting of, 252. 
Dura mater, 223. 

Dural nerves, hyperesthesia of, 868. 
Dynamometer, 165. 
Dysarthria, 162. 
Dyslexia, 297. 
Dyspnea, 595. 

Dystrophia muscularis progressiva, 147. 
Dysuria, 981. 

Echolalia, 274. 
Eclampsia, 1029. 
Ectoderm, 1. 
Edema, flying,. 142. 

in hysteria, 1075. 

in nervous disease, 142. 

in syringomyelia, 600. 

of head, 215. 
Edinger's compensation theory, 427, 467, 

621, 768. 
Effusions, subdural, 252, 



1178 



INDEX OF SUBJECTS 



Egophony, 163. 

Ehret's contracture, 1107. 

Ejaculatio praecox, 991. 

Ejaculatory center, 986. 

Emaciation in Graves' disease, 961. 

Embolus, 22. 

Eminence, anterior, 21. 

posterior, 21. 
Emissions, nocturnal, 983. 
Encephalitis, 114. 

chronic, 118. 
Encephalopathia saturnina, 801. 
Encephalopathy, lead, 800. 
Endarteritis, syphilitic, 126. 
Enteric fever, lumbar puncture in, 251. 
Enuresis nocturna, 1029. 
Epidermolysis bullosa hereditaria, 1145. 
Epilepsia procursiva, 1022. 
rotatoria, 1022. 
spasmodico-paralytica, 1020. 
tarda, 1030. 
Epilepsie marmottante, 1022. 

retropulsive, 1022. 
Epilepsy, 1012. 

abortive attacks of, 1021, 1039. 
alcoholic, 1035. 
apoplectiform, attacks of, 1038. 

variety of, 1021. 
arteriosclerotic, 1039. 
atypical attacks of, 1021. 
cortical, 957. 

description of attack of, 180. 
diagnosis of, 1037. 
differential diagnosis of, 1039. 

from hysteria, 1039. 
etiology of, 1031. 
alcohol in, 1032. 
ammonium carbonate in, 1035. 
chronic disease in, 1034. 
disorders of metabolism in, 1034. 
heredity in, 1032. 
infectious diseases in, 1033. 
poisons in, 1035. 
psychical trauma in, 1036. 
syphilis in, 1033. 
tobacco in, 1035. 
trauma in, 1033, 1035. 
fully developed, a typical attack of, 1021. 
genuine, 1013. 
history of, 1012. 

interval between attacks of, 1028. 
Jacksonian, 1013, 1038. 
larval, 1029. 
lead, 1035. 

lumbar puncture in, 250. 
malingering in, 1040. 
migraine and relations of, 1032. 



Epilepsy, menstrual type of, 1033. 
nocturnal, 1029. 
parasyphilitic, 1033. 
pathology of, 1037. 
primary, 1031. 
prognosis of, 1030. 
reflex, 1030, 1035. 
rudimentary attack of, 1021, 
series of attacks in, 1029. 
simulation in, 1040. 
spinal, 386. 

symptomatic, 1013, 1033. 
symptoms of, 1013. 

after the attack, 1018. 

amblyopia in, post-paroxysmal, 1021. 

aphasia in, 1020. 

ataxia in, 1019. 

aura cursativa in, 1016. 

aura in, 1015. 

aura of the special senses in, 1015. 

Babinski's toe reflex in, 1018. 

blood pressure in, 1023. 

cholin in cerebrospinal fluid in, 1019. 

clonic spasms in, 1017. 

contractions in, 1018. 

convulsions in, 1017. 

cutaneous hemorrhages in, 1023. 

cyanosis in, 1023. 

deafness in, 1021. 

diarrhea in, 1018. 

digestive disturbances in, 1018. 

edema in, 1019. 

evacuation of feces in, 1024. 
of semen in, 1024. 
of urine in, 1024. 

globus in, 1015. 

hemoglobin in, 1019. 

initial cry in, 1023. 

isolated clonic convulsions in, 1018. 

intellectual aura in, 1016. 

locomotor movements in, 1018. 

loss of power in, 1019. 
of weight in, 1018. 

lymphocytosis of cerebrospinal fluid in, 
1019. 

motor aura in, 1016. 

nystagmus in, 1019. 

Oppenheim's phenomenon in, 1018. 

pallor in, 1023. 

paresis in, 1019. 

polyuria in, 1018. 

post-paroxysmal phenomena in, 1018. 

premonitory, 1014. 

prodromes in, 1014. 

psychic aura in, 1016. 

pupils in, 1024. 

remote prodromes in, 1014. 



INDEX OF SUBJECTS 



1179 



Epilepsy, symptoms of, saliva in, 1024. 
sensory aura in, 1015. 
somatic prodromes in, 1014. 
temperature in, 1018. 
tendon reflexes in, 1019. 
the attack, 1016. 
tongue in, 1019, 1024. 
tonic spasm in, 1017. 
toxicity of urine in, 1019. 
tremor in, 1018. 
urine in, 1018. 
vasomotor aura in, 1016. 
vasomotor prodromes in, 1014. 
visceral aurse in, 1016. 
vision limited in, 1021. 
vomiting in, 1024. 
syncopal conditions in, 1038. 
tabes and, 1034. 
traumatic, treatment of, 1041. 
treatment of, 1040. 
bromids in, 1041. 
chloral hydrate in, 1042. 
diet in, 1041. 
drugs in, 1042. 
hygiene in, 1041. 
opium in, 1042. 
surgical, 1041. 
theories of, 1024. 
toxicity of urine in, 1034. 
varieties of the attack of, 1021. 
Epileptic equivalent, 1023. 
spasms in tetany, 917. 
stupor, 1021, 1029. 
vertigo, 1023. 
Epileptiform attacks in tabes, 490. 
Epiphysis, 19, 20. 
Equilibrium, test of, 170. 
Erb's sign, 915. 
Erection, center for, 986. 
Ergograph, 341. 
Ergotin tabes, 530. 
Erogenous zone, 985. 
Erethism, 976. 

Erythema in polyneuritis, 793. 
Erythromelalgia, 1139. 
course of, 1140. 
diagnosis of, 1140. 
etiology of, 1139. 
in arsenical polyneuritis, 799. 
pathology of, 1140. 
symptoms of, 1139. 
treatment of, 1141. 
Erythrophobia, 141. 

Examination of patients with nervous dis- 
ease, 129. 
Exhaustion, excessive, 976. 
Exhibitionism, 981. 



Exothyreopexy, 973. 
Exophthalmic goiter, 960. 
Exophthalmus cachectique, 961. 
External cutaneous femoral nerve, paralysis 
of, 729. 
prognosis of, 730. 
symptoms of, 729. 
treatment of, 730. 
Extremities, atrophy of muscles of, 147. 
lower, center for, 61. 
upper, center for, 61. 
Extremity, muscles of, examination of, 152. 
Eye, movements of, center for, 63. 
Eye muscles, paralysis of, in polyneuritis, 
806. 
paresis of, 158. 
Eyelid, edema of, 1146. 
Eyelids, convulsive movements of, 158. 

tremor of, 158. 
Eyes, accommodation of, 208. 
movements of, 158. 

Face, expression of, in nervous disease, 136. 

muscles of, at rest, 151. 
Facial expression in diagnosis, 136. 
hemiatrophy, 146, 1142. 
muscles, hysterical contracture of, 944. 
nerve, origin of, 56. 
paralysis of, 662. 
bilateral, 674. 

complications of, contractures in, 668. 
congenital, 673. 
differential diagnosis between central 

and peripheral lesions in, 669. 
electric reactions in, 665. 
etiology of, 667. 

diseases of the ear in, 667. 
infectious diseases in, 668. 
neuritis in, 66S. 
poisons in, 668. 

recurrence during labor in, 667. 
refrigeration in, 667. 
syphilis in, 667. 
hysterical, 676. 
in syringomyelia, 595. 
onset of, 665. 
pathology of, 671. 
reactions of degeneration in, 666. 
relapse in, 667. 
symptoms of, 662, 663, 664. 
anomalies of secretion in, 664. 
herpes in, 664. 
pain in, 665. 
sensation in, 664. 
trophic changes in, 664. 
vasomotor signs in, 664. 
treatment of, 672. 



1180 



INDEX OF SUBJECTS 



Facial paralysis, treatment of, antifebrin 
in, 673. 
antipyrin in, 673. 
electricity in, 673. 
hot applications in, 673. 
Facial paralysis in polyneuritis, 788. 
Facial spasm, 943. 
Falsetto voice, 344. 
Family spastic spinal paralysis, 624. 
Fango in treatment of neuritis, 776. 
Fascia dentata, 9, 17. 
Fasciculus arcuatus, 104. 

fronto-occipitalis, 104. 

longitudinalis inferior, 104. 

uncinatus, 104. 
Feces, reflex for discharge of, 209. 
Fellation, 998. 
Fetichism, 981. 
Fever, hysterical, 1076. 
Fibers, centrifugal, 45. 

centripetal, 45. 
Fibrae arcuatae internee, 89. 
Fibrillae theory, 46. 
Fibrillary contraction, 177, 933, 934. 

in syringomyelia, 595. 
Fibrillary net of basic substance, 37. 
Fifth nerve. See also Trigeminal nerve. 

ascending root of, 24. 
Filum terminale, 28. 
Fimbria, 16, 17. 
Fissura calcarina, 9. 
Fissure, parieto-occipital, 10. 

Sylvian, 9. 
Fixed ideas in hysteria, 1077. 
Flagellation, 981. 
Flat-hand, neuritic, 763. 
Flocculus, 22. 
Fluid, cerebrospinal, outlet of, 226. 

regulating effect of, 226. 
Flute player's cramp, 1157. 
Flying edema, 1113. 
Folie musculaire, 936. 
Foramen Monroi, 3. 
Formication, 744. 

in polyneuritis, 806. 
Fornix, 3, 16. 
Fossa, Sylvian, 4, 9. 

central, 4. 

formation of, 4. 

rhomboid. See Ventricle, fourth. 

supraspinatus, depression of, 146. 
Fossae, cerebral, importance of, 5. 

transitory, 4. 
Friedreich's ataxia, 112. 
Friedreich's disease. See Hereditary 
ataxia. 

ataxia in, 484. 



Frenkel's reeducation method, 549. 

Frigidity, 992. 

Function of motion, segmentary localization 

of, 404, 405. 
Function paralysis, 1150. 
spasm, 1150. 

Gait, ataxic, 173, 483. 

equine, 173. 

investigation of, 172. 

limping, 175. 

spastic, 173. 

in paralysis agitans, 886. 

spastic-paretic, 173. 

steppage, 172, 786. 

sweeping, of hysteria, 175. 

waddling, 172. 
Gait-stuttering, 1060. 
Galactorrhea, 1076. 
Gall's theory, 214. 
Galvanic resistance, decreased in Graves' 

disease, 961. 
Ganglia cerebral, 3. 

anterior, 3. 
Ganglia nodules, 73. 
Ganglion, spinal, 82. 
Ganglion habenulae, 19, 20. 
Gangrene due to ergot, 1138. 
Gasserian ganglion, 102. 
Gastrectasis, tetany in, 920. 
Gastric crises in Graves' disease, 961. 

juice, examination of, in tabes, 486. 
Geigel's reflex, 725. 
Genital neurosis, 995. 
Giantism, 149. 

Glance paralysis in polyneuritis, 788. 
Gland, pineal, 19, 20. 
Glia nuclei, 43. 

tissue, structure of, 43. 
Gliomata, 588. 
Gliosis, 120, 588. 
Globus, 1111. 

in epilepsy, 1015. 

in hysteria, 1078. 
Globus pallidus, 15. 

Glassopharyngeal nerve, paralysis of, 681. 
etiology of, 682. 
prognosis of, 682. 
symptoms of, 682. 
treatment of, 682. 
Glassoplegia, 690. 
Glossy skin, 143. 
Gluteal fold, significance of , 152. 

nerves, paralysis of, 730. 
Glycosuria in Graves' disease, 961, 

in tabes. 490. 

in tetany, 917. 



INDEX OF SUBJECTS 



1181 



Glycosuria in traumatic neurosis, 1116. 

Golgi method, 38. 

Goll's column, 28. 

Gonalgia, 1072. 

Gonitis, 1072. 

Goose-flesh reflex, 1112. 

Gorgon contracture, 1056. 

Gowers' bundle, 107. 

Graefe's sign, 158. 

in Graves' disease, 961. 
Grands mouvements, 1080. 
Granulation tumors in polyneuritis, 793. 
Graphospasm, 1150. 
Gratiolet, optic radiation of, 16. 
Graves' disease, 960. 
diagnosis of, 962. 
direct transmission of, 974. 
history of, 960. 
rudimentary form of, 961. 
symptoms of, 961. 

cardiac palpitation in, 961. 
diarrhea in, 961. 
exophthalmos in, 961. 
goiter in, 961. 
muscular weakness in, 961. 
pigmentation in, 961. 
sweating in, 961. 
tachycardia in, 961. 
tremor in, 961. 
theories of, 963. 
treatment of, 970. 
diet in, 970. 
electricity in, 971. 
exothyreopexy in, 973. 
hydrotherapy in, 970. 
operative measures in, 973. 
organotherapy in, 971. 
psychotherapy in, 971. 
surgery in, 971. 
thyreoidectomy in, 973. 
Gray substance, 28. 
Gray substance, structure of, 42. 
Greeting spasm, 950. 
Grinder's disease, 562. 
Gyrus angularis, 8. 
centralis, 8. 
cinguli, 9. 
descendens, 10. 
fornicatus, 9. 
frontalis, 8, 10. 
fusiformis, 9. 
hippocampi, 9, 17. 
lingualis, 9, 10. 
occipitalis, 9 
parietalis, 8. 
supramarginalis, 8. 
temporalis, 9. 



Hair, grayness of, 145. 

in nervous disease, 145. . 
Hand, grasp of, 165. 
Harp player's cramp, 1157. 
Head tetanus, 937. 
Head's zones, 1071. 
Headache, 851. 
anemia, 859. 
theories of, 860. 
treatment of, diet in, 860. 
iron in, 860. 
as a symptom, 851. 
chronic, 856. 
diagnosis of, 855. 
due to brain tumor, 869. 
due to intracerebral disease, 869. 
etiology of, 852. 
eyes in, 857. 
frontal sinuses in, 852. 
middle ear in, 852. 
pharynx in, 852. 
teeth in, 852. 
examination of eye ground in, 858. 

of urine in, 858, 869. 
hysterical, 853. 

clinical history of, 853. 
in children, S56. 
indurative, 863. 

clinical history of, 864. 
etiology of, 864. 
.symptoms of. 86 I, 865. 
treatment of, N<>(>. 
electricity in, 866. 
massage in, 866. 
sodium salicylate in, 866. 
in syringomyelia, 605. 
intracranial, treatment of, 869. 
of adolescents, 858. 
organic, 863. 
symptoms of, 854, 855. 
syphilitic, 869. 
diagnosis of, 870. 
symptoms of, 869. 
treatment of, 870. 
theory of, 875, 876, 877. 
vasomotor, diagnosis of, 862. 
eye-grounds in, 862. 
symptoms of, 861. 
treatment of, 862. 
vasoparalytic, 861. 
Heart, valvular lesions of, in tabes, 492. 
Hematomyelia, 374 
diagnosis of, 428. 
traumatic, description of, 399. 
Hemialgia, 1071. 
Hemianopsia, 16, 186. 
Hemiatrophia facialis, 680. 



1182 



INDEX OF SUBJECTS 



Hemichorea, 900. 
Hemiepilepsy, idiopathic, 1020. 
Hemispasmus glossolabialis, 944. 
Hemispheres, cerebral, commissures of, 16. 
Hemorrhage in hysteria, 1076. 

in nervous disease, 142. 
Hemoptysis in traumatic neurosis, 1116. 
Hereditary ataxia. See Friedreich's ataxia. 
Hereditary muscular atrophy, 639. 
Hereditary spastic spinal paralysis, 627. 

differential diagnosis of, from Little's dis- 
ease, 631. 

etiology of, 630. 

pathology of, 631. 

symptoms of, 630. 

treatment of, 632. 
Heredo-ataxie, 612. 
Herpes, 144. 
Herpes zoster, changes in, 103. 

in neuralgia, 829. 

in tabes, 492. 
Hidrorrhea in hysteria, 1076. 
Hippus, 1112. 
Hirsuties, 145. 
History, factors of importance in, 131-134. 

taking of, in patients with disease of ner- 
vous system, 130. 
Hoffmann's sign, 915, 916. 
Horn, anterior, 16, 28. 

lateral, 28. 

lower, 16. 

posterior, 16, 28. 
Huntingdon's chorea, 624, 936. 
Hutchinson's teeth, 145. 
Hydrocephalus, 218. 

chronic, 246. 

congenital, 233. 
Hydrops articulorum intermittens, 1146. 
Hyperdipsia in hysteria, 1069. 
Hyperesthesia plantaris, 785. 
Hyperhedonia, 1069. 
Hypertrichosis, 142. 

in hysteria, 1076. 

in polyneuritis, 793. 
Hypertonia, 153. 
Hypertrichosis, 145. 
Hypertrophy of muscles, 147. 
Hyperthyreosis, 972. 
Hypnoid attacks in hysteria, 1083. 
Hypoglossal nerve, paralysis of, 690. 
diagnosis of, 692. 
etiology of, 690. 
prognosis of, 693. 
symptoms of, 691. 
treatment of, 693. 
Hypophysis, 24. 

in acromegalia, 24, 



Hypospermia, 993. 
Hypotonia, 153, 157. 
Hysteria, 1045. 
course of, 1084. 
definition of, 1045. 
description of, 181. 
differential diagnosis of, 1085. 

from brain syphilis, 1088. 

from brain tumor, 1088. 

from chorea, 1086. 

from dementia paralytica, 1088. 

from disease of the vertebrae, 1089. 

from diseases of other organs, 1090. 

from epilepsy, 1085. 

from focal diseases of the brain, 1088. 

from meningitis, 1089. 

from multiple sclerosis, 1087. 

from myelitis, 1089. 

from neurasthenia, 1087. 

from neuritis, 1089. ' 

from organic disease of the nervous sys- 
tem, 1087. 

from syringomyelia, 1089. 

from tetany, 918. 
etiology of, 1047. 

age in, 1048. 

chronic intoxication in, 1050. 

exhaustion in, 1050. 

genitalia in, 1050. 

hereditary predisposition in, 1047. 

imitation in, 1049. 

infections in, 1050. 

psychical influences in, 1049. 

sex in, 1048. 

trauma in, 1049. 
imitation, 1049. 
lumbar puncture in, 250. 
migraine and, 1072. 
prognosis of, 1084. 
reflex, 1050. 
superimposed, 1047. 
symptoms of, 1050. 

abasia in, 1051. 

acceleration of respiration in, 1060. 

amaurosis in, 1067. 

aphasia in, 1051, 1053. 

arthralgia in, 1070. 

arthritic hyperalgesia in, 1065. 

astasia in, 1051. 

attack of, termination of, 1081. 

attack of, variations in, 1082. 

aura in, 1078. 

bradycardia in, 1075. 

cataleptic seizures in, 1082. 

chorea in, 1062. 

Chvostek's sign in, 1056. 

clonus in, 1059. 



INDEX OF SUBJECTS 



11S3 



Hysteria, symptoms of , contractures in, 1055. 
coprolalia, 1061. 
cutaneous reflexes in, 1073. 
delirious phase in, 1080. 
disorders of motility in, 1051. 
disturbances of coordination in, 1062. 

of gait in, 1060. 

of hearing in, 1069. 

of metabolism in, 1076. 

of sight in, 1067. 

of smell in, 1069. 

of speech in, 1060. 

of taste in, 1069. 

of the sexual sphere in, 1069. 
edema in, 1075. 
electric contractility in, 1054. 
epileptoid phase in, 1079. 
fever in, 1076. 
field of vision in, 1068. 
fixed ideas in, 1077. 
gait-stuttering in, 1060. 
hemiplegias in, 1051. 
hemorrhage in, 1076. 
hypnoid attacks in, 1083. 
intestinal disturbance in, 1076. 
menstruation in, 1076. 
monoplegias in, 1051. 
mucous membrane reflexes in, 1073. 
muscles of the eye in, 1053. 

of the tongue in, 1052. 
mutism in, 1055. 
narcolepsy in, 1083. 
neuralgia in, 1071. 
paralysis in, 1051. 

reflexes in, 1054. 
paraplegias in, 1051. 
phase of spasmodic movements in, 1079. 
pressure points in, 1066. 
prodromes in, immediate, 1078. 

remote, 1078. 
pseudo-neuralgia in, 1071. 
pseudoptosis in, 1053. 
psychic phenomena in, 1077. 
pupillary reflexes in, 1073. 
reflexes in, 1073. 
regional paralysis in, 1052. 
secretory disturbances in, 1076. 
sensory disturbances in, 1063-1065. 
singultus in, 1060. 
sleeping attacks in, 1083. 
somatic signs in, 1051. 
somnambulism in, 1083. 
spasms in, 1059. 
spasmodic attack in, 1078. 
spontaneous pains in, 1071. 
stuttering in, 1060. 
sympathetic nervous system in, 1075. 



Hysteria, symptoms of, tachycardia in, 
1075. 

thermoanesthesia in, 1067. 

temperature in, 1075. 

tendon reflexes in, 1073. 

topoalgia in, 1070. 

transference in, 1064. 

tremor in, 1061. 

Trousseau's sign in, 1056. 

vertigo in, 1069. 

vocal cords in, 1053. 

vasomotor phenomena in, 1075. 

zonifugal test in, 1065. 

zonipetal test in, 1065. 
traumatic, 1048. 
treatment of, 1091. 

bromids in, 1097. 

drugs in, 1097. 

education in, 1091. 

electricity in, 1096. 

exercise in, 1095. 

general management in, 1092. 

hydrotherapy in, 1096. 

hypnotism in, 1097. 

psychotherapy in, 1093. 
zones of analgesia in, 597. 
Hysterical abasia, 1054. 
akathisia, 1054. 
anarthria, 1055. 
astasia, 1054. 
attacks in tetany, 918. 
shaking spasm, 1022. 
tachypnea, 1060. 
Hysteroepilepsy, 1040, 1081, 1086. 

Ichthyosis, 143. 

Ictus laryngeus, 487. 

Idee fixe, 141. 

Impotence, 988. 

Infantile bilateral cerebral paralysis, 631. 

Influenza, lumbar puncture in, 251. 

Infundibulum, 20. 

Inspection in diseases of brain, 214. 

Inter-brain, 2, 3, 19. 

Intermittent claudication, 174, 175, 1138. 

Intention rigidity, 926. 

Internal capsule, 15, 64. 

Iodin reaction, 222. 

Iodothyrin in Graves' disease, 972. 

Irritable bladder, 984. 

Irrumation, 998. 

Ischuria, 981. 

Isthmus, 9. 

Jacksonian epilepsy, 1013, 1038, 1082. 
Joints, hysterical swelling of, 149. 
trophic changes in, 148. 



1184 



INDEX OF SUBJECTS 



Kakke. See Beri-beri. 
Kernig's sign, 157. 
Kinesitherapy, 1006. 
Klumpke's paralysis, 722. 
Korsakow's syndrome, 794. 

Labyrinth, ganglia of, 83. 
Labyrinthine shock, 1109. 
Lachrymation in hysteria, 1076. 
Lachschlag, 1039. 
Lamina cribrosa, 86. 
Landry's paralysis, 824. 

clinical history of, 824. 

course of, 825. 

pathology of, 825. 
Lantermann's segments, 42. 
Larval epilepsy, 1029. 
Laryngospasm in syringomyelia, 593. 
Larynx, center for, 61. 

muscles of, testing, 162. 

paralysis of, 683. 
Lasegue's symptom, 1089. 
Lathyrus poisoning, 627. 
Laughing, significance of, 159. 
Law of degeneration, 125. 
Lead line in polyneuritis, 800. 
Lead paralysis, 655. 
Lemniscus, 92, 93. 

layer, 24. 
Leptomeninx, 223. 
Leukemia, lumbar puncture in, 251. 
Leukomyelitis, 376. 
Leukoplakia in Graves' disease, 961. 
Leukotrichia, 834. 
Libido, deficient, 988. 
Liebreich's protagon, 42. 
Lipochromes, 37. 
Lip-reading, 329. 
Lips, atrophy of, 146. 
Lisping, 345. 
Lissauer's zone, 77. 

Litten's diaphragm phenomenon, 696, 956. 
Little's disease, 631. 
Lobe, frontal, 7. 

occipital, 8. 

olfactory, 106. 

paracentral, 8. 

parietal, 8. 

quadrangular, 22. 

temporal, 9. 
Lobes of brain, 7. 

olfactory, 2. 
Local syncope, 1132. 
Locksmith's spasm, 1158. 
Locomotor ataxia. See Tabes dorsalis. 
Locus cceruleus, 55, 79. 
Lowenthal's bundle, 124. 



Lower extremities, spasm of, 957. 
Lumbar cord, 28. 
Lumbar enlargement, 28. 
Lumbar puncture, 139, 218, 223. 

anatomical relations in, 238. 

antitoxin introduced by, 255. 

difficulties in, 243. 

drugs ingested by, 254. 

hemorrhage in, 251. 

indications for performing, 244. 

method of performing, 236. 

therapeutically, 254. 
Luy's body, 20. 
Lymphocytosis in tabes, 502. 
Lymphocytosis of cerebrospinal fluid, 219. 
in epilepsy, 1019. 
significance of, 219. 
Lymphangitis, syphilitic, in tabes, 503. 
Lyra Davidis, 106. 

Microcephalia, 214. 
Magendie, foramen of, 22. 
Maladie des tics, 935, 1059. 
Malingering, 1040, 1090. 
Mai perforant, 144. 
in polyneuritis, 793. 
in tabes, 492. 
Manege epilepsy, 1022. 
Marchi's method, 115. 
Marie's disease, 614. 
Marriage in Graves' disease, 974. 
Masochism, 981. 
Mastalgia, 1071. 
Mastication, muscles of, 161. 

spasm of, 949. 
Masticatory facial paralysis, 676. 

lingual spasm, 947. 
Masturbation, effects of, in nervous disease, 

134. 
Masturbation, influence of, 134. 
Median nerve, paralysis of, 715. 

claw-hand in, 717. 

clinical case of, 715. 

diagnosis of, 718. 

etiology of, 716. 

prognosis of, 718. 

symptoms of, 717. 

treatment of, 718. 
spasm of, 954. 
Medulla oblongata, 23. 
Medullary sheath, structure of, 42. 
Memory pictures, optical, 291. 
Meniere's disease, 190, 1039. 

symptom-complex, in tabes, 490. 
Meningeal edema, acute, 226. 
Meninges, syphilosis of, 463, 503. 
Meningitis, acute, 246. 



INDEX OF SUBJECTS 



1185 



Meningitis, purulent, 221, 247. 

serous, 218, 221. 

syphilitic, 502. 

tuberculous, 218, 221, 249. 
Menstruation, 212. 

in hysteria, 1076. 
Mental disturbance in polyneuritis, 794. 
Meralgia paresthetica, 729, 748. 
Mesencephalon, 3. 

Metabolism, disturbances of, in hysteria, 
1076. 

in Graves' disease, 961. 
Meteorism, hysterical, 1077. 
Metencephalon, 2, 3. 
Method of teaching deaf-mutes to speak, 

327. 
Microcephalia, 214. 

Microorganisms in cerebrospinal fluid, 220. 
Micturition spermatorrhea, 985. 
Mid-brain, 3, 20. 
Migraine, 870. 

etiology of, 871. 

in tabes, 490. 

hysteria and, 1072. 

lumbar puncture in, 251. 

relation of, to epilepsy, 875. 

scotoma in, 875. 

symptoms of, 870, 871. 

treatment of, 873. 

drugs in, 873. 

Milker's cramp, 1158. 

paralysis, 716. 
Mimetic paralysis, 662. 
Mind blindness, 12, 296. 

deafness, 12. 
Miners, nystagmus of, 1158. 
Mirror writing, 300. 
Mobius' sign in Graves' disease, 961. 
Mogigraphia, 1150. 
Mogiphonia, 1158. 
Monakow's bundle, 124. 
Monakow's fibers, 69. 
Monakow's tract, 112. 
Mononeuritis, post-infectious, 748. 
Monro, foramen of, 17. 
Morbus astralis, 1012. 

daemonicus, 1012. 

lunaticus, 1012. 

sacer, 1012. 
Morvan's disease, 593. 
Motility, 149. 

passive, 153. 
Motor, central organs, mechanism of, 150. 
Motor functions, spinal localization of, 406, 

407, 408. 
Motor speech center, 268. 
Motor tract, 48. 



Mouth, muscles of, 158. 
Movement, athetotic, 178. 

choreic, 178. 
Movements, affective, 159. 
conjoined, 935. 
coordinated, 175. 
voluntary, 150. 
Miiller's muscle, 151. 

paralysis of, 415. 
Multiple sclerosis, 557. 
course of, 570. 
differential diagnosis of, 575. 

from brain tumor, 578. 

from cerebral diplegia, 577. 

from cerebral hemorrhage, 577. 

from cerebral softening, 577. 

from cerebrospinal syphilis, 578. 

from encephalitis, 577. 

from hysteria, 579. 

from paralysis agitans, 578. 

from progressive paralysis, 577. 
etiology of, 558. 

age in, 558. 

alcoholism in, 561. 

infectious diseases in, 560. 

intoxications in, 561. 

metallic poisons in, 561. 

pregnancy in, 561. 

psychical factors in, 559. 

refrigeration in, 560. 

sex in, 558. 

syphilis in, 561. 

trauma in, 562. 
history of, 557. 
pathology of, 571. 

axis cylinder in, retention of, 572. 

general, 573. 

histology of lesions in, 571. 

lesions in brain in, 571. 

lesions in cord in, 571. 

optic nerve, in lesions of, 572. 

vessels in, 572. 
prognosis of, 570, 580. 
subacute cases of, 570. 
symptoms of, 563. 

abortive types in, 569. 

apoplectiform attacks in, 568. 

ataxia in, 565. 

atrophy in, 564. 

bladder disturbance in, 565. 

bradyphasia in, 568. 

cranial nerves in paralysis of, 567. 

dementia in, 569. 

dysuria in, 565. 

irregular types in, 569. 

motor disturbance in, 565. 

myotonic disturbance in, 594. 



1186 



INDEX OF SUBJECTS 



Multiple sclerosis, symptoms of, nystagmus 
in, 563. 

optic nerve in, 566. 

pupils in, 567. 

psychical disturbance in, 568. 

reflexes in, 564. 

scanning speech in, 568. 

sensory disturbance in, 566. 

spasm in, 568. 

spastic pareses in, 564. 

speech in, disturbance of, 568. 

tremor in, 563. 

trophic symptoms in, 566.. 

vasomotor signs in, 566. 

vision in, 567. 
treatment of, 580. 

arsenic in, 580. 

baths in, 580. 

drugs in, 580. 

electricity in, 580. 

ergot in, 580. 

exercise in, 580. 

quinin in, 580. 

rest in bed in, 580. 

silver nitrate in, 580. 

sodium iodid in, 580. 
tremor in, 177. 
Munk's centers, 14. 
Murmurs in skull, 138. 
Muscle deliria, 181. 
elasticity of, 152. 
tonus of, 150. 
waves, 177. 
Muscles, atrophy of, in neuralgia, 835. 
electric examination of, 148. 
hypertrophy of, 147. 
mechanical examination of, 148. 
of abdomen, paralysis of, 725. 
of back, function of, 164. 

paralysis of, 724. 
of chest, spasm of, 956. 
of legs, examination of, 166. 
of mastication, spasm of, 948. 
of neck, paralysis of, 724. 

spasm of, 949. 
of respiration, paralysis of, 724. 
of trunk, spasm of, 956. 
of upper extremity, function of, 164. 
pseudo-hypertrophy of, 147. 
spasticity of, 153. 
trophic disturbance of, 145. 
Musculocutaneous nerve, paralysis of, 707. 

diagnosis of, 707. 

prognosis of, 708. 

symptoms of, 707. 

treatment of, 708. 
Musculospinal paralysis, 708. 



Mutism, complete, 333. 
Myasthenia, 166. 

gravis, 660. 
Mydin sheath, 40. 
Mydriasis, 207. 
Myeleecephalon, 3. 
Myelin sheath, 40. 
Myelite cavitaire, 589. 
Myelitis, 371. 

acute, clinical picture of, 382, 383. 
etiology of, 424, 425. 

chemical poisons in, 426. 
exertion in, 427. 
infectious diseases in, 425. 
microorganisms in, 424. 
trauma in, 426. 
acute disseminated, 377. 
bulbar, 417. 
cervical, 413. 

cerebral symptoms in, 414. 
fall in temperature in, 416. 
glycosuria in, 417. 
headache in, 416. 
inferior type of, 413. 
Klumpke's paralysis in, 414. 
pain in, 416. 
priapism in, 417. 
reflexes in, 416. 
superior type of, 413. 
symptoms of, 413, 414. 
complicated with spinal meningitis, 415. 
compression, 380. 
contusion, 426. 
differential diagnosis of, 428. 

from Brown-Sequard's paralysis, 429. 
from caudal disease, 423. 
from compression of the cord, 429. 
from hysteria, 432. 
from Landry's paralysis, 430. 
from meningitis, 429. 
from paraplegia due to embolism, 429. 
from polyneuritis, 431. 
from tumor, 430. 
dorsal, 384. 

acute bed-sore in, 398. 

anesthesia dolorosa in, 390. 

angina pectoris in, 389. 

apoplectiform development of, 385. 

arthropathies in, 399. 

atrophy in, 387. 

Babinski's reflex in, 396. 

bed-sores in, 397. 

bladder in, 396. 

cerebral symptoms in, 402. 

chronic forms of, 385. 

cystitis in, 397. 

ear symptoms in, 402. 






INDEX OF SUBJECTS 



1187 



Myelitis, dorsal, fever in, 384. 

gastric crises in, 389. 

heart in, 402. 

hyperalgesia in, 390. 

hyperesthesia in, 390. 

incontinentia paradoxa in, 396. 

male potency in, 397. 

menstruation in, 397. 

muscle contractions in, 390. 

optic nerve in, 402. 

pain in, 389. 

paralysis in stage of, 386. 

paralysis of sphincters in, 396. 

paresthesia in, 389. 

paroxysmal cause in, 385. 

priapism in, 397. 

pregnancy in, 397. 

prodromal stage of, 384. 

pulmonary complications in, 402. 

reactions of degeneration in, 388. 

rectum in, 396. 

reflexes in, 394. 

regeneration in stage of, 403. 
duration of, 403. 

retentio alvi in, 397. 

secretions in, 397, 398. 

sensory symptoms in, 388, 389. 

sepsis in, 384. 

spermatorrhea in, 397. 

subacute forms of, 385. 

tenesmus ani in, 397. 

trophic changes in, 398. 

vasomotor symptoms in, 397. 
focal, 377. 
hemorrhagic, 375. 
history of, 371. 
lumbar, 417. 

symptoms of, 417. 
multiple, 377. 
pathology of, 372. 

ascending degeneration in, 377. 

axis cylinders in, 373. 

central canal in, 376. 

connective tissue in, 374. 

descending degeneration in, 377. 

ganglion cells in, 373. 

glia cells in, 375. 

inflammatory edema in, 376. 

Lissauer's zone in, 377. 

medullary sheaths in, 373. 

nerve fibers in, 373. 

nerve roots in, 377. 

parenchyma in, 373. 

regeneration of cells in, 375. 

sclerotic cicatrix in, 375. 

softening in, 375. 

spider cells in, 375. 



Myelitis, pathology of, Tiirck's degeneration 
in, 377. 
vascular apparatus in, 375. 
yellow softening in, 375. 
prognosis of, 432. 
prophylaxis of, 433. 
sacral, 418. 

symptoms of, 418, 419. 
syphilitic, 381. 
terminations of, 403. 
transverse, 377, 383. 
diagnosis in, 410. 
treatment of, 433. 

arrangement in bed in, 435. 
baths in, 444. 
by apparatus, 447, 448. 
by spas, 446. 
corsets in, 437. 
drugs in, 438. 
electricity in, 438. 
etiologic, 439. 
food in, 434. 

lumbar infusions in, 439. 
nursing in, 437. 
of bed-sores in, 435, 436. 
of cystitis in, 436. 
of contracture, 412. 
of incontinence of bladder in, 436. 
physico-mechanical, 439. 
specific, 434. 
surgical, 450. 
suspension in, 437. 
without softening, 372. 
X-ray in, 429. 
Myelitic softening, 114. 
Myeloencephalitis, disseminated, 826. 
Myelomalacia, 371, 372. 
Myelomeningitis, 373. 
Myoclonia, 942. 
epilepsy and, 1023. 
treatment of, 944. 
Myokymia, 177, 934, 957. 
of the facial nerve, 940. 
of the tongue, 946. 
Myopathia saturnina, 816. 
Myopathic paralysis, 824. 
Myosis, 207. 
Myospasia impulsiva, 936. 

simplex, 936. 
Myotonia, 934. 

syringomyelitica, 930. 
Myotonic disturbance, in syringomyelia, 

594. 
Myxedema, 143. 

Nails in nervous disease, 145. 
Narcolepsy, 1023, 1083. 



1188 



INDEX OF SUBJECTS 



Nerfs radiculaire, 502. 
Nerve, abducens, 25, 57. 

origin of, 25. 
' auditory, 82, 83, 90. 
origin of, 25. 
cochlear, 82. 
facial, 56. 

origin of, 25. 
fifth, 24. See also Trigeminal and Tri- 
facial nerve, 
origin of, 24. 
glossopharyngeal, 25, 90. 

paralysis of, 681. 
hypoglossal, 59. 
origin of, 25. 
paresis of, 160. 
oculomotor, 24, 51. 

origin of, 24. 
olfactory, origin of, 86. 
optic, atrophy of, 185. 
examination of, 184. 
fibers of, 99. 
origin of, 84. 
phrenic, in polyneuritis, 787. 
pneumogastric, 25, 57. See also Vagus, 
nuclei of, 81. 
origin of, 25. 
seventh. See Facial nerve, 
spinal accessory, 58. 

origin of, 25. 
trigeminal, 55, 78. 
trochlear, 24, 54. 

origin of, 24. 
ulnar, analgesia of, in tabes, 478. 
vestibular, 83, 92,514. 
Nerve fiber, peripheral, 40. 
process, description of, 38. 
tissue, 35. 

tracts, columnar, 45. 
Nerve-cell, basic substance of, 36. 
body of, 36. 
nucleus of, 35. 
size of, 35. 

staining property of, 36. 
staining of, 36. 
structure of, 35. 
Nerve-cells, 35. 

Nerves, multiple cerebral, paralysis of, 693. 
diagnosis of, 695. 
etiology of, 693. 
prognosis of, 695. 
symptoms of, 693. 
treatment of, 695. 
of special sense, 19. 
Nervi Lancisii, 9. 
Nervous system, syphilis of, 125. 
Neuralgia, 828. 



Neuralgia, autotoxic, 840. 
diagnosis of, 835. 
differential diagnosis of, 836. 
duration of, 838. 
epileptiform, 837. 
etiology of, 836. 

alcohol in, 838, 841. 

bacterial toxins in, 841. 

chlorosis in, 839. 

disease of nerve in, 841. 

disturbances of metabolism in, 838. 

infection in, 839. 

refrigeration in, 839. 

toxemia in, 840. 

trauma in, 839. 
false, 845. 
idiopathic, 845. 
in hysteria, 1071. 
intercostal, 829. 
intermittent, 830, 837. 
leukotrichia in, 834. 
masked, 840. 
of the fifth nerve, 829. 
pathology of, 842, 843. 
prognosis of, 845. 
radiation phenomena in, 838. 
relapse in, 838. 
remittent, 830. 
rheumatic, 839. 
root, 423. 
symptoms of, 828. 

abnormal sensation in, 833. 

abortive attacks in, 830. 

anesthesia dolorosa in, 833. 

herpes zoster in, 829. 

muscles in, atrophy of, 835. 

pain in, 828. 

paroxysms in, 830. 

reflex disturbances in, 830. 

secretory disturbances in, 834. 

trophic disturbances in, 834. 

Valleix's points in, 831. 

vasomotor disturbances in, 833. 
toxic, 840. 
traumatic, 839. 
treatment of, 845. 

antipyretics in, 848. 

antirheumatics in, 848. 

atropin in, 848. 

bromids in, 846. 

cautery in, 848. 

derivants in, 848. 

diet in, 846. 

electricity in, 849. 

massage in, 849. 

morphin in, 847. 

nerve tonics in, 848. 



INDEX OF SUBJECTS 



1189 



Neuralgia, treatment of, psychotherapy in, 
849. 
quinin in, 845. 
rest in, 846. 
specifics in, 846. 
surgery in, 846. 
surgical, 850. 

syphilitic remedies in, 846. 
symptomatic, 847. 
visceral, 829. 
Neuralgia anovesicalis, in tabes, 486. 
ascendens, 830. 
descendens, 830. 
paresthetica, 1158. 
Neurasthenia, definition of, 976. 
frequency of, 994. 
history of, 976. 
infantile sexual, 983. 
sexual, 976. 

etiology of, 994. 
age in, 994. 
alcohol in, 999. 
chancroids in, 995. 
congressus interruptus in, 998. 
constitutional weakness in, 995. 
education in, 999. 
gonorrhea in, 995. 
onanism in, 995. 
sex in, 994. 

sexual diseases in, 995. 
sexual excesses in, 998. 
syphilis in, 995. 
tobacco in, 999. 

urogenital organs, local disease of in, 
996. 
prophylaxis in, 999. 

early marriage in, 1001. 
educational influences in, 1000. 
sexual hygiene in, 999. 
symptoms of, 980. 

anomalies of sexual sensation in, 980. 
clitoral crises in, 985. 
emissions in, 983. 
hypospermia in, 993. 
impotence in, 988. 
irritable bladder in, 984. 
pollutions in, 982. 
priapism in, 986. 
prostatorrhea in, 986. 
reflexes in, 981. 
relative impotence in, 992. 
sensory disturbances in, 981. 
spermatorrhea in, 985. 
temporary impotence in, 992. 
vulvovaginal, crises in, 985. 
treatment of, 1002. 
aphrodisiacs in, 1008. 



Neurasthenia, sexual, treatment of, bromism 
in, 1009. 
camphor in, 1009. 
cantharides in, 1009. 
diet in, 1007. 
drugs in, 1009. 
electricity in, 1003. 
electrotherapy in, 1005. 
hydrotherapy in, 1004. 
hygiene in, 1003. 
hygiene of sleep in, 1008. 
lecithin in, 1009. 
marriage in, 1003. 
mechanotherapy in, 1006. 
organotherapy in, 1010. 
yahimbin in, 1009. 
theories of, 978. 
Neuritis, acute progressive, 795. 
amyotrophic plexus, 751. 
course of, 774. 
diagnosis of, 774. 
etiology of, 748. 
alcohol in, 754. 
anesthesia in, 756. 
autointoxication in, 760. 
chronic intoxication in, 770. 
nicotin in, 770. 
occupations in, 771. 
pathogenic agents in, 752. 
pregnancy in, 759. 
refrigeration in, 749. 
trauma in, 748. 
toxic products in, 749. 
latent, 810. 
leprous, 810. 
maternal, 757. 
occupation, 767. 
optic. See Optic neuritis, 
parenchymatous, 779. 
pathology of, 777. 

axis cylinders in, changes in, 777, 780. 

degeneration in, retrogressive, 778. 

degenerative processes in, 779. 

gangrene in, 782. 

glossy skin in, 782. 

inflammatory parenchymatous disease 

in, 780. 
muscle in, changes in, 781. 
nerve cicatrix in, 778. 
nerve degeneration in, 777. 
perforating ulcer in, 782. 
regeneration in, autogenous, 778. 
Schwann's sheaths in, 778. 
secondary Wallerian degeneration in, 

780. 
skin in, changes in, 782. 
Waller's degeneration in, 777. 



1190 



INDEX OF SUBJECTS 



Neuritis, periaxial, 815. 
peripheral, 764. 
polyneuritis and, 742. 
puerperal, 759. 
rheumatic, 749. 
segmentary, 780. 
sensory, 748. 
symptoms of, 743, 761, 771. 

acroparesthesia in, 771. 

anomalies in the secretion of sweat in, 
762. 

ape-hand in, 745. 

bed-sores in, 765. 

claw-hand in, 745. 

contractions in, 765. 

crural nerve in, 772. 

deformities in, 745. 

electric contractility in, 745. 

erythromelalgia in, 765. 

formication in, 744. 

general nervousness in, 771. 

glossy skin in, 761. 

herpes zoster in, 763. 

hyperhidrosis in, 762. 

mal perforant in, 764. 

motor phenomena in, 771. 

nerves of the leg in, 772. 

pain in, 771. 

paralysis in, 744. 

paresthesia in, 744. 

peroneal nerve in, 773. 

Raynaud's gangrene in, 765. 

reflexes in, 745. 

sensory qualities in, 747. 

subjective, 744. 

swelling of nerve in, 743. 

trophic changes in, 762. 
toxic, 503. 
treatment of, 774. 

anti-neuralgics in, 775. 

antipyrin in, 775. 

arsenic in, 775. 

baths in, 776. 

blood-letting in, 775. 

cocain in, injections of, 775. 

diaphoresis in, 775. 

drugs in, 775. 

electricity in, 775. 

fango in, 776. 

lactophenin in, 775. 

massage in, 776. 

moist heat in, 775. 

neurolysis in, 777. 

pyramidon in, 775. 

rest in, 774. 

sali pyrin in, 775 

sodium salicylate in, 775. 



Neuritis, treatment of, spas in, 776. 
strychnin in, 775. 
surgical measures in, 776. 
Tallermann apparatus in, 775. 
trigemin in, 775. 
apoplectica, 820. 
ascendens, 766. 
Neurofibromata, 144. 
Neuro-fibrils, 46. 
Neuroglia, 43. 

Neurokeratin structure of nerve fiber, 40. 
Neurolysis, 777. 

Neuromyositis, 642, 745, 785, 824. 
Neuron, first motor, 48. 
diseases of, 70. 

circumscribed, 71. 
in brain, 50. 
in spinal cord, 48. 
first sensory, 73. 
function of, 101. 
in spinal cord, 74. 
of cerebral nerve, 77. 
second motor, 60. 
origin of, 60. 
tract of, 66. 
second sensory, 86. 
sensory, diseases of, 100. 
third sensory, 97. 
Neuron diseases, 47, 70. 
systems, 47. 
theory, 34, 44, 45. 
Neuroses, traumatic. See Traumatic neu- 
roses, 
vasomotor trophic. See Vasomotor 
trophic neuroses. 
Neurotabes peripherica, 518, 791, 823. 
symptoms of, 791. 

spontaneous movements in, 791. 
Nictitatio, 943. 
Nissl's body, 36. 
Nocturnal epilepsy, 1029. 
Nodulus, 22. 
Nucleus, red, 21. 
tegmental, 22. 
Nucleus ambiguus, 57. 
amygdalae, 3. 
caudatus, 3, 15. 
cuneatus, 24. 
dentatus, 110. 
globosus, 22. 
gracilis, 24. 
lentiformis, 3, 15. 

ruber, 20, 21. See also Nucleus, red. 
Nuclei, sensory, 107. 
Nystagmus, 177. 
ataxic, 484. 
in epilepsy, 1019. 



INDEX OF SUBJECTS 



1191 



Nystagmus in polyneuritis, 788. 
of miners, 1158. 
rotatory, 564. 

Obstetrical paralysis, 724. 
Obturator nerve, paralysis of, 728. 
prognosis of, 729. 
symptoms of, 729. 
treatment of, 729. 
Oculomotor nerve, nuclei of, 52. 
Occupation, effects of, in nervous disease, 
132. 

influence of, 132. 
Occupation neuritis, 748. 
Occupation neuroses, 1149. 

pathogenesis of, 1159. 

treatment of, 1159. 
Occupation paralysis, 716, 769, 773. 
Oedemebleu, 1113. 

rouge, 1075. 
Olive, lower, 23. 

upper, 90. 
Oligospermia, 993. 
Oligozoospermia, 993. 
Oliguria in hysteria, 1076. 
Onanism, in sexual neurasthenia, 980. 
Onychophagy, 949. 
Ophthalmia neuroparalytica, 680. 
Ophthalmoplegia, progressive, 71. 
Ophthalmoplegia externa, 488, 641. 

hysterical, 1053. 
Ophthalmoplegia interna, 488. 

in polyneuritis, 788. 
Oppenheim's phenomenon in epilepsy, 1018. 

sign in hysteria, 1074. 
Operculum, 8. 
Opisthotonos, 933. 

in syringomyelia, 593. 
Optic atrophy in polyneuritis, 792. 

chiasm, 24. 
Optic neuritis, 184. 

in polyneuritis, 792, 806. 
Optic radiation of Gratiolet, 16. 
Optic thalamus, 19. 

nuclei of, 19. 
Organ, parietal. 20. 
Organs of speech spasm of, 339. 
Osteomalacia, 636. 
Ovarie, 938. 
Oxyekoia, 663. 

Pachymeningitis, hypertrophic, 653. 
Pachymeningitis interna, 867. 
Pain, delayed, 194. 

hallucinations, 1073, 1078. 
Palate, movements of, 159, 160. 

paralysis of, 688, 805. 
76 



Palate, spasm of, 948. 
Pallium, 2, 3, 4. 
Palsy, shaking, 934. 
Paper sorter's neurosis, 1158. 
Paralgia, 1072. 

Paralysis of nerves of arm and shoulder, 718. 
clinical history of, 719. 
diagnosis of, 723. 
etiology of, 720. 
prognosis of, 723. 
treatment of, 724. 
Paralysis of the peripheral nerves, 662. 
Paralysis, acute bulbar, 118. 

amyotrophic progressive bulbar, 656. 
atrophic spastic bulbospinal, 72. 
dissociated sensory, in syringomyelia, 

596. 
drummer's, 769. 
facial syphilitic, in tabes, 488. 
flaccid, cause of, 60. 
hystero-traumatic, 723. 
ischemic muscular, 765. 
lead, 799. 

acute onset in, 801. 
bulbar nerves in, 801. 
claw-foot in, 801. 
face, pallor of, 800. 
pain in, 800. 
radial nerve in, 800. 
sensor}' disturbance in, 802. 
tremor in, 176, 800. 
wrist-drop in. 800. 
obstetric, 758. 

of multiple cerebral nerves, 693. 
of muscles of larynx, in syringomyelia, 

595. 
of muscles of palate, in syringomyelia, 

595. 
progressive, 493. 
spastic, treatment of, 445. 
spinal, due to syphilis, 126. 
subacute ataxic, 624. 
Paralysis agitans, 880. 
cause of, 889. 
definition of, 881. 
diagnosis of, 893. 
differential diagnosis of, 893. 
from hysterical tremor, 894. 
from multiple sclerosis, 893 
from neurasthenic tremor, 893. 
from tremor of Graves' disease, 893. 
etiology of, 881. 
age in, 883. 
alcoholism in, 883. 
heredity in, 882. 
over-exertion in, 884. 
predisposition in, 884. 



1192 



INDEX OF SUBJECTS 



Paralysis agitans, etiology of, refrigeration 
in, 883. 
sex in, 883. 
syphilis in, 883. 
trauma in, 883. 
history of, 880. 
pathology of, 889. 
prognosis of, 895. 
symptoms of, 884. 

cutaneous sensibility in, 887. 
irregular types of, 888. 
mental condition in, 887. 
muscular rigidity in, 884, 886. 
propulsion in, 886. 
retropulsion in, 886. 
skin in, changes in, 887. 
spastic gait in, 886. 
tremor in, 884, 885. 
vasomotor disturbance in, 886. 
synonyms of, 880. 
treatment of, 895. 
arsenic in, 895. 
diet in, 895. 
duboisin in, 897. 
electricity in, 896. 
hydrotherapy in, 896. 
hyoscin in, 897. 
organotherapy in, 897. 
sodium cacodylate in, 896. 
surgical measures in, 897. 
Paralysis saturnina, 710. 
Paragraphia, 302. 
Paramyoclonus, 936. 
Paramyotonia, 931. 
Paraphasia, 269. 
Paraplegia cervicalis, 786. 
dolorosa, 389, 390. 
in leukemia, 428. 
in pernicious anemia, 428. 
in the climacterium, 428. 
Parasite, hyperphysiologic, 1027. 
Paresis in epilepsy, 1019. 
Parietal organ, 20. 

Parkinson's disease. See Paralysis agitans. 
Paroxysmal hemoglobinuria in Raynaud's 

disease, 1135. 
Pars frontalis, 17. 
occipitalis, 17. 
Pavor nocturnus, 1029. 
Pectoralis major, atrophy of, 146. 
Pederastia, 981. 
Peduncle, anterior, 20. 

posterior, 20. 
Peduncles, anterior cerebellar, 110. 
cerebellar, 110. 
superior, 20, 21. 
Perimeter, use of, 185. 



Periostitis traumatica, 867. 
Peroneal nerve, paralysis of, 732, 
cerebral paralysis in, 735. 
in progressive neurotic muscular atrophy, 

646. 
paralysis of, symptoms of, 733. 
Petit mal, 1022. 

symptoms of, 1022. 
Photophobia, 1109. 
Phrenic nerve, paralysis of, 695. 
Piano player's cramp, 1154. 

symptoms of, 1 156. 
Pigment in nervous disease, 143. 

of skin, decrease of, in paralysis, 143. 
Pigmentation in scleroderma, 1141. 
Piltz's sign, 209. 
Plates, medullary, 1. 
Plexus, choroid, 3, 22. 
Plexus choroidea, 22. 
Plexus neuritis, amyotrophic, 723. 
Plexus paralysis, 720. 
Pli courbe, 8. 

Pneumogastric nerve, origin of, 57. 
paralysis of, 683. 
diagnosis of, 685. 
etiology of, 683. 
prognosis of, 686. 
symptoms of, 683. 
treatment of, 686. 
Pneumonia, deglutition, in polyneuritis, 805 

lumbar puncture in, 251. 
Points, hysterogenic, 1082. 

hysterogenic, 1082. 
Points douloureaux, 830. 
Pole, frontal, 8. 

occipital, 8. 
Poliomyelitis, 378. 
acute, 117,590,723. 
histology of, 117. 
anterior, 815. 
chronic, 119. 
chronic anterior, 654. 
cause of, 655. 

differential diagnosis of, 655. 
from lead paralysis, 655. 
from polyneuritis, 655. 
etiology of, 655. 
pathology of, 655. 
symptoms of, 655. 
treatment of, 656. 
Polioencephalitis, superior, 788. 

hemorrhagic, 815. 
Pollutions, 212. 
diurnal, 983. 
pathologic, 982. 
Polymyositis, 642, 809. 
Polyneuritis, 782. 



INDEX OF SUBJECTS 



1193 



Polyneuritis, alcoholic, 785. 
amyotrophic, 791, 810. 
arsenical, 797. 

alopecia in, 799. 

clinical history of, 797. 

erythromelalgia in, 799. 

keratoses in, 799. 

nails, changes in, 799. 

pigmentation in, 799. 

prognosis of, 799. 

symptoms of, 798, 799. 
autointoxication in, 811. 
cancerous cachexia in, 812. 
clinical history of, 783. 
course of, 796. 
differential diagnosis of, 820. 

from poliomyelitis, 820. 

from tabes, 823. 
diphtheritic, 804. 

accommodation in, 805. 

apnea in, 807. 

ataxia in, 805, 806. 

bradycardia in, 805. 

clinical history of, 804. 

course of, 810. 

duration of, 808. 

esophagus in, 805. 

eye in, paralysis of muscles of, 806. 

heart in, 805, 809. 

optic neuritis in, 806. 

paralysis in, 807. 

paralysis of palate in, 805. 

pathology of, 816. 

pharynx in, muscles of, 805. 

pneumonia in, 805. 

prognosis of, 808. 

sensory disturbances in, 806. 

symptoms of, 805. 

tachycardia in, 805. 

tendon reflexes in, 806. 

treatment of, 808. 

vocal cords in, paralysis of, 805. 
dropsical, 793. 
endemic, 793. 
etiology of, age in, 784. 
hyperhidrosis, 793. 
infectious diseases in, 810. 
leprous, pathology of, 818. 
mercurial, 802. 
pathology of, 813. 

atrophic-parenchymatous processes in, 
813. 

atrophy in nerve fiber in, 813. 

bacilli in, 819. 

connective tissue in, 814. 

muscles in, 814. 

posterior columnus in, 814. 



Polyneuritis, pathology of , spinal cord in, 814. 

reflex pupillary rigidity in, 788. 
saturnine, 801. 
senile, 812. 

symptoms of, 783. 

acuity of vision in, 792. 

albuminuria in, 785. 

anesthesia dolorosa in, 790. 

arthritis in, 793. 

ataxia in, 791. 

atrophy in, 789. 

auditory nerve in, 792. 

bladder disturbance in, 792. 

bradycardia in, 787. 

cerebral nerves in, 787. 

chronic development of, 784. 

cyanosis in, 793. 

defective memory in, 794. 

delirium tremens in, 794. 

diaphragmatic respiration in, 787. 

difficulty in deglutition in, 787. 

edema in, 789, 793. 

electric contractility in, 789. 

erythema in, 793. 

facial paralysis in, 788. 

gastro-intestinal phenomena in, 785. 

glance paralysis in, 788. 

granulation tumors in, 793. 

hypalgesia in, 790. 

hyperesthesia in, 785, 790. 

inequality of pupils in, 788. 

intermittent claudication in, 786. 

Korsakow's syndrome in, 794. 

mental disturbance in, 794. 

muscles in, 785, 789. 

nerve in swelling of, 785. 

nystagmus in, 788. 

ocular muscles in, 787. 

optic nerve in, 792. 

optic neuritis in, 792. 

pain in, 784. 

paralysis in, 786. 

paresthesia in, 785. 

perforating ulcer of the foot in, 793. 

phrenic nerve in, 787. 

position in, sensation of, 791. 

pulse in, 787. 

scotoma in, 792. 

secretory disturbance in, 793. 

sensation in double, 791. 

spleen in enlargement of, 785. 

steppage gait in, 786. 

tendon reflexes in, 789. 

trophic disturbance in, 793. 

urobilinuria in, 785. 

vasomotor disturbance in, 793. 
syphilitic, 802. 



1194 



INDEX OF SUBJECTS 



Polyneuritis, toxic, 802. 
treatment of, 826. 
diaphoresis in, 827. 
diet in, 827. 
prophylaxis in, 826. 
rest* in, 826. 
spas in, 827. 

tuberculous, pathology of, 819. 
Polyclonia, 936. 
Polymia in Graves' disease, 961. 

in hysteria, 1069, 1076. 
• in tabes, 490. 

in tetany, 917. 
Pons, nerve-cells of, 67. 
Posterior cutaneous femoral nerve, paralysis 

of, 730. 
Postscapular nerve, paralysis of, 698. 
Potentia cceundi, 988. 

generandi, 988. 
Preacher's hand in syringomyelia, 594. 
Precuneus, 8, 10. 
Pregnancy, influence of, in Graves' disease, 

974. 
Prejuvenile sexual neurasthenia, 983. 
Present condition in the history, 135. 
Pressure, intracranial, 231. 
Priapism, 212, 986. 
Process, metasyphilitic, 219. 
Professional paralysis, 716. 
Projection system, 45, 267. 
Progressive muscular atrophy, anomalies of 
skull in, 639. 
atrophy of bones in, 638. 
bulbar type of, 640. 
clinical description of, 634. 
complications in, 638. 
differential diagnosis of, 642. 
from diseases of the joints, 342. 
from neuromyositis, 642. 
from polymyositis, 642. 
etiology of, 642. 
alcohol in, 643. 
exertion in, 643. 
heredity in, 642. 
syphilis in, 643. 
trauma in, 643. 
facio-scapulo-humeral form of, 640. 
infantile form of, 639. 
infantile hereditary form, symptoms of, 

649. 
ophthalmoplegia externa in, 641. 
pathogenesis of, 642, 644. 
pathology of, 635. 
scapulo-humeral form of, 640. 
symptoms of, 636. 

curvature of spine in, 636. 
fibrillary contractions in, 642. 



Progressive muscular atrophy, symptoms 
of, gait in, 637. 
muscles of face in, 639. 
muscular movements in, 636. 
pain in, 636. 

pseudohypertrophy of muscles in, 637. 
reactions of degeneration in, 641. 
respiratory muscles in, 63S. 
shoulder girdle, muscles of, 640. 
symmetry of muscular change in, 638. 
tapir lip in, 639. 
treatment of, 645. 
baths in, 646. 
Bier's stasis in, 646. 
exercise in, 645. 
massage in, 646. 
orthopedic measures in, 646. 
Progressive muscular dystrophy, 633. 
Progressive neurotic muscular atrophy, 646. 
course of, 648. 

differential diagnosis of, from polyneuri- 
tis, 648. 
etiology of, 649. 
pathology of, 648. 
prognosis of, 649. 
symptoms of, 646. 
atrophy in, 647. 
electric examination in, 647. 
face in, 647. 
muscle sense in, 647. 
pain in, 647. 
peroneal nerve in, 646. 
reflexes in, 647. 
sensation in, 647. 
tibial nerve in, 646. 
tremor in, 647. 

vasomotor phenomena in, 648. 
treatment of, 649. 
Progressive paralysis, juvenile, 576. 
Progressive spinal amyotrophy, 652. 
differential diagnosis of, 653. 

from arthrogenous muscular atrophies, 

653. 
from disease of the vertebrae, 653. 
from hypertrophic pachymeningitis, 

653. 
from neuritis, 653. 
from syringomyelia, 653. 
from tumors of the meninges, 653. 
prognosis of, 653. 
symptoms of, 652. 
treatment of, 653. 
Progressive spinal muscular atrophy, infan- 
tile hereditary form of, course of, 
650. 
diagnosis of, 651. 
pathology of, 650. 



INDEX OF SUBJECTS 



1195 



Progressive spinal muscular atrophy, infan- 
tile hereditary form of, prognosis 
of, 651. 
treatment of, 651. 

with bulbar symptoms, 649. 

without bulbar symptoms, 649. 
Prosoplegia, 662. 
Prostatorrhea, 986. 
Protylin, 972. 
Pruritus, hysterical, 1072. 
Pseudohypertrophy of muscles, 147. 
Pseudo-Jacksonian epilepsy, 1020. 
Pseudoneuralgia in hysteria, 1071. 
Pseudosclerosis, 575. 
Pseudotabes, 531. 

alcoholica, 531. 

arsenicosa, 799. 

diabetica, 812. 
Psychosis, Korsakow's, 794, 795. 

neuritic, 794. 
Psychrophore, 1005. 
Psychalgias, 1070. 

Psychical condition, examination of, 167. 
Psychical disturbance in Graves' disease, 
961. 

in tabes, 492. 
Psychopathic constitution, 1077. 
Ptosis, 151, 158. 

spastica pseudoparalytica, 944. 
Pudendic plexus paralysis, 736. 

differential diagnosis of, 738. 

etiology of, 736. 

prognosis of, 739. 

symptoms of, 737. 

treatment of, 740. 
Pulvinar, 19, 86. 

Pupillary rigidity in polyneuritis, 788. 
Pupils, inequality of, in polyneuritis, 788. 

reaction of, in epilepsy, 182. 
in hysteria, 182. 
method of testing, 208. 

rigidity of, to light, 208. 
Purkinje's cells, 38, 110, 619. 
Purpura, 1145. 
Put amen, 16. 
Pyramidal tract, disease of, 410. 

Quincke's circumscribed edema, 1075. 
disease, 1144. 

Ischialgia, 1089. 
Radial paralysis, 708. 

clinical picture of, 708. 

diagnosis of, 711. 

etiology of, 709. 

prognosis of, 711. 

treatment of, 711. 



Radiation phenomena in neuralgia, 838. 
Radix mesencephalica, 80. 
Ranvier, segments of, 778. 
Ranvier's nodes, 42. 
Raynaud's disease, 599, 1132. 

clinical description of, 1133. 

diagnosis of, 1137. 

differential diagnosis of, 1138. 

disease of the central nervous system and 
1136. 

etiology of, 1134. 

pathology of, 1137. 

symptoms of, 1134. 
gangrene in, 1134. 
paroxysmal hemoglobinuria in, 1135. 

treatment of, 1138. 
Reaction, sympathetic pupillary, 207. 
Recurrent laryngeal nerve, paralysis of, in 

syringomyelia, 595. 
Reflex, abdominal, 203. 

cremaster, 203. 

cutaneous, 155, 202. 

eyelid, 204. 

internal, 206. 

jaw, 197. 

palate, 204. 

patella tendon, 197. 

pupillary. 207. 

retching, 204. 

sexual, 211. 

sneezing, 204. 

tendon, 155. 

tendo Achillis, 199. 

to... 201. 

pathologic, 203. 

triceps, 197. 
Reflex arcs, genital, 991. 

collaterals, 816. 

epilepsy, 1030, 1035. 
Reflexes, complicated, 514. 

examination of, 196. 

method of eliciting, 155. 

spinal localization of, 409. 

tendon, 196. 
Regeneration, autogenous, 778. 
Region, subthalamic, 20. 
Reil, island of, 4, 10, 268. 
Resistance reflex in hysteria, 1054. 
Retinitis albuminurica, 184. 
Rheumatic nodules, 867. 
Rhinencephalon, 13. 
Rhinorrhea, 1076. 

Rhizitis posteiior, treatment of, 438. 
Rhodagan, 972. 

Rhomboid fossa. See Fourth ventricle. 
Rhomboideus, atrophy of, 146. 
Rinne'stest, 189. 



1196 



INDEX OF SUBJECTS 



Rhine's test in hysteria, 1069. 
Rods and cones, 84. 
Romberg's sign, 170, 476. 

various forms of, 171. 
Root, ascending trigeminal, 79. 
Roots, anterior, origin of, 48. 
Rosenbach's reflex, 725. 
Rostrum, 16. 
Rumpf'ssign, 1115. 

Sacral cord, 28. 
Sacralgia, 1071. 
Sadism, 981. 
Salaam spasm, 950. 
Saliva in epilepsy, 1024. 

in nervous disease, 142. 
Saltatory reflex spasm, 959. 
Scalp, hairy, hyperesthesia of, 863. 
Scarlatina, lumbar puncture in, 251. 
School nervousness, 983. 
Schultze's degeneration, 122. 
Schwann's sheath, 42, 778. 
Sciatic nerve, paralysis of, 731. 
Sciatic phenomenon, 785. 
Sciatica, 1071. 

diabetes the cause of, 838. 
Sclerodactylia, 599, 1135. 
Scleroderma, 143, 599, 1141. 
cause of, 1143. 
diagnosis of, 1143. 
etiology of, 1141. 
symptoms of, 1141. 

pigmentation in, 1141. 
treatment of, 1143. 
thiosinamin in, 1144. 
Sclerosis, 371. 

amyotrophic lateral, 72, 570, 593, 651, 
656. 
differential diagnosis of, 659. 

from acute folioencephalitis, 660. 
from asthenic bulbar paralysis, 660. 
from chronic myelitis, 660. 
from syringobulbia, 660. 
from syringomyelia, 659. 
duration of, 658. 
etiology of, 659. 
fibrillary contractions in, 657. 
pathology of, 658. 
prognosis of, 658. 
symptoms of, 656. 
atrophy in, 657. 
bulbar signs in, 657. 
electric examination in, 657. 
facial nerve in, 657. 
fifth nerve in, 658. 
heart in, 658. 
intelligence in, 658, 



Sclerosis, amyotrophic lateral, symptoms of, 
saliva in, 658. 
tongue in, 657. 

vocal cords in paralysis of, 658. 
treatment of, 661. 

diffuse, 576. 

multiple, 120. 
Scotoma, 792. 

in migraine, 875. 
Seamstress's spasm, 1158. 
Secretory disturbances in hysteria, 1076. 

in neuralgia, 834. 
Segment, innervation of muscles, 354. 
Sensation, vibratory, 196. 
Sense of hearing, examination of, 189. 

of locality, 193. 

of smell, examination of, 187. 

of taste, examination of, 188. 

of temperature, 193. 

stereognostic, 195. 
Sensory functions, examination of, 183. 

speech center, 268, 270. 

sphere, 267. 
Septum pellucidum, 16. 
Serratus, paralysis of, 701, 772. 
diagnosis of, 703. 
prognosis of, 704. 
symptoms of, 702. 
treatment of, 704. 
Serratus anticus, atrophy of, 147. 
Sero-diagnosis, 220. 
Sexual frigidity, 212. 

inversion, 981. 

neurasthenia, 976. 

psychopathy, 981. 

relations, effects of, in nervous disease, 
134. 

influence of, 134. 
Sheaths, medullary, destruction of, in my- 
elitis, 115. 
Shock-hysteria, 1050. 
Sialorrhea, 1076. 
Sight, cortical center of, 8. 
Sigmatism, 345. 

Simulation in traumatic neurosis, 1118, 1119. 
Singultus, 956. 

in epilepsy, 1014. 

in hysteria, 1060. 
Sinus thrombosis, 250. 
Skin, color of, 141. 

sensory conditions of, 190, 191. 

temperature of, 141. 
Skull, auscultation of, 217. 

exostosis of, 215. 

murmurs in, 217. 

osteoporosis of, 215. 

percussion of, 216. 



INDEX OF SUBJECTS 



1197 



Skull, sensitiveness of, 216. 

vertebral column and, in diagnosis, 137. 
Sleep wandering, 1083. 
Sleeping attacks in hysteria, 1083. 
Smell, center for, 13. 
Snuffling tic, 943. 
Somnambulism, 1083. 
Spore, subdural, 224. 
Spores, subarachnoid, 224. 
Spasm, 156. 

articulatory, 948. 
auctioneer's, 945. 
clonic, 934. 
cortical facial, 945. 
greeting, 950. 
intention, 934. 
in tetanus, 157. 
localized, 933. 

clinical varieties of, 940. 
course of, 939. 

differentiation of forms of, 934. 
etiology of, 938. 
symptoms of, 936. 

cramps in the calves in, 937. 
electric reactions in, 938. 
hypertrophy of muscles in, 937. 
pain in, 937. 
paralysis in, 937. 
reflex irritability in, 938. 
sensory disturbances in, 938. 
tendon reflexes in, 938. 
treatment of, 939. 
drugs in, 939. 
electricity in, 939. 
iodin in, 939. 

psychomotor education in, 940. 
surgery in, 939. 
milker's, 938. 
muscular, 153. 
nodding, 950. 
occupation, 934, 945. 
of abdominal muscles, 956. 
of diaphragm, 956. 
of muscles of chest, 956. 
of. muscles of face, 940. 
of muscles of lower extremity, 957. 
of muscles of mastication, 948, 949. 
of muscles of neck, 949. 
of muscles of trunk, 956. 
of tongue, 946. 
reading, 945. 
salaam, 950. 
saltatory, 959. 
tonic, 934. 
Spasm center, 1025. 
Spasms in traumatic neurosis, 1114. 
tetanic, 934. 



Spasms, tonic, in syringomyelia, 593. 
Spasmophilia, 1043. 
Spastic paralysis, 387. 
Spastic spinal paralysis, 627. 
diagnosis of, 629. 
differential diagnosis of, 629. 

from amyotrophic lateral sclerosis, 629. 
from caries of vertebne, 629. 
from chronic myelitis, 630. 
from compression of the cord, 629. 
etiology of, 627. 
pathology of, 628. 
symptoms of, 628. 
treatment of, 632. 
Speech, centers of, 12. 
disturbances of, 162. 
central, 333. 
paralytic, 312. 
pathology of, 284. 
peripheral impressive, 325. 
phonetic, 265. 
internal, 270. 

peripheral expressive disturbances of, 343. 
scanning, 163, 568. 
Speech center, 61, 266, 309. 
function, motor, 13. 
region, anatomical position of, 312. 
Spermatorrhea, 985. 
Splenium, 9, 16. 

Spinal accessory nerve, paralysis of, 687. 
etiology of, 687. 
prognosis of, 690. 
symptoms of, 687. 
treatment of, 690. 
Spinal ataxia, acute, 824. 
Spinal cord, 28. 

angioma of, 347, 349. 

carcinoma of, 347. 

cholesteatoma of, 347. 

cervical enlargement of, 28. 

cysts of, 347. 

exostoses of, 349. 

extramedullary tumors of, 347. 

fibroma of, 347. 

gray degeneration of. See Tabes dor- 

salis. 
gray substance of, 28, 42. 
glioma of, 347. 
gliosarcomata of, 347. 
gumma of, 347. 

intramedullary tumors of, 347. 
length of, 28. 

lumbar enlargement of, 28. 
meninges of, cysticerci of, 348. 
echinococci of, 348. 
endothelioma of, 348. 
fibroma of, 348. 



1198 



INDEX OF SUBJECTS 



Spinal cord, meninges of, myxoma of, 348. 
psammoma of, 348. 
sarcoma of, 348. 
tumors of, 348. 
metastatic tumors of, 347. 
myeloma of, 349. 
neoplasms of, 347. 
composition of, 368. 
differential diagnosis of, 350. 

tumor of lumbar enlargement and 

tumors of the cauda, 362. 
from disease of vertebrae, 352. 
from meningomyelitis, 351. 
from multiple sclerosis, 351. 
from myelitis, 351. 
from pachymeningitis, 351. 
from syringomyelia, 352. 
extension of, 353. 
pain in, 349. 
seat of, 352. 
symptoms of, 348, 349. 
tendon reflexes in, 360. 
treatment of, 369. 
varieties of, 347. 
neurofibroma of, 347. 
neurosis of, 1 107. 
osteoma of, 349. 
sarcoma of, 347. 
secondary descending degeneration of, 

122. 
structure of, 28. 
tubercle of, 347. 
tumors of, 347. 

tumors of, intramedullary, 351. 
vascular supply of, 30. 
vesicular substance of, 42. 
Spinal ganglia, cells of, 73. 
Spinal puncture. See Lumbar puncture. 
Spindle cells, 43. 
Spondylitis, tuberculous, 430. 

treatment of, 437. 
Stadium melancholicum, 1014. 
Stammering, 333. 
adenoids in, 336. 
etiology of, 334. 
heredity in, 334. 
symptoms of, 337. 

psychical, 340. 
treatment of, 342. 
Stasis papillae, 184. 
Status epilepticus, 1029. 

praesens, 133. 
Stell wag's sign in Graves' disease, 961. 
Sternocleidomastoid, atrophy of, 146. 
Stigmata of degeneration, 136. 
Stokes- Adams disease, 1035. 
Strabismus, 151, 158. 



Strangury, 987. 

in neurasthenia, 981. 
Stratum zonale, 86. 
Stria terminalis, 15. 
Striae acousticae, 84, 91. 
Structure, neurokeratin, of axis cylinder, 40. 
Stupor, epileptic, 1021. 
Stuttering, as a symptom, 162. 

in hysteria, 1060. 
Subscapular nerve, paralysis of, 700. 
Substantia gelatinosa, 24. 

nigra Soemmeringi, 20. 

perforata anterior, 24, 96. 

reticularis, 92. 
Sucking tic, 943. 
Sulcus corporis callosi, 9. 

cruciatus orbitalis, 8. 

frontalis, 8. 

interparietalis, 8. 

occipitalis, 8. 

olfactorius, 8. 

paracentralis, 10. 

pra>centralis, 7. 

retrocentralis, 8. 
Suprascapular nerve, paralysis of, 698. 
diagnosis of, 699. 
etiology of, 698. 
symptoms of, 698. 
treatment of, 699. 
Suppuration in syringomyelia, 600. 
Surdo-mutitas, 325. 
Sweat in nervous disease, 142. 

secretion of, significance of, 142. 
Sydenham's edema, 1075. 
Syllable stumbling, 163, 312. 
Sylvian fossa, 4. 
Sylvius, aqueduct of, 3, 21. 
Symmetrical gangrene, 1132. See also Ray- 
naud's disease. 
Symptoms, involuntary motor, 176. 
Syphilis, effects of, in nervous disease, 133. 

fourth stage of, 504. 

influence of, 133. 

lumbar puncture in, 251. 
Syphilophobia in neurasthenia, 995. 
Syphilosis, 503. 
Syringobulbia, 591, 596. 
Syringomyelia, 582. 

apoplectiform attacks in, 605. 

bulbomedullary form of, 593. 

cervical form of, 592. 

course of, 605, 625. 

description of a case of, 582. 

diagnosis of, 607. 

differential diagnosis of, 607, 623. 

from amyotrophic lateral sclerosis, 607. 
from cerebral diseases, 610. 



INDEX OF SUBJECTS 



1199 



Syringomyelia, differential diagnosis of, 
from dystrophia muscularis, 607. 

from family spastic spinal paralysis, 
624. 

from heredo-ataxie cerebelleuse, 624. 

from Huntingdon's chorea, 624. 

from hysteria, 607. 

from meningomyelitis, syphilitic, 608. 

from multiple sclerosis, 608, 624. 

from myelitis, chronic, 608. 

from pachymeningitis, 608. 

from paralysis of brachial plexus, 609. 

from paralysis, subacute ataxic, 624. 

from poliomyelitis, 607. 

from polyneuritis, 609. 

from Raynaud's disease, 610. 

from sclerodactylia, 610. 

from spastic paralysis, 608. 

from tabes dorsalis, 608. 

from tumor of the cerebellum, 624. 

from tumors, extramedullary, 608. 
etiology of, 606, 623. 

acute infectious diseases in, 623. 

consanguinity of parents in, 623. 

heredity in, 623. 

histology of, 615. 
occurrence of, 606. 
osteo-arthritic form of, 593. 
pathogenesis of, 620. 
pathology of, 587, 615. 

arteriosclerosis in, 590. 

congenital anomalies in, 590. 

cord in, cavities in, 588. 
condition of, 588. 
hypophasia of, 616. 

ganglion cells in, 591. 

gliomata in, 590. 

hypophasia in, 616. 

peripheral nerves in, 616. 

Purkinje's cells in, 619. 

pyramidal lateral column tracts in, 
616. 

spinal cord in, 616. 

spinal ganglia in, 616. 

Stcherbak's view in, 618. 

vessels in, condition of, 589. 
prognosis of, 605, 625. 
symptomatology of, 592. 

ape's hand in, 594. 

arthritis in, 600, 601. 

Babinski's reflex in, 614. 

bladder disturbance in, 604. 

bones in, fractions of, 602. 

bradycardia in, 595. 

bradylalia in, 614. 

cerebral derangement in, 605. 

cerebral nerves in, 604. 



Syringomyelia, symptomatology of, choreic 
contractions in, 593. 

claw-hand in, 594. 

diabetes insipidus in, 605. 

dyspnea in, 595. 

eczema in, 600. 

edema in, 600. 

electric reactions in, 595. 

fibrillary contractions in, 593, 595. 

fissures in, 600. 

gangrene in, 601. 

gastrointestinal functions in, 604. 

glossy skin in, 599. 

headache in, 605. 

kyphoscoliosis in, 603. 

laryngospasm in, 593. 

mal perforant in, 601. 

mental faculties in, 615. 

motor disturbance in, 593. 

mucous membranes in, 598. 

muscular atrophy in, 594. 

necrosis in, 601. 
•spontaneous in, 603. 

opisthotonos in, 593. 

pain in, 596. 

paralysis in, dissociated sensory, 596. 

paralysis of bulbar nerves in, 595. 
of facial nerve in, 595. 
of muscles of palate and larynx, 595. 

preacher's hand in, 594. 

pressure sense in, 599. 

psychical disturbance in, 605. 

recurrent laryngeal nerve, paralysis of, 
595. 

scoliosis in, 603. 

sensory disturbances in, 596. 

sexual functions in, 604. 

spasms in, clonic, 593. 

speech alterations of, in. 614. 

stereognostic sense in, 599. 

suppuration in, 600. 

sweat in, secretion of, 600. 

tachycardia in, 595. 

temperature sense in, 598. 

tonic spasm in, 593. 

tongue in, hemiatrophy of, 595. 

tremor in, 593. 

trophic disturbances in, 599. 

urticaria in, 600. 

vertigo in, 605. 

vomiting in, 605. 

whitlows in, 600. 

zones in, hypalgesic, 597. 
treatment of, 611, 625. 
types of, 592. 
System, ventricular, 3. 
System disease, combined, 617. 



1200 



INDEX OF SUBJECTS 



Tabes, abortive, 526. 
ergotin in, 530. 
foot in, 491. 
monoradicular, 498. 
peripheral, 531. 
pseudo, 531. 
recovery from, 525. 
Tabes amanrotica, 523. 
atactica, 523. 
bulbaris, 524. 
cervicalis, 524. 
dolorosa, 472, 523. 
Tabes dorsalis, 100, 451. 
ataxia in, cause of, 513. 
benign form of, 522. 

combination of, with progressive paraly- 
sis, 532. 
course of, 521. 
cranial nerves in, disturbance of, cause of, 

511. 
cytodiagnosis in, 529. 
diagnosis of, 527. 
differential diagnosis of, 529. 

from amyotrophic lateral sclerosis, 530. 

from ataxic paraplegia, 531. 

from cerebellar disease, 531. 

from combined system disease, 531. 

from diabetes mellitus, 531. 

from disease of the brain, 531. 

from Friedreich's disease, 531. 

from functional neuroses, 529. 

from hypochondriasis, 529. 

from hysteria, 530. 

from leprosy, 531. 

from multiple sclerosis, 530. 

from myelitis, transverse, 530. 

from neurasthenia, 529. 

from neuritis, 531. 

from spinal amyotrophies, 530. 

from spinal cord tumors, 530. 

from spastic spinal paralysis, 530. 

from syringomyelia, 530. 
disturbance of cutaneous sensibility in, 

cause of, 509. 
early diagnosis of, 527. 
etiology of, 454. 

age in, 455. 

climate in, 455. 

exertion in, 467. 

gonorrhea in, 469. 

heredity in, 465. 

infections in, 468. 

influence of race in, 464. 

mental exertion in, 467. 

neurotic predisposition in, 466. 

poisons in, 468. 

refrigeration in, 466. 



Tabes dorsalis, etiology of, sex in, 455. 
sexual excesses in, 466. 
statistics of, 456, 457. 
syphilis in, 455, 456. 
trauma in, 467. 
family type of, 461. 
herpes zoster in, cause of, 510. 
history of, 451. 
infantile, 460. 
in virgins, 461. 
juvenile, 460. 
malignant form of, 523. 
motor disturbance in, cause of, 510. 
muscle sense in disturbance of, cause of, 

509. 
pain in, lancinating, cause of, 509. 
pathogenesis of different symptoms of, 

508. 
pathologic anatomy of, 494. 
aneurysm in, 506. 
arthropathy in, tabetic, 506. 
brain in, 499. 
cerebellum in, 500. 
cervical cord in, 497. 
cranial nerves in, degeneration of nuclei, 

499. 
cytodiagnosis in, 505. 
fractures in, spontaneous, 506. 
gray degeneration of posterior columns 

in, 495. 
histological details of, 496. 
lumbar cord in, 496. 
lymphangitis in, syphilitic, 503. 
lymphocytosis in, 502. 
meninges in, 495. 
lesions of, 498. 
syphilosis of, 503. 
meningitis in, syphilitic, 502. 
nerfs radiculaires in, 502. 
nerve in, optic, atrophy of, 499. 
nerves in, motor, 499. 

peripheral. 499. 
neuritis in, toxic, 503. 
posterior columns in, 495. 

roots in, 495. 
root fibers in, posterior, 497. 
root neuritis in, 502. 
root tracts in, segmentary disease of, 

500. 
sclerosis of posterior columns in, 495. 
spinal ganglia in, 499. 
spinal cord in, lesions of, 498. 

microscopic examination of, 495, 
sympathetic nerve in, 499. 
thoracic cord in, 496. 
pathologic physiology of, 508. 
prognosis of, 532. 



INDEX OF SUBJECTS 



1201 



Tabes dorsalis, pupillary rigidity in, cause 
of, 511. 
reflex disturbance in, cause of, 509. 
sexual disturbance in, cause of, 510. 
symptomatology of, 471. 
abdominal reflexes in, 480. 
absence of sensation of fatigue in, 479. 
Achilles tendon in, 476. 
acuity of vision in, decrease in, 480. 
alopecia in, 492. 

alveolar processes in, atrophy of, 491. 
amaurosis in, 480. 
amblyopia in, 481. 
analgesia in, 477. 
angina pectoris in, 487. 
apoplectiform attacks in, 490. 
arthropathies in, 490. 
astereognosis in, 478. 
ataxia in, 482. 

of muscles of face in, 484. 

of muscles of trunk in, 484. 

nystagmus in, 484. 
ataxic stage in, 482. 
atrophy in, 487. 
bulbar paralysis in, 488, 489. 
cardiac crises in, 487. 
clitoral crises in, 487. 
cold in, hyperesthesia to, 478. 
color sense in, loss of, 480. 
cough in, spasmodic, 487. 
cremaster reflex in, 480. 
cutaneous reflexes in, 479. 
disorders of hearing in, 489. 
electric changes in contractility in, 481. 
epileptiform attacks in, 490. 
farado-cutaneous sensibility in, 477. 
fatigue in, 474. 
feminine pollutions in, 487. 
field of vision in, limitation of, 480. 
gait in, ataxic, 483. 

uncertainty of, 473. 
gastric crises in, 485. 

juice in, examination of, 486. 

pain in, 485. 
girdle sensation in, 473, 478. 
glycosuria in, 490. 
heart in, valvular lesions of, 492. 
herpes zoster in, 492. 
hypalgesia in, 477. 
hyperesthesia in, 478. 
hypotonia of muscles in, 481. 
ictus laryngeus in, 487. 
intestinal crises in, 486. 
laryngeal crises, 487. 
loss of sensation of posture in, 479. 
mal perforant in, 492. 
migraine in, 490. 



Tabes dorsalis, symptomatology of, motion 
in, 481. 
muscles in, abnormal flaccidity of, 481. 
muscles of eye in, paralysis of, 480. 
mydriasis in, 475. 
myosis in, 474. 
nails in, falling out, 492. 
neuralgia anovesicalis in, 486. 
nuclear paralysis in, 489. 
ocular muscles in, disturbance of, 473. 
olfactory nerve in, 489. 
ophthalmoplegia externa in, 488. 

interna in, 488. 
optic nerve in, atrophy of, 480. 

white atrophy of, 480. 
pain sense disturbed in, 477. 
pains in, lancinating, 472. 
paralysis in, 488. 

of hypoglossal nerve in, 489. 

of muscles of eye in, 488. 

of muscles of larynx in, 488. 

of muscles of mastication in, 488. 

of tongue in, 489. 

of vocal cords in, 488. 
paralytic stage in, 494. 
paresthesia in, 473. 
perforating ulcer of foot in, 492. 
pharyngeal crises in, 487. 
plantar reflexes in, 479. 
polyuria in, 490. 
pre-ataxic stage in, 471. 
psychical disturbances in, 492. 
ptosis in, 488. 
pupil in, Argyll-Robertson, 474. 

rigidity of, 474. 
pupils, condition of, 473. 

size of, in, 475. 
rectal crises in, 486. 
renal crises in, 486. 
Romberg's sign in, 476. 
sensation disturbed in, 477. 
sexual function in, 474. 
skin in, hyperesthesia of, 473. 
spontaneous fractures in, 490. 
swaying with closed eyes in, 476. 
tachycardia in, 490. 
taste in, 489. 

tearing of tendons in, 491. 
teeth in, falling out of, 492. 
temperature sense in, 477. 
tendon reflex in, 475. 
terminal stage in, 494. 
testicular crises in, 486. 
tinnitus aurium in, 490. 
triceps tendon in, 476. 
trophic disturbances in, 490. 
tracheo-bronchial crises in, 487. 



1202 



INDEX OF SUBJECTS 



Tabes dorsalis, symptomatology of, ulnar 
nerve in, analgesia of, 478. 

urine in, retention of, 474. 

vegetative functions in, 482. 

vertigo in, 490. 

vesical crises in, 486. 

vision in, acuity of, 473. 

vocal cords in, ataxia in, 484. 

vomiting in, 485. 

vomitus in, examination of, 486. 
termination of, 521. 
theories regarding origin of, 500, 501. 
treatment of, 534. 

antisyphilitic, 436. 

arsenic in, 542. 

balneotherapy in, 542. 

bromin in, salts of, 542. 

compresses in, 547. 

corset brace in, 547. 

derivatives in, 545. 

Determann's method in, 541. 

drugs in, 541. 

electrotherapy in, 544. 

ergot in, 541. 

ether spray in, 547. 

exercise in, 546. 

Frenkel's method for ataxia in, 549. 

hydrotherapy in, 543. 

internal remedies for pain in, 548. 

iodin in, intradural injections of, 539. 

iodin therapy in, 539- 

iodipin in, 542. 

iron in, 542. 

lecithin in, 542. 

massage in, 545. 

mode of life in, 540. 

nerve stretching in, 546. 

opotherapy in, 542. 

phytin in, 542. 

potassium iodid in, 542. 

prophylaxis in, 534. 

remedies for the crises in, 548. 

rest in bed in, 541 . 

sea and river bathing in, 544. 

silver nitrate in, 541. 

sodium nitrite in, 548. 

specific, contraindicated, 537. 

strychnin in, 542. 

suspension in, 546. 

thermocautery in, 545. 

tonic method in, 538. 

Zittmann's method in, 539. 
trophic disturbance, cause of, 510. 
varieties of, 521. 
visceral crises in, 485. 
Tabes fruste, 528. 
incompleta, 526. 



Tabes fruste, infantilis, 524. 

juvenilis, 524. 

paralytica, 523. 

paresthetica, 523. 

superior, 524. 

visceralis, 524. 
Taboparalysis, 493, 524. 
Tabophobia, 988. 
Tachycardia, 595. 

in hysteria, 1075. 

in polyneuritis, 805. 
Tachypnea, 1116. 
Tailor's spasm, 1158. 
Talalgia, 1140. 
Tallermann apparatus, 775. 
Talon hand, 714. 
Tapir lip, 639. 
Tapetum, 17. 
Tarsalgia, 1140. 
Taste, center of, 13. 
Teeth in nervous disease, 145. 
Tegmental radiation, 99. 
Tegmentum, 21, 109. 

region of, 21. 
Telachorioidea, 4. 
Telegrapher's cramp, 1154. 

symptoms of, 1156. 
Telencephalon, 2, 3. 
Temperature in hysteria, 1075. 
Tendon reflex, 155. 

mechanism of, 200, 201. 
Tenesmus vesicae, 987. 

in neurasthenia, 981. 
Teres major, atrophy of, 146. 
Test, zonifugal, 1065. 

zonipetal, 1065. 
Tetania strumipriva, 911. 
Tetanoidie, 919. 
Tetanus, 918. 
Tetany, 907. 

acute relapses in, 919. 

classification of, 907. 

diagnosis of, 918. 

differential diagnosis of, 918. 
from hysteria, 918. 
from tetanus, 918. 
from Thomsen's disease, 919. 

etiology of, 908, 909,911. 

gastrectasis in, 920. 

history of, 907. 

infantile, 937. 

pathology of, 920. 

prognosis of, 919. 

symptoms of, 914. 
albuminuria in, 917. 
Chvostek's sign in, 916. 
circulation in, 917. 



INDEX OF SUBJECTS 



1203 



Tetany, symptoms of, electric hyperirrita- 
bility in, 915. 
epileptic spasms in, 917. 
Erb's sign in, 915. 
glycosuria in, 917. 
Hoffmann's sign in, 915, 916. 
hysterical attacks in, 918. 
mechanical hyperirritability in, 916. 
organs of special sense in, 917. 
polydipsia in, 917. 
polyuria in, 917. 
respiration in, 917. 
sensorium in, 916. 
spasms in, 914. 
temperature in, 917. 
tendon reflexes in, 917. 
trophosecretory anomalies in, 917. 
Trousseau's sign in, 915. 
treatment of, 920. 
drugs in, 921. 
electricity in, 921. 
prophylaxis in, 920. 
with myotonic symptoms, 931. 
Thalamus opticus, 3. 
Thiosinamin in scleroderma, 1144. 
Thomsen's disease, 922. 
clinical history of, 922. 
etiology of, 925. 

anomaly of metabolism in, 930. 
nature of the disease, 930. 
diagnosis of, 930. 
differential diagnosis of, 931. 

from occupation neuroses, 931. 
pathology of, 929. 
prognosis of, 932. 
symptoms of, 925, 926. 
intention rigidity in, 926. 
muscles in, stiffness of, 926. 
treatment of, 932. 
Thoracic cord, 28. 

Thoracicus longus nerve, paralysis of, 701. 
Thorax en bateau, 603. 
Thumb, paralysis of, 716. 
Thyreoidactomy, 973. 
Thyreoidin, 972. 

Tibial nerve, in progressive neurotic mus- 
cular atrophy. 646. 
paralysis of, 733. 
diagnosis of, 734. 
prognosis of, 735. 
symptoms of, 734. 
treatment of, 735. 
Tic, 179. 

chewer's, 947. 
licking, 947. 
lingual, 947. 
pyschogenous, 951. 



Tic, scratching, 956. 

significance of, 179. 

watchmaker's, 945. 
Tic convulsif, 935, 941. 

de salaam in epilepsy, 1022. 

impulsif, 942. 
Tigroid substance, 36. 
Tigrolysis, 499. 
Tinnitus aurium, 190. 

hysterical, 1072. 

in traumatic neuroses, 1109. 
Tinsmith's spasm, 1158. 
Tissues, deeper sensibility of, 194. 
Tissue neurosis in nervous disease, 144. 
Toes, movements of, 166. 
Tongue, atrophy of, 146. 

hemiatrophy of, in syringomyelia, 595. 

hysterical spasm of, 947. 

idiopathic spasm of, 946. 

injuries of, in epilepsy, 1024. 

movements of, 159. 

myokymia of, 946. 

paralysis of, 161, 690. 

spasm of, 946. 
Topoalgia, 1070. 
Torticollis, 950. 

mental, 951. 
Torticollis spasticus, 950. 
Tourbillous, 619. 
Trachelismus, 1023. 
Tract, association, 87. 

central tegmental, 92, 106. 

lemniscus, 106. 

motor, diseases of, 69. 

olfactory central, 99. 

optic, 84. 

gray substance of, 96. 
secondary, 97. 

pyramidal, 18, 64, 67, 123. 
lateral column, 69. 

reflex, 86. 

sensory, 73. 
Tracts in the cerebellum, 107. 

in the cerebrum, 103. 

in peduncles, 103. 
Trauma, effects of, in nervous disease, 133. 

influence of, 133. 
Traumatic neuroses, 1098. 

abortive types of, 1116. 

course of, 1122. 

diagnosis of, 1116. 

differential diagnosis of, 1117. 
from brain tumor, 1117. 

etiology of, 1101. 
alcohol in, 1101. 
injuries in, 1 101. 
metallic poisons in, 1101. 



1204 



INDEX OF SUBJECTS 



Traumatic neuroses, etiology of, mental 
inferiority in, 1101. 

psychical genesis in, 1 104. 
shock in, 1101. 
history of, 1098. 

idiomuscular contraction in, 1107. 
pathogenesis of, 1101. 
prognosis of, 1122. 
prophylaxis of, 1124. 
simulation, 1116. 
symptoms of, 1105. 

adynamia in, 1108. 

anesthesia in, 1110. 

appetite in, 1106. 

arteriosclerosis in, 1115. 

atrophy in, 1 107. 

bladder disturbance in, 1112. 

consciousness in, 1114. 

contractions in, 1114. 

convulsions in, 1114. 

coordination in, 1108. 

cough in, 1116. 

cutaneous reflexes in, 1112. 

defective memory in, 1105. 

ear disturbance in, 1109. 

edema in, 1113. 

eyes in, 1109. 

facial expression in, 1105. 

gait in, 1108. 

headache in, 1111, 1114. 

heart in, 1115. 

hemorrhage in, 1116. 

hypotonia in, 1108. 

intellection in, 1105. 

invalidism in, 1106. 

kidneys in, 1116. 

lungs in, 1116. 

mental condition in, 1105. 

mucous membrane reflexes in, 1112. 

muscular rigidity in, 1107. 
tension in, 1107. 

paralysis in, 1108. 

photophobia in, 1109. 

pupils in, 1.112. 

rectal disturbance in, 1112. 

reflexes in, 1111. 

secretion of sweat in, 1113. 

sensation in, 1110. 

skin in, 1113. 

spasms in, 1114. 

trophic signs in, 1113. 

vasomotor disturbance in, 1113. 

vertigo in, 1114. 

weakness of will in, 1106. 
treatment of, 1 124. 

bromidsin, 1125. 

drugs in, 1125. 



Traumatic neuroses, treatment of, hydro- 
therapy in, 1125. 
massage in, 1125. 
Transference in hysteria, 1064, 1078. 
Trapezius, atrophy of, 146. 
Tremor, hysterical, 176, 894. 

in epilepsy, 1018. 

in Graves' disease, 961. 

in hysteria, 1061. 

in paralysis agitans, 885. 

in progressive neurotic muscular atrophy, 
647. 

intention, 176. 

neurasthenic, 177, 893. 

senile, 177, 893. 

significance of, 176. 

tobacco, 177. 
Tribadism, 981. 

Trigeminal nerve, paralysis of, 676. 
prognosis of, 681. 
symptoms of, 677. 
treatment of, 681. 
Tripperfaden, 985. 
Trismus, 948, 949. 

hysterical, 949. 

in neuralgia, 156. 

psychogenous, 949. 

reflex, 153. 
Trochlear nerves, nuclei of, 52, 54. 
Trophic changes, in cerebral paralysis of 
children, 148. 

in infantile paralysis, 148. 

in nervous disease, 143. 

in neuralgia, 834. 

significance of, 141. 
Trophcedema chronicum hereditarium, 1147. 
Trousseau's sign, 915. 
Trumpet player's cramp, 1157. 
Trunk, center for, 61. 

muscles of, examination of, 152. 
Tube, medullary, 1. 
Tuber cinereum, 20, 84. 
Tuberculum acousticum, 83, 91. 
Turk's bundles, 113, 124. 
Typist's cramp, 1154. 

clinical history of, 1155. 

etiology of, 1155. 

symptoms of, 1 155. 

Ulcer, perforating, of foot, 601. 

significance of, 144. 
Ulcers in nervous disease, 144. 
Ulnar nerve, paralysis of, 712. 

etiology of, 712. 

diagnosis of, 714. 

prognosis of, 714. 

symptoms of, 713. 



INDEX OF SUBJECTS 



1205 



Ulnar nerve, paralysis of, treatment of, 

715. 
Uncus, 9. 

Ungius inearnatus, 1056. 
Uranism, 981. 
Uremia, 1013. 

lumbar puncture in, 251. 
Urethralgia, 981. 
Urethrospasm, 981. 
Urine, reflex for discharge of, 210. 
Urobilinuria, 785. 
Urticaria factitia, 142. 
Urticaria in syringomyelia, 600. 

Vagus. See Pneumogastric nerve, 683. 

Valleix's points, 830, 831. 

Varolian bend, 2. 

Vasocorona, 30. 

Vasomotor ataxia, 1137. 

Vasomotor collar, 141. 

Vasomotor disturbance, diagnosis of, 141. 

in neuralgia, 833. 
Vasomotor epilepsy, 1023. 
Vasomotor trophic neuroses, 1128. 

general description of, 1128. 

symptoms of, 1128, 
Velum anticum, 107. 
Ventricle, fourth, 4, 21, 23. 

lateral, 3, 16. 

third, 3, 19, 20. 
Ventricular system, 3. 
Vertebrae, carcinoma of, 367, 389. 

sarcomata of, 367. 
Vertebral column, examination of, 138. 
Vertigo, hysterical, 1072. 

in syringomyelia, 605. 

in tabes, 490. 

in traumatic neurosis, 1115. 
Vertigo epileptica, 1022. 
Vesicles, cerebral, 2. 

occipital, 4. 
Vesicular substance, structure of, 42. 
Vestibule, 82. 
Vision, acuity of, 185. 

field of, 185. 

limited, in epilepsy, 1021. 
Violinist's cramp, 1156. 



Vocal cords, ataxia of, 484. 

paralysis of, in amyotrophic lateral scle- 
rosis, 658. 
in polyneuritis, 805. 
Voice, falsetto, 163. 
Vomiting in epilepsy, 1024. 

in syringomyelia, 605. 
Vulvovaginal crises, 985. 

Waller's degeneration, 777. 

secondary, 780. 
Watchmaker's neurosis, 1158. 
Weber's test in hysteria, 1069. 
Weigert's law, 374. 
Werdnig-Hoffmann's disease, 649. 
Westphal's sign, 475. 
White edema, 1075. 
Whitlow, 600. 

Will, conscious impulses of, 60. 
Willis, circle of, 26. 

nerve of. See Spinal accessory nerve. 
Wood-sawyer's cramp, 1158. 
Word conception, 270. 

deafness, 275. 

mutism, 275. 

sounds, understanding of, 270. 
Wrist-drop, in lead paralysis, 800. 

in radial paralysis, 745. 
Writer's cramp, 953, 1149. 

clinical case of, 1149. 

diagnosis of, 1154. 

etiology of, 1153. 

prognosis of, 1154. 

symptoms of, 1150. 

treatment of, 1154. 
Writer's pain, 1151. 
Written language, disturbance of, 284. 
Writing center, 266. 

X-rays in disease of the brain, 215. 

Yohimbin, 1009. 

Zones, epileptogenous, 1036. 

hypalgesic, in syringomyelia, 597. 
Zoster ophthalmicus, 680. 



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